A stent-graft technique was developed by Parodi et al. and has been used clinically for thoracic endovascular aortic repair (TEVAR) since the 1990s. We evaluated how the new stent-graft technology contributed to expanding aortic surgery and improving surgical outcomes of aortic surgery. TEVAR was performed in a limited number of institutes in the early 2000s and was greatly enhanced by the approval of commercially available stent grafts in 2008. Its performance increased steadily thereafter, with 0 cases performed in 1999, 1658 in 2009, and 6461 in 2019. The ratio of TEVAR was 0% in 1999, which increased to 13.9% in 2009 and 28.5% in 2019, respectively. TEVAR has greatly contributed to the improvement of surgical outcomes, especially in non-dissection ruptured aneurysms and type B acute aortic dissection. TEVAR was performed in 53% of ruptured aneurysms, and the 30-day mortality rate improved to 13.9% in 2019 due to a 30-day mortality rate of 12.5% in TEVAR. The effect of TEVAR was more remarkable in patients with acute type B aortic dissection, where the 30-day mortality rate was 5.7%, and the procedure was performed in 75% of cases. The overall 30-day mortality rate improved to 7.0% for all patients with type B acute aortic dissection in 2019. The expansion of TEVAR using stent grafts greatly increased the number of aortic surgeries and played a significant role in improving surgical outcomes. Stent-graft technology has influenced the field of aortic surgery.

INTRODUCTION: Cardiac surgery in patients with osteogenesis imperfecta is a high-risk procedure because of tissue fragility, which leads to complications, such as bleeding, fractures, and increased mortality. A less invasive approach, such as right anterior mini-thoracotomy, may help reduce these risks and improve outcomes.

CASE PRESENTATION: A 36-year-old woman with severe aortic regurgitation was referred for surgery. She had type I osteogenesis imperfecta, which was diagnosed based on childhood fractures, deafness, and a family history of the disease. To avoid sternotomy and minimize the risk of fracture, aortic valve replacement was performed through a small right anterior thoracotomy using a wound retractor instead of a thoracotomy device. A 23-mm mechanical valve was implanted with cross-clamp and cardiopulmonary bypass times of 62 and 107 min, respectively. The patient’s postoperative course was uneventful. At the 2-year follow-up, she remained in good condition, with no significant echocardiographic findings.

CONCLUSIONS: For patients with osteogenesis imperfecta, the right anterior mini-thoracotomy approach is an excellent surgical option that may help improve outcomes.

INTRODUCTION: Cronkhite-Canada syndrome (CCS) is a rare non-hereditary disorder in which multiple non-tumorous polyps occur throughout the digestive tract. Although surgical treatment is sometimes indicated due to the occurrence of gastric and colorectal cancer, in CCS, the polyposis makes it difficult to distinguish from cancer and requires careful follow-up. CCS is characterized by the presence of inflammatory findings, and steroids and immunosuppressants are used as key drugs. So, it is crucial to perform minimally invasive surgeries whenever possible, and in this regard, robotic-assisted surgery might prove to be one of the appropriate approaches.

CASE PRESENTATION: The patient was a 72-year-old woman who had developed CCS 5 years previously and had been in remission with medication. During annual colonoscopy, multiple colorectal polyps were found in the ascending colon and resected endoscopically. One of the polyps was diagnosed as an adenocarcinoma with deep infiltration and vascular invasion. The patient was referred to our department for additional resection and a robotic-assisted right colectomy was performed. Pathological findings showed no residual tumor in the area after endoscopic mucosal resection (EMR), but laterally spreading tumor (LST) lesion showed adenocarcinoma in adenoma. The patient was discharged home on POD 7 without any postoperative complications.

CONCLUSIONS: This report is the first case of robotic surgery for colorectal cancer complicated by CCS with a safe surgical outcome.

INTRODUCTION: Although several studies have highlighted the importance of recognizing gastroduodenal artery (GDA) anomalies during pancreaticoduodenectomy, their relevance during distal pancreatectomy has not been explored. Herein, we describe the safe performance of laparoscopic distal pancreatectomy in a patient with a rare vascular anomaly, specifically a GDA originating from the superior mesenteric artery (SMA).

CASE PRESENTATION: A 63-year-old woman presented with recurrent pancreatitis due to a cystic lesion in the pancreatic body. Imaging suggested a branch-duct intraductal papillary mucinous neoplasm with substantial ductal stenosis. Multidetector CT (MDCT) revealed a rare anatomical variant: the GDA, arising from the SMA and traversing along the inferior border of the pancreas. Laparoscopic distal pancreatectomy was performed after placing an endoscopic nasopancreatic drainage tube. The aberrant GDA was successfully preserved through careful dissection and vessel loop isolation. Pancreatic transection was completed without vascular injury. A postoperative pancreatic fistula developed and was conservatively managed. Histopathological examination confirmed that the lesion was an intraductal papillary mucinous carcinoma without any invasive features. Follow-up MDCT revealed sustained patency and perfusion of the preserved GDA, right gastroepiploic artery, and anterior superior pancreaticoduodenal artery.

CONCLUSIONS: Laparoscopic pancreatectomy can be safely performed in patients with SMA-derived GDA anomalies, when supported by detailed preoperative imaging and precise intraoperative techniques. These findings highlight the necessity of routine preoperative vascular assessment in patients undergoing minimally invasive pancreatic surgery, reinforcing the broader applicability of these approaches for patients with complicated vascular anatomy.

INTRODUCTION: Smooth muscle tumors, such as leiomyoma or leiomyosarcoma, are mesenchymal tumors that originate from smooth muscle cells. Smooth muscle tumors in the abdominal cavity often originate from the uterus, gastrointestinal wall, and inferior vena cava, but rarely originate from the pancreas. We present a rare case of a pancreatic leiomyoma that was successfully treated via partial pancreatectomy.

CASE PRESENTATION: A 74-year-old man with a pancreatic head tumor was referred to our hospital. Imaging revealed a heterogeneous 61 × 45 mm mass with a contrast effect in the pancreatic head. Distant metastases were not detected. The tumor was diagnosed by endoscopic ultrasonography-guided fine-needle aspiration as a leiomyoma, and follow-up was planned. Five years after the initial visit, the patient developed abdominal tension, and a CT scan revealed that the tumor had grown to 80 × 60 mm. It was decided that the tumor required surgical treatment comprising pancreaticoduodenectomy. However, the intraoperative findings revealed that the tumor was able to be moved freely but was unable to be dissected from a part of the pancreatic head. Therefore, partial pancreatectomy was performed. Histopathology showed spindle-shaped tumor cells with mild atypia. Pancreatic tissue was present at the tumor margins, and the tumor was adjacent to the vasculature. Based on the clinical and pathological findings, the tumor was diagnosed as a smooth muscle tumor originating from the pancreas. The patient was discharged without complications on POD 8 and has now been doing well without recurrence for more than 6 months after surgery.

CONCLUSIONS: A very rare slow-growing pancreatic smooth muscle tumor was removed via partial pancreatectomy after the patient developed abdominal symptoms after a 5-year observation period.

INTRODUCTION: Castleman’s disease is a rare lymphoproliferative disorder. Unicentric Castleman disease (UCD) is usually benign and can be treated by complete resection. We herein report a pediatric case of UCD that occurred in the transverse mesocolon and was successfully treated with a laparoscopic approach.

CASE PRESENTATION: The patient was a 10-year-old girl at the time of the surgery. She was referred to our hospital with a persistent fever of unknown origin and high C-reactive protein levels. Abdominal enhanced MRI showed a 38 × 25-mm enhanced mass in the left transverse mesocolon, and fluorodeoxyglucose PET-CT showed an abnormal accumulation in the same lesion. UCD was the most suspected diagnosis, and laparoscopic tumor resection was planned and performed. The tumor was adjacent to the inferior mesenteric vein and the left branch of the middle colic artery. There was no evidence of vascular invasion into these vessels. The feeding arteries and drainage veins were securely clipped and cut using an ultrasonic harmonic scalpel. Further dissection revealed swollen bead-like lymph nodes connected to the tumor. After all swollen lymph nodes and the tumor were isolated, they were completely resected en bloc. Colectomy was unnecessary because the main mesenteric vessels were preserved. The histopathological diagnosis was hyaline vascular-type Castleman disease. Her symptoms, such as the fever, disappeared soon after surgery. The patient was discharged on day 9 without any complications. The C-reactive protein level dropped within the normal range 2 weeks after surgery. There was no evidence of recurrence or symptoms 3 years after surgery.

CONCLUSIONS: We encountered a rare case of pediatric UCD. Although only limited cases of the laparoscopic approach for pediatric UCD have been reported, we believe that the laparoscopic approach is useful for UCD in children.

INTRODUCTION: With advancements in multidisciplinary treatments such as chemotherapy and radiation therapy, there have been an increasing number of recent reports regarding surgical interventions for unresectable pancreatic ductal adenocarcinoma with metastasis (UR-M PDAC), known as “conversion surgery”. Olaparib, which is one of the treatment options, has been approved as a maintenance treatment in patients with UR-M PDAC and germline BRCA mutations who achieve disease control after a platinum-based regimen such as modified FOLFIRINOX (mFFX).

CASE PRESENTATION: A 49-year-old male patient was diagnosed with PDAC in the pancreatic body-tail. The 1st staging laparoscopy (SL) revealed hepatic metastases in both lobes and positive peritoneal lavage cytology. A germline BRCA2 mutation was considered, and mFFX was introduced and continued for 8 courses, followed by 8 months of olaparib. The initially elevated levels of duke pancreatic monoclonal antigen type 2 and carcinoembryonic antigen subsequently returned to normal, and CT revealed more than 60% shrinkage of the primary tumor. The 2nd SL revealed the disappearance of multiple hepatic metastases and negative conversion of peritoneal lavage cytology, so we performed chemoradiotherapy with olaparib to ensure an antitumor effect and surgical margin negativity, followed by distal pancreatectomy with celiac axis resection. Histopathological findings revealed R0 resection and a pathological complete response (pCR). Adjuvant olaparib treatment was administered for 10 months starting 2 months after surgery, and the patient has remained alive without recurrence for 2 years after surgery.

CONCLUSIONS: We report the very rare case of a patient with BRCA-positive PDAC with multiple liver metastases who underwent conversion surgery after treatment with mFFX and olaparib, achieved a pCR, and has remained recurrence-free for 2 years.

INTRODUCTION: Cervical esophageal duplication is a rare congenital anomaly that occasionally causes compressive symptoms. Herein, we present a unique case of unilateral vocal cord paralysis caused by a cervical esophageal duplication cyst with a granuloma containing a foreign body.

CASE PRESENTATION: A 10-year-old boy presented with a 1.5-month history of hoarseness and choking. Laryngoscopy revealed left vocal cord paralysis, and CT revealed a mass near the inferior pole of the left thyroid lobe. Fine-needle aspiration cytology revealed no evidence of malignancy. Ultrasonography and MRI findings suggested an esophageal duplication cyst with an inflammatory granuloma. Antibiotic therapy was ineffective, and a surgical approach was considered. The esophageal duplication cyst was resected through a collar incision, and a film-like foreign body with granulation tissue was extracted from the abscess. The recurrent laryngeal nerve was preserved, and the vocal cord function improved within 2 months.

CONCLUSIONS: This case highlights the unusual presentation of an inflamed cervical esophageal duplication cyst with an embedded foreign body, which resulted in recurrent laryngeal nerve impairment and subsequent unilateral vocal cord paralysis. Early surgical intervention with careful nerve preservation can result in a functional recovery.

INTRODUCTION: Hepatoblastoma with tumor thrombi involving three hepatic veins or extending into the inferior vena cava (IVC) toward the right atrium is mostly considered unresectable. In the current era of liver transplantation for unresectable hepatoblastoma with stable outcomes, it is challenging to choose extreme hepatectomy with hypothermic perfusion under total hepatic vascular exclusion. We herein report a successful case of extreme liver resection with in situ hypothermic perfusion.

CASE PRESENTATION: A 10-month-old girl was referred for an unresectable hepatoblastoma (alpha-fetoprotein [AFP] 592037 ng/mL), approximately 12 cm in size, occupying the right lobe with a tumoral thrombus extending into the IVC toward the right atrium. The tumor continued to involve three hepatic veins and the IVC after intensive chemotherapy, which was categorized into POSTTEXT III (P1, V3, M0) staging. We planned an ante situm technique or in situ hypothermic perfusion to accomplish the complete removal of the large tumor and venous reconstruction. The adopted surgical technique was as follows: (1) hepatic partition on the line of extended right hepatectomy, including segment Iva; (2) mobilization of the suprahepatic IVC with tumor thrombi; (3) insertion of a cannula for hypothermic perfusion was from the stump of the right portal vein to the left lobe; (4) complete occlusion of the hepatic inflow following systemic heparinization with cutting the left hepatic vein and hypothermic perfusion with cold preservation solution using crushed ice; (5) complete tumor removal with the IVC tumoral thrombi; and (6) reconstruction of the IVC and left hepatic vein using an artificial vessel graft without requiring ante situm position, followed by reperfusion of the remnant liver. Time for cooling and preservation of the remaining liver in the body was 40 minutes, and time for IVC reconstruction was 21 minutes. The histological examination was margin-negative, and the AFP level was normalized.

CONCLUSIONS: Resection of liver tumors invading the IVC or hepatic veins has become possible with technical lessons learned from liver transplantation skills. This procedure is a realistic option for achieving surgical cure and improving the quality of life in select pediatric patients with otherwise unresectable hepatoblastomas.

INTRODUCTION: Partial anomalous pulmonary venous connections (PAPVC) are rare. We report a case of right pneumonectomy for right lower lobe lung cancer, wherein a PAPVC was detected in the right upper lobe vein before surgery.

CASE PRESENTATION: A 71-year-old man was diagnosed with a mass in the right lower lobe on chest CT. Non-small-cell lung cancer was diagnosed using bronchoscopy. Right bilobectomy (middle and lower lobes) was planned to secure a margin for resection due to hypoplasia of the middle lobe; however, preoperative contrast-enhanced CT revealed a PAPVC involving the right upper lobe. If a PAPVC is identified in a non-resected lung lobe, the patient is at risk of postoperative right heart failure. In cases of PAPVC in a non-resected lobe with Qp/Qs ≥1.5, preoperative or intraoperative revascularization is recommended. Echocardiography demonstrated a pulmonary to systemic flow ratio (Qp/Qs) of 1.39; however, right heart catheterization showed an increase in Qp/Qs to 1.66. Therefore, we considered repairing the right upper pulmonary vein. However, owing to the unstable position of the right upper lobe and the length of the repaired vessel, we performed a right pneumonectomy considering the risk of vascular flexion and occlusion. The postoperative course was uneventful, and echocardiography performed 3 months after the procedure revealed an ejection fraction of 57%.

CONCLUSIONS: In the present case, by assessing Qp/Qs using both echocardiography and right heart catheterization, we determined an appropriate surgical approach. However, right pneumonectomy is a risk factor for right-sided heart failure, and limited resection or nonsurgical treatment should be considered in certain cases. The presence of PAPVC in a non-resected lung requires caution when selecting the surgical approach. In cases of PAPVC, detailed preoperative CT evaluation is essential.

INTRODUCTION: In patients with Peutz–Jeghers syndrome (PJS), repeated laparotomy and bowel resection can lead to short bowel syndrome and adhesions. Minimizing surgical invasiveness while effectively managing intussusception caused by polyps is therefore essential. We present a pediatric case of recurrent intussusception due to a jejunal polyp near the ligament of Treitz, successfully treated with a hybrid approach combining laparoscopic reduction and double-balloon enteroscopy (DBE)–assisted polypectomy.

CASE PRESENTATION: A 9-year-old girl presented with small bowel intussusception and underwent laparoscopic-assisted resection of a 40-cm jejunal segment containing a 35-mm polyp and multiple adjacent polyps. Postoperative findings led to a diagnosis of PJS. Two weeks later, she developed recurrent intussusception. CT revealed a lead-point mass without signs of ischemia or strangulation. At reoperation, laparoscopic reduction was achieved except for residual intussusception near the ligament of Treitz. Under continuous laparoscopic observation, DBE identified a 40-mm polyp 30–40 cm distal to the pylorus. En bloc removal was not feasible; therefore, piecemeal resection was performed, resulting in complete release of the intussusception. Follow-up capsule endoscopy at 2 months confirmed no residual lesion.

CONCLUSIONS: This case highlights the feasibility and safety of a hybrid laparoscopic–endoscopic approach for intussusception caused by proximal small bowel polyps in PJS, allowing bowel preservation and effective lesion removal. This strategy may serve as a valuable treatment option in selected cases.

INTRODUCTION: While para-aortic lymph node recurrence may occur after curative resection for pancreatic ductal adenocarcinoma (PDAC), it is rarely seen as a late recurrence and is usually treated with systemic chemotherapy. In contrast, recurrence of pancreatic neuroendocrine neoplasms (Pan-NENs) can occur more than a decade after surgery and may be amenable to surgical resection. Although immunohistochemical techniques have significantly enhanced the diagnostic distinction between PDAC and Pan-NEN, challenges remain, particularly in older cases.

CASE PRESENTATION: A 77-year-old man underwent distal pancreatectomy for pancreatic body cancer 11 years prior, initially diagnosed as PDAC based on glandular morphology, and received adjuvant S-1 chemotherapy. Surveillance ended after 5 years without recurrence. Eleven years after surgery, imaging performed during evaluation for elevated prostate-specific antigen revealed a 16-mm para-aortic lymph node enlargement. CT-guided biopsy showed neuroendocrine morphology with chromogranin A and synaptophysin positivity, consistent with neuroendocrine tumor (NET). Retrospective histological review of the original surgical specimen revealed previously unrecognized NET-G2 components (Ki-67, 5.0%). Surgical resection of the para-aortic and mesenteric lymph nodes was performed, and pathology confirmed NET-G2 metastases (Ki-67, 6.7%). The patient recovered uneventfully and remains recurrence-free at 7 months postoperatively under active surveillance.

CONCLUSIONS: This case highlights the importance of tissue diagnosis when late-onset recurrence is observed in patients with a prior diagnosis of PDAC. It also underscores the potential for extremely delayed recurrence in Pan-NENs and the importance of long-term surveillance. For patients presenting with isolated distant recurrence long after initial treatment for PDAC, biopsy and pathological re-evaluation should be considered to ensure appropriate therapeutic decision-making.

INTRODUCTION: Bronchopleural fistula (BPF) is a severe complication of lung cancer surgery that is often associated with impaired blood supply and poor healing. Thoracic aortic aneurysms (TAA) frequently coexist with lung cancer owing to their shared risk factors, and thoracic endovascular aortic repair (TEVAR) may further increase the risk of BPF by compromising bronchial artery blood flow.

CASE PRESENTATION: A 72-year-old male with a history of cerebral infarction, hypertension, and a 4.8 cm TAA was incidentally found to have a 1.4 cm pulmonary nodule in the right lower lobe. Staged treatment was planned using TEVAR, followed by right lower lobectomy. TEVAR was performed successfully, and 48 days later, lobectomy confirmed stage IB large-cell carcinoma. On POD 27, the patient developed fever and dyspnea. CT revealed BPF, which was likely due to reduced bronchial blood flow after TEVAR. Chest tube drainage and an open-window thoracostomy were performed. Eight months later, thoracoplasty was performed using a muscle flap and omentopexy. The patient recovered completely and remained recurrence-free for 2 years.

CONCLUSIONS: This case underscores the importance of careful planning and surgical strategies to prevent BPF in patients with concomitant lung cancer and TAA. Early recognition of risk factors, including TEVAR-related bronchial ischemia, is crucial for optimizing outcomes.

INTRODUCTION: Pulmonary artery intimal sarcoma (PAIS) is a rare tumor, and the prognosis is extremely poor. Radical surgery for PAIS is the best option for prolonging survival. Thus, PAIS should be diagnosed as soon as possible, and radical surgery should be performed rapidly. However, it is difficult to differentiate PAIS from pulmonary embolism in the early stage. We report the case of a patient who was diagnosed with PAIS by endovascular catheter biopsy in the early period and who underwent left pneumonectomy, accompanied by resection of the main and right pulmonary arteries and right pulmonary artery reconstruction via median sternotomy for radical surgery.

CASE PRESENTATION: A 53-year-old man presented to cardiologists for dyspnea. Contrast-enhanced CT revealed an occlusive mass in the left and main pulmonary arteries. The cardiologists diagnosed the patient with pulmonary embolism and started administering an anticoagulant. However, the mass did not shrink, and the cardiologist consulted the cardiovascular surgeons. A cardiologist performed an endovascular catheter biopsy to differentiate between the thrombus and the tumor. The biopsy specimen tissue contained atypical cells and was negative for Mouse Double Minute protein 2. The surgery was performed via median sternotomy. Cardiopulmonary bypass was established. The tumor filled the left pulmonary artery and extended to the main and right pulmonary arteries. With sufficient margins from the tumor, the main and right pulmonary arteries were resected and subjected to rapid intraoperative pathological diagnosis, with positive margins of the main pulmonary artery. Additional resection just above the pulmonary artery valve and reconstruction of the right pulmonary artery were performed, followed by left pneumonectomy. The postoperative histological diagnosis of the tumor was PAIS. The surgical margin of the main pulmonary artery was microscopically positive for tumor cells. Chemoradiotherapy was started postoperatively. There was no recurrence in the pulmonary artery, but a head MRI revealed a metastatic brain tumor, and the patient died approximately 1.5 years after surgery.

CONCLUSIONS: Endovascular catheter biopsies are useful for differentiating thrombi from tumors. Furthermore, an early diagnosis of PAIS is important to ensure adequate time for discussing the surgical strategy with thoracic surgeons.

INTRODUCTION: Aorto-esophageal fistula (AEF) is a relatively rare and life-threatening condition. Although various treatment strategies have been developed, including conventional surgical repair, long-term survival remains uncommon. This report presents a case of AEF successfully treated without aortic reconstruction, resulting in long-term survival.

CASE PRESENTATION: The patient was a 74-year-old male with a medical history of total arch replacement and severe emphysema, who presented to our department with hematemesis. Upper gastrointestinal endoscopy results revealed an AEF caused by the prosthetic graft. Emergency thoracic endovascular aortic repair was performed, followed by urgent subtotal esophagectomy the next day, without aortic reconstruction due to the patient’s frailty. The patient underwent gastric conduit reconstruction via the presternal route 7 months later and has remained uneventful for over 6 years.

CONCLUSIONS: The treatment without aortic reconstruction combined with conservative treatment for AEF might be feasible for high-risk surgical patients.

INTRODUCTION: Paraduodenal hernia (PDH), a rare cause of acute abdominal pain in the emergency department, is difficult to diagnose and can cause serious issues by mimicking common conditions.

CASE PRESENTATION: We present a 49-year-old male with a right PDH causing a 2-day history of nausea, bowel obstruction, and periumbilical/right abdominal pain. CT revealed bowel obstruction and a right PDH, confirmed intraoperatively. A Ladd procedure was performed, and the patient recovered well.

CONCLUSIONS: This case highlights specific CT signs that were instrumental in diagnosing this rare disease, ultimately guiding a successful treatment strategy for the patient.

INTRODUCTION: Alectinib, a 2nd-generation anaplastic lymphoma kinase–tyrosine kinase inhibitor (ALK-TKI), is an established 1st-line therapy for advanced ALK fusion gene–positive non–small cell lung cancer (NSCLC). However, the role of salvage surgery following alectinib for locally advanced disease remains uncertain.

CASE PRESENTATION: A 41-year-old woman was diagnosed in the postpartum period with Stage IIIA (cT1cN2M0) ALK fusion gene–positive lung adenocarcinoma. She received 1st-line alectinib, achieving a 55.3% reduction in tumor size over 11 months. Subsequent salvage surgery revealed a pathological complete response with no residual tumor cells. During postoperative follow-up off alectinib, recurrence was observed 20 months after surgery, with new brain and pulmonary metastases. Reintroduction of alectinib achieved renewed disease control, and the patient has remained progression-free for 23 months since restarting therapy.

CONCLUSIONS: This case highlights the potential role of salvage surgery following alectinib in locally advanced ALK fusion gene–positive NSCLC. Furthermore, it suggests that maintenance ALK-TKI therapy after salvage surgery might be associated with a reduced risk of recurrence. Further studies are warranted to optimize perioperative ALK-targeted strategies.

INTRODUCTION: Aberrant right subclavian artery (ARSA) is the most common congenital anomaly of the subclavian artery, occurring in 0.2%–1.5% of the population. While typically asymptomatic, ARSA can cause dysphagia in older patients due to esophageal compression. In pediatric cases, it is less frequent and usually presents with chronic respiratory symptoms resulting from tracheal compression. Acute respiratory failure as a presentation of ARSA is extremely rare. This case report describes a pediatric patient with ARSA-associated esophageal stricture, leading to severe respiratory distress following food intake and requiring surgical treatment.

CASE PRESENTATION: A 1.8-year-old girl developed an inspiratory stridor after eating. Initial inhalation therapy provided temporary relief, but persistent respiratory distress prompted further evaluation. CT revealed an esophageal foreign body, which was successfully extracted using a balloon catheter. However, continued respiratory distress necessitated intubation and intensive care. Bronchoscopy and enhanced CT confirmed tracheal compression due to ARSA, along with esophageal stenosis. Despite initial medical management, recurrent respiratory distress and feeding difficulties required surgical intervention. A right thoracotomy was performed to reposition the aberrant artery anterior to the trachea, alleviating the vascular compression. Postoperatively, the patient made a full recovery, resumed a normal diet, and remained symptom-free at the 6-month follow-up.

CONCLUSIONS: Symptomatic ARSA is rare in pediatric patients but can cause severe respiratory distress due to esophageal and tracheal compression. In cases of recurrent or severe symptoms, surgical intervention is essential to prevent life-threatening complications. This case highlights the importance of considering ARSA in pediatric patients with unexplained respiratory distress, especially when associated with feeding difficulties.

INTRODUCTION: Crohn’s disease (CD) can be complicated by complex fistulas and abscesses, and laparoscopic surgery for such cases is difficult. We report a patient with CD who developed a complex fistula and retroperitoneal abscess that were treated using single-incision laparoscopic surgery.

CASE PRESENTATION: A 34-year-old man presented to the hospital after collapsing and losing consciousness while at work. Blood tests showed a white blood cell count of 20000/μL and severe anemia. Abdominal CT showed a right retroperitoneal abscess, which was drained under CT guidance. After gastrointestinal bleeding and an anal fistula were observed, CD was suspected and confirmed by total colonoscopy. The patient was eventually discharged but readmitted 2 months later for abscess recurrence and a colocutaneous fistula. Once the inflammation had improved after 3 weeks of fasting and intravenous antibiotics, the patient underwent single-incision laparoscopic subtotal colectomy, duodenal fistula closure, and seton drainage.

CONCLUSIONS: Single-incision laparoscopic subtotal colectomy can be performed safely to treat CD-related abscesses and complicated fistulas after inflammation has improved.

INTRODUCTION: Benign peritoneal multicystic mesothelioma (BPMM) is a rare mesothelial tumor with a high local recurrence rate and potential for malignant transformation. It predominantly affects women of reproductive age and is often associated with prior abdominal surgery or inflammation. Complete surgical resection is the standard treatment; however, tumor recurrence remains a concern. BPMM occurring outside the pelvis is extremely rare, and lesions requiring colectomy because of adherence to the gastrointestinal tract are infrequent.

CASE PRESENTATION: A 63-year-old woman presented with right lower abdominal pain. She had undergone cervical cancer surgery 20 years ago. Contrast-enhanced CT and MRI revealed multiple well-defined cystic lesions adjacent to the ascending colon. Colonoscopy revealed extrinsic compression, and ¹⁸F-fluorodeoxyglucose (¹⁸F-FDG)-PET/CT showed no abnormal uptake of ¹⁸F-FDG. Given the patient’s symptoms, a laparoscopic right hemicolectomy with intracorporeal anastomosis was performed. The cystic lesions were firmly adhered to the appendix, cecum, and ascending colon, requiring en bloc resection. Histopathological and immunohistochemical analyses confirmed a diagnosis of BPMM. The patient had an uneventful postoperative course and was discharged on POD 7. No recurrence was observed during the 1-year follow-up period.

CONCLUSIONS: A laparoscopic approach may be a feasible and safe option for complete resection of BPMM as it allows for magnified visualization and careful handling of cystic lesions, avoiding their intraoperative rupture. Given the high recurrence rate of BPMM, close postoperative surveillance is essential. This case illustrates the feasibility of laparoscopic resection for BPMM.

INTRODUCTION: Primary cardiac schwannoma (PCS) is an exceptionally rare neurogenic tumor, with only 53 documented cases worldwide.

CASE PRESENTATION: We report the case of a 62-year-old woman with a 3-year history of paroxysmal palpitations, who subsequently developed progressive symptoms, such as chest tightness, dyspnea, and dizziness. Diagnostic evaluation revealed supraventricular tachycardia along with a 3.4 × 3.5 cm mass located at the coronary sinus ostium. Multimodal imaging—including echocardiography, CT, and MRI—revealed features characteristic of schwannoma, such as T1 hypointensity, T2 hyperintensity, and peripheral enhancement with internal septations. The patient underwent complete surgical resection of an encapsulated interatrial septal mass measuring 5.0 × 4.0 cm via a right atrial approach, with preservation of cardiac architecture and maintenance of compensatory venous drainage through an accessory ostium. Histopathological analysis confirmed a benign schwannoma exhibiting secondary degenerative changes, supported by positive SOX-10 and S-100 immunostaining and a low Ki-67 proliferation index. At the 3-year follow-up, the patient remained free of recurrence and exhibited no postoperative complications.

CONCLUSIONS: This case underscores the diagnostic challenges associated with PCS, particularly in the context of arrhythmias, and emphasizes the importance of multimodal imaging in the preoperative evaluation. The successful surgical resection demonstrates the importance of meticulous surgical planning and technique in managing these rare tumors. This report adds to the limited body of literature on PCS and reinforces the need for a multidisciplinary approach in the diagnosis and management of these complex cases.

INTRODUCTION: Gastric neuroendocrine carcinoma (NEC) is a rare histological subtype of gastric malignancy. A small NEC component may coexist with gastric adenocarcinoma, making preoperative diagnosis challenging. Currently, no established treatment strategies exist for cases in which an NEC component is identified only in postoperative pathological findings.

CASE PRESENTATION: An 82-year-old man underwent esophagogastroduodenoscopy during a routine health checkup and was diagnosed with early-stage gastric cancer. The lesion was a superficial tumor, 30 mm in diameter, located on the lesser curvature of the cardia. Biopsy revealed well-differentiated adenocarcinoma. The patient subsequently underwent laparoscopic proximal gastrectomy with D1+ lymph node dissection. Histopathological examination of the resected specimen revealed adenocarcinoma with an NEC component, accounting for approximately 10% of the tumor. The pathological stage was T1bN1M0 (Stage IB), and the patient did not receive adjuvant chemotherapy. Five months after the surgery, the patient developed anorexia and weight loss. CT revealed multiple liver tumors. Diagnostic laparoscopy with excisional biopsy of the liver lesion was performed, revealing no peritoneal dissemination. Histopathological examination confirmed that the liver tumors were metastatic NEC, indicating hepatic recurrence of gastric NEC. Given the patient’s rapidly deteriorating condition and a performance status of 3–4, aggressive chemotherapy was deemed inappropriate, and palliative care was initiated. The patient died of the primary disease 8 months after surgery.

CONCLUSIONS: A small NEC component may be undetectable preoperatively. The prognosis of mixed tumors is dictated by the malignancy of the NEC component rather than its tumor burden. Therefore, clinicians should consider NEC-equivalent adjuvant chemotherapy and intensive surveillance.

INTRODUCTION: Venous aneurysms in the anterior mediastinum are rare. While there are scattered reports on thoracic venous aneurysms, such as those involving the azygos or brachiocephalic veins, those involving the internal thoracic vein are exceedingly rare. We herein report the case of an internal thoracic venous aneurysm that was initially suspected of being a tumor and diagnosed intraoperatively.

CASE PRESENTATION: A 50-year-old woman underwent CT during hospitalization for cerebral infarction, which revealed a well-defined 14-mm nodular lesion in the anterior mediastinum. Contrast-enhanced CT performed 4 months later showed that the anterior mediastinal lesion had increased to 16 mm and exhibited slight contrast enhancement. ¹⁸F-fluorodeoxyglucose PET/CT demonstrated only a slight uptake in the anterior mediastinal lesion with no abnormal uptake elsewhere. Thymic epithelial tumors and cysts were included in the differential diagnosis, and video-assisted thoracoscopic surgery was planned. Intraoperative findings revealed a dark-red nodule beneath the mediastinal pleura. The right internal thoracic vein was observed to flow into the nodule and an outflow vessel draining into the right brachiocephalic vein was identified. Based on these findings, the lesion was diagnosed as an internal thoracic venous aneurysm. The right internal thoracic vein and outflow vessel were ligated, and the nodule was resected. A histopathological examination confirmed the diagnosis of venous aneurysm.

CONCLUSIONS: Although extremely rare, venous aneurysms can occur in the internal thoracic vein. This condition should be considered in the differential diagnosis of enhancing solid nodules of the anterior mediastinum.

INTRODUCTION: Jejunal diverticulosis is a rare condition, often asymptomatic, but it can lead to serious complications such as diverticulitis or perforation. Management of perforated jejunal diverticula is challenging, particularly in patients with autosomal dominant polycystic kidney disease (ADPKD) who have undergone kidney transplantation and are receiving immunosuppressive therapy. Early diagnosis is often difficult due to nonspecific symptoms and the frequent presence of multiple diverticula, which increases the risk of postoperative complications.

CASE PRESENTATION: We present the case of a 61-year-old woman with ADPKD, who developed jejunal diverticular perforation 3 years after undergoing ABO-incompatible kidney transplantation. She initially presented with mild abdominal pain and was managed conservatively, but her condition worsened 1 month later with evidence of free air on CT. Emergency surgery revealed multiple jejunal diverticula with a perforation on the mesenteric side, and a double-barrel stoma was created to avoid anastomotic leakage. Four months postoperatively, endoscopic and fluoroscopic evaluation from both the oral and stoma sides enabled accurate identification and marking of the diseased segment. A second-stage surgery was successfully performed with segmental jejunal resection and stoma closure. Histopathology confirmed multiple true diverticula, including at the perforation site. The patient recovered well and was discharged approximately 1.5 months later.

CONCLUSIONS: This rare case of perforated multiple jejunal diverticula in a patient with ADPKD highlights the value of a two-stage surgical approach with preoperative endoscopic evaluation to enable safe and targeted resection in complex settings.

INTRODUCTION: Umbilical cord cysts detected after the 2nd trimester of pregnancy are associated with a variety of complications, including omphalocele, and thus require careful monitoring. Congenital hepatic cysts are rare, as are reports of their coexistence with omphaloceles. Herein, we present an unusual case of an omphalocele complicated by a large hepatic cyst that was initially suspected to be an umbilical cord cyst during the fetal period.

CASE PRESENTATION: A male infant was delivered via caesarean section at 36 weeks of age, with ruptured membranes and cloudy amniotic fluid. Fetal ultrasonography at 16 weeks had previously revealed an omphalocele with intestinal prolapse, and at 20 weeks, an umbilical cord cyst was suspected. At birth, the herniation sac ruptured, and abdominal wall closure and cyst excision were performed. The cyst, initially thought to be umbilical in origin, was instead identified as a hepatic cyst connected to the liver. Pathological examination confirmed the diagnosis. The infant was further diagnosed with Beckwith–Wiedemann syndrome, and was ultimately discharged 32 days postoperatively, with no recurrence to date.

CONCLUSIONS: Although asymptomatic congenital liver cysts can often be managed conservatively with follow-up, we considered surgical intervention necessary in this case due to the presence of an omphalocele associated with suspected Beckwith–Wiedemann syndrome

INTRODUCTION: Minimally invasive surgery has recently become the standard approach for pulmonary lesion resection, and its usefulness has been reported. However, these techniques involve small incisions, making intraoperative palpation difficult. Therefore, a simple and minimally invasive method is required to identify these lesions. Herein, we present a case of thoracoscopic lung wedge resection in which intra-thoracic ultrasonography equipped with a new technology was successfully used for lesion localization.

CASE PRESENTATION: A male patient in his 60s presented with a right intrapulmonary nodule following chemoradiotherapy for oropharyngeal cancer. Surgical resection was planned for the suspected malignancy. The procedure was a single-port thoracoscopic wedge resection of the right upper lobe. Intraoperatively, intra-thoracic ultrasonography successfully identified a pulmonary lesion, eliminating the need for palpation. Histopathological examination confirmed squamous cell carcinoma and complete resection was achieved.

CONCLUSIONS: We successfully performed a single-port thoracoscopic pulmonary wedge resection using intraoperative thoracic ultrasonography for precise lesion localization. The ultrasound devices we used were equipped with advanced technology, enabling clearer images. This case highlights the potential utility of intra-thoracic ultrasonography in minimally invasive lung surgery, as it enables accurate lesion localization and successful resection.

INTRODUCTION: Brain metastasis from esophageal cancer is rare. In particular, recurrent brain metastasis following multimodal treatment, such as preoperative chemotherapy and surgical resection, is extremely uncommon.

We present a case of early brain metastasis from esophageal cancer despite achieving pathological complete response (pCR).

CASE PRESENTATION: A middle-aged man presented with dysphagia. Upper gastrointestinal endoscopy revealed stenosis caused by an ulceroinfiltrative tumor located in the middle of the thoracic esophagus. Targeted biopsies confirmed squamous cell carcinoma. Contrast-enhanced CT revealed circumferential, irregular wall thickening with contrast enhancement, showing infiltration into the left main bronchus and enlarged lymph nodes in the paraesophageal and the left supraclavicular regions. The diagnosis was middle thoracic esophageal squamous cell carcinoma cT3br N2M0 cStage IIIB (according to the Japanese Classification of Esophageal Cancer, 12th Edition). The patient underwent chemotherapy, including 5-fluorouracil, cisplatin, and pembrolizumab, as the combined positive score exceeded 10. Following this chemotherapy with an immune checkpoint inhibitor, the tumor had regressed, and targeted biopsies revealed no malignant findings. The post-chemotherapy diagnosis was ycT3rN0M0 ycStage II, and the patient subsequently underwent thoracoscopic esophagectomy. Surgical findings showed no evidence of tumor infiltration. Postoperative histopathological examination showed no residual tumor cells in either the esophagus or resected lymph nodes, corresponding to histological response of grade 3. However, the patient presented with depression 2 months after the surgery, and abnormal behavior was shown 3 months after the surgery. Although cranial CT and MRI revealed ring-enhancing lesions in the right cerebellar hemisphere and the right frontal lobe, there was no recurrence or metastasis other than in the brain. The patient underwent resection of the frontal lobe tumor and was diagnosed with brain metastasis of esophageal cancer. Stereotactic radiation therapy and pembrolizumab were started; however, the patient died 5 months after esophagectomy due to brain metastasis progression.

CONCLUSIONS: 5-Fluorouracil and cisplatin plus pembrolizumab therapy may allow conversion surgery in advanced esophageal cancer. However, even in patients who achieve pCR at the primary lesion, brain metastasis may occur after surgical treatment. Preoperative and postoperative surveillance for brain metastases is necessary in patients at high risk of distant metastasis, even if the local lesion is controlled.

INTRODUCTION: Sarcomatoid carcinoma is a rare histological variant of carcinoma characterized by a mesenchymal-like morphology, often arising through the epithelial-mesenchymal transition. Although sarcomatoid carcinoma is occasionally observed in primary esophageal carcinosarcoma, its diagnosis at metastatic sites is rare.

CASE PRESENTATION: A man in his 60s was diagnosed with esophageal squamous cell carcinoma with synchronous liver and cutaneous metastases. A cutaneous nodule in the abdomen was surgically resected and pathologically confirmed to be metastatic squamous cell carcinoma. Owing to the unresectable nature of the tumor, the patient underwent 10 cycles of combined pembrolizumab and fluorouracil + cisplatin therapy, followed by 20 cycles of S-1 monotherapy owing to immune-related adverse events. This treatment resulted in a complete clinical response of the primary esophageal tumor and a significant reduction in most liver metastases. However, one liver metastasis in the left lateral segment exhibited progressive disease, requiring surgical resection. Pathological examination of the hepatic lesion revealed a well-demarcated lobulated tumor with necrosis and hemorrhage. Microscopically, the tumor comprised polygonal-to-short, spindle-shaped atypical cells with pleomorphism, frequent mitotic figures, and sinusoidal infiltration. Immunohistochemically, the hepatic lesion exhibited diffuse vimentin positivity, partial Cam5.2, and scattered INSM-1 expression, suggesting a mesenchymal transition of the carcinoma with partial neuroendocrine differentiation. CK AE1/AE3, CK5/6, and p40 were negative. p53 and BRM/SMARCA2 showed a complete loss of expression. A retrospective analysis of esophageal and cutaneous lesions revealed a progressive loss of BRM/SMARCA2 expression across different metastatic sites, supporting the hypothesis of epithelial-mesenchymal transition-driven transformation during hepatic metastasis. Despite surgical intervention, multiple hepatic recurrences were detected within 2 months postoperatively, highlighting the aggressive nature of sarcomatoid carcinoma and the limitations of surgery alone in controlling this disease.

CONCLUSIONS: This case highlights the rare phenomenon of histological transformation of squamous cell carcinoma to sarcomatoid carcinoma within a metastatic site, emphasizing the importance of surgical resection for both diagnosis and treatment. The loss of BRM/SMARCA2 may have contributed to the epithelial-mesenchymal transition-driven transformation and, together with neuroendocrine differentiation, may have played a role in the tumor’s aggressiveness. Histological reassessment of chemotherapy-resistant lesions is crucial for elucidating tumor evolution and optimizing future therapeutic strategies.

INTRODUCTION: Central airway obstruction (CAO) due to malignancy is an oncological emergency that requires prompt management. On the other hand, esophagobronchial fistula (EBF) is also a serious condition that can lead to aspiration pneumonia and difficulty with oral intake. Secondary involvement due to malignant lymphoma is a very rare cause of both CAO and EBF. Here, we report a case of esophageal bypass surgery for an EBF that became apparent after palliative irradiation for left main bronchial obstruction caused by mediastinal malignant lymphoma.

CASE PRESENTATION: A 76-year-old man was referred to our hospital with a chief complaint of dyspnea and dysphagia. Contrast-enhanced chest CT revealed a 10-cm irregularly shaped space-occupying lesion (SOL) in the middle mediastinum, involving the left main bronchus and esophagus. The left main bronchus was obstructed by the SOL, and the presence of gas within the lesion suggested the formation of a fistula between the tumor and the bronchus. Bronchoscopy revealed almost complete obstruction of the left main bronchus due to an ulcerative tumor. Upper gastrointestinal endoscopy revealed a deeply excavated esophageal ulcer, raising suspicion of a fistulous connection with the tumor. Although fistula formation between the tumor and the bronchus and/or esophagus was suspected, due to the oncological emergency of CAO presumably caused by mediastinal-type lung cancer, palliative irradiation and intravenous administration of betamethasone sodium phosphate were initiated prior to obtaining a pathological diagnosis. The tumor shrank remarkably after the treatment. However, as the tumor regressed, an EBF became apparent. Given the patient’s strong desire to resume oral intake and the need for a definitive diagnosis, we planned a surgical intervention (esophageal bypass surgery with lymph node sampling). The patient’s postoperative course was uneventful. He resumed oral intake on POD 9, and was discharged on day 20. Histopathological examination of the lymph nodes revealed diffuse large B-cell lymphoma. The patient began chemotherapy for malignant lymphoma 2 months after surgery.

CONCLUSIONS: Esophageal bypass surgery is a valuable option for patients with an EBF, as it improves QOL and enables subsequent chemotherapy.

INTRODUCTION: The optimal follow-up period and appropriate examinations for patients with esophageal squamous cell carcinoma (ESCC) who have remained disease-free for 5 years remain controversial.

CASE PRESENTATION: A 73-year-old man was diagnosed with ESCC and underwent curative esophagectomy. Pathological examination revealed a superficial tumor without lymph node metastases, despite lymphatic and vascular involvement. The patient underwent routine postoperative follow-up at our institution and showed no signs of recurrence until the 5th postoperative year. However, his serum p53 antibody titer increased 5 years postoperatively, and careful follow-up using imaging modalities, including CT, was scheduled. No lesions suspected of recurrence were noted over the next 2 years until bilateral pleural effusion was detected on CT in the 7th postoperative year. Cytological examination of the pleural effusion revealed pleural-seeded ESCC cells.

CONCLUSIONS: Although several cases of late recurrence (>5 years) have been previously reported, most have a deeper infiltration depth than that of T2 or pathologically positive lymph nodes. However, patients with lymphatic/vascular involvement, even those with pT1bN0 ESCC, require careful surveillance using imaging modalities and laboratory tests, given the possibility of late recurrence occurring beyond 5 years.

INTRODUCTION: Medullary carcinoma of the colon was formerly classified as poorly differentiated adenocarcinoma. The prognosis is relatively good, with a high degree of microsatellite instability and a predilection for the right colon.

CASE PRESENTATION: The mean age of the 4 patients was 69 years (range, 47–90 years), with 2 males and 2 females. Preoperative biopsy results showed 3 cases of moderately differentiated adenocarcinoma and 1 case of poorly differentiated adenocarcinoma. All cases were right-sided colon cancer, with 3 cases of ascending colon cancer and 1 case of transverse colon cancer. One case was classified as cT3, 2 as cT4a, and 1 as cT4b. One case was cN-negative, and 3 were cN-positive. No cases were cM-positive. The approach was laparoscopic in 3 cases and robot-assisted surgery in 1 case. Postoperative complications included postoperative ileus in 1 case. Pathological staging was Stage II in 3 cases and Stage III in 1 case. All cases showed high microsatellite instability, and no adjuvant therapy was administered. All patients remain under observation, with no recurrences identified at the time of writing.

CONCLUSIONS: Medullary carcinoma is rare but has been increasing in recent years. This pathology shows characteristic histological features and immunophenotypes, and accurate diagnosis is important because of its effect on postoperative follow-up.

INTRODUCTION: Esophageal sarcomatoid carcinoma (ESC) is a rare malignancy characterized by both carcinomatous and sarcomatous components, with no established perioperative chemotherapy standards.

CASE PRESENTATION: We report a case of advanced ESC (T3N1M0, Stage III) treated with 2 courses of neoadjuvant docetaxel, cisplatin, and fluorouracil (DCF) chemotherapy, followed by robotic-assisted thoracoscopic esophagectomy. Histopathology revealed the complete disappearance of spindle cell components, although residual squamous cell carcinoma was found in the flat mucosa. The patient remained recurrence-free for 26 months postoperatively without adjuvant therapy.

CONCLUSIONS: This case suggests that preoperative DCF chemotherapy may be effective for ESC, especially when combined with complete surgical resection, highlighting the importance of addressing both the sarcomatoid and squamous components for long-term survival.

INTRODUCTION: Multimodal treatment is required for advanced thymic epithelial tumors. However, to date, no pharmacotherapy has been established. Herein, we present 2 cases in which preoperative cisplatin, doxorubicin, and methylprednisolone (CAMP) therapies were effective.

CASE PRESENTATION: Case 1: A woman in her 70s was diagnosed with clinical stage IVA thymoma with intrathoracic dissemination. The patient received 2 cycles of induction therapy with CAMP, resulting in a partial response. Robot-assisted thoracoscopic resection of a mediastinal tumor and pleural lesions was successfully performed. Pathology revealed a type B2 thymoma (ypT1aN0M1a, stage IVA). Case 2: A woman in her 70s was diagnosed with clinical stage IIIA thymoma invading the upper lobe of the left lung. Two cycles of CAMP therapy resulted in sufficient tumor shrinkage to allow resection of only the upper division of the left lung along with resection of the thymoma, thereby avoiding lobectomy. Pathology confirmed type B2 thymoma (ypT3N0M0, stage IIIA), and adjuvant radiotherapy was administered postoperatively.

CONCLUSIONS: CAMP therapy was effective as induction chemotherapy in 2 cases of advanced thymoma, allowing for less invasive surgical approaches. Following this regimen, minimally invasive surgery was performed in one case, and the extent of pulmonary resection was reduced in the other. Therefore, this regimen may offer functional preservation and serve as a useful option for multimodal treatment.

INTRODUCTION: Mediastinal non-seminomatous germ cell tumors (NSGCTs) are rare tumors. Neoadjuvant chemotherapy followed by complete surgical resection of residual masses is recommended, and is often performed through a median sternotomy or thoracotomy with regard to the influence of induction chemotherapy and tumor size. We herein report 2 cases of mediastinal NSGCT that were surgically resected by robot-assisted thoracoscopic surgery (RATS) using the subxiphoid approach.

CASE PRESENTATION: Case 1: A 23-year-old man was diagnosed with an anterior mediastinal mass measuring 95 × 73 × 73 mm while undergoing an examination due to fever. He was diagnosed with a yolk sac tumor based on percutaneous needle biopsy. After 4 cycles of neoadjuvant chemotherapy, the patient underwent tumor resection combined with wedge resection of the left upper lung via a robot-assisted subxiphoid approach. It was particularly difficult to identify the left phrenic nerve because of stiff adhesions and thickening of the tissue. Therefore, we decided to perform dissection of tissue other than that surrounding the left phrenic nerve. Subsequently, the thymus and tumor were flipped into the left thoracic cavity, and the left phrenic nerve was easily identified and preserved from the pericardial side. The patient was discharged without any postoperative complications. Case 2: An 18-year-old man was diagnosed with a yolk sac tumor measuring 86 × 68 × 150 mm during an examination to investigate intermittent right chest pain. After 4 cycles of neoadjuvant chemotherapy, the patient underwent anterior mediastinal tumor resection via a robot-assisted subxiphoid approach. The tumor was close to the right pulmonary hilum, and the inflammation was so intense that it was difficult to identify the right phrenic nerve. In this case, indocyanine green fluorescence imaging was helpful for identifying the right phrenic nerve. The tumor was completely resected. The patient was discharged on POD 6 without any postoperative complications.

CONCLUSIONS: We report 2 cases of mediastinal NSGCT after induction chemotherapy that were completely resected using RATS. The use of techniques to accurately identify the phrenic nerve and the advantages of robot-assisted surgery via the subxiphoid approach enabled safe and minimally invasive surgical procedures.

INTRODUCTION: Reports of fistulas in the common bile duct caused by pancreatic pseudocysts have increased. However, to the best of our knowledge, no prior report has described pseudocysts filled with microbiliary stones that were difficult to differentiate from neoplastic lesions.

CASE PRESENTATION: A 64-year-old man presented with groove pancreatitis attributable to heavy alcohol consumption and a hypovascular mass in the groove area with duodenal bleeding. The lesion, which was initially considered a pancreatic groove tumor with groove pancreatitis, was treated with subtotal stomach-preserving pancreaticoduodenectomy. However, a post-surgical pathological analysis revealed that it was a pancreatic pseudocyst in the groove area containing bilirubin calcium stones and pancreatic stones.

CONCLUSIONS: Cases comprising fistula formation in the common bile duct are rare. A pancreatic pseudocyst that formed a fistula with the common bile duct was suspected in the present case. This case was unique because the pseudocyst was filled with microbiliary stones. This report highlights the difficulty in differentiating a pseudocyst filled with bilirubin calcium stones from a neoplastic lesion and underscores the importance of the accurate diagnosis and management of this rare pathology.

INTRODUCTION: The main causes of right-to-left shunting (RLS) in patients with atrial septal defect (ASD) are pulmonary hypertension, right ventricular outflow tract obstruction, severe tricuspid regurgitation, and a large ASD resulting in equal bi-atrial pressures. Reports of a case of an unintentional connection of the inferior vena cava (IVC) to the left atrium (LA) discovered many years after the repair of ASD are rare.

CASE PRESENTATION: A 47-year-old male with a history of congenital ASD repair was found to have large RLS during examination of choledocholithiasis. Details of the former surgeries, performed twice for some reason, were unknown. He had cyanosis of fingers, but neither pulmonary hypertension nor right ventricular outflow obstruction. Transesophageal echocardiography, cardiac CT, and cardiac catheterization demonstrated a direct connection between the IVC and the LA as well as a residual ASD. The patient subsequently underwent successful surgical repair. It was speculated that the eustachian valve (EV) of the IVC had been wrongly taken as the lower margin of the defect in the first repair, and then a new ASD was created in the second surgery to maintain his hemodynamics. Though he had RLS with ASD, he subsequently lived a normal life for approximately 40 years, albeit with exertional dyspnea and mild cyanosis.

CONCLUSIONS: We report on a case of iatrogenic RLS after ASD closure. The surgeon must always check the intracardiac anatomy carefully and close the ASD without using other structures such as the EV.

INTRODUCTION: Pleuroperitoneal communication (PPC) is an abnormal connection between the thoracic and abdominal cavities, allowing ascitic fluid to migrate into the pleural space and cause pleural effusion. Although PPC is a recognized complication in peritoneal dialysis patients, it is rarely reported as a postoperative complication.

CASE PRESENTATION: A 54-year-old female patient with a history of right hemicolectomy and right hepatectomy for ascending colon cancer and metastatic liver tumor developed persistent right pleural effusion 3 months postoperatively. She also had ovarian metastases, peritoneal dissemination, and malignant ascites. Despite treatment with diuretics and 2 attempts at pleurodesis, the pleural effusion persisted. A combined thoracoscopic and laparoscopic approach was performed. Intraoperatively, a diaphragmatic hernia with a pinhole defect was identified, suggesting migration of ascitic fluid into the thoracic cavity. Resection of the hernia sac and suture closure of the diaphragmatic defect were carried out. Postoperatively, the pleural effusion resolved, and her dyspnea improved.

CONCLUSIONS: In case of refractory pleural effusion following hepatectomy, PPC due to diaphragmatic defects should be considered, particularly in the presence of ascites. A combined thoracoscopic and laparoscopic approach is a viable surgical option for definitive treatment.

INTRODUCTION: Steatotic liver disease (SLD) may develop in some patients after pancreaticoduodenectomy (PD), but no cases requiring liver transplantation (LT) have been reported to date. Here, we present two cases in which LT was performed for decompensated liver cirrhosis (LC) after PD.

CASE PRESENTATION: Case 1 was a 53-year-old man with obesity, metabolic-associated SLD (MASLD), and diabetes mellitus. The patient underwent PD for an intraductal papillary mucinous neoplasm. His liver function worsened and he developed decompensated LC 6 years later, eventually requiring LT. Due to poor mobility of the jejunal limb caused by severe adhesions and the presence of a pancreatojejunostomy, a choledochojejunostomy was performed at the more distal site of the common bile duct than usual. He developed hemobilia and biliary leakage but was discharged on POD 107. Liver function has been good for 2 years after LT without MASLD recurrence, although endoscopic treatment is periodically required for biliary stricture. Case 2 was a 46-year-old man with obesity, SLD, and a history of excessive alcohol consumption. The patient underwent PD for duodenal cancer. Five years later, he developed decompensated LC, which required living-donor LT. For biliary reconstruction, a new jejunal limb was created and elevated. He was discharged on POD 79. He has repeatedly developed cholangitis, but his liver function has been good for 6 years without SLD recurrence.

CONCLUSIONS: Steatohepatitis can worsen following PD and may lead to decompensated LC, ultimately requiring LT. Therefore, screening for steatohepatitis and its risk factors prior to PD is essential, and prophylaxis should be considered. LT after PD presents surgical challenges and biliary reconstruction with some procedural modifications.

INTRODUCTION: von Hippel-Lindau (VHL) disease is an autosomal dominant hereditary disorder characterized by the development of tumor-like lesions in multiple organs. While central nervous system hemangioblastomas, pancreatic neuroendocrine tumors, and pancreatic cysts are commonly associated with VHL disease, there have been few reported cases of pancreatic hemangioblastoma in patients with VHL disease.

CASE PRESENTATION: A male patient in his 30s had been diagnosed with VHL disease and had been followed for cerebellar and spinal hemangioblastomas, and renal cell carcinoma, for which he had undergone several tumor resections, radiation therapy, and a ventriculoperitoneal shunt. A pancreatic head tumor deemed to be a neuroendocrine tumor on imaging findings exhibited a gradual increase in size from 12 to 33 mm for the past 2 years, but it had been monitored due to his comorbidities and declining daily living activities. Severe anemia was detected during his regular outpatient visit, and an emergency esophagogastroduodenoscopy revealed a submucosal tumor near the duodenal papilla with ulceration and active bleeding, making endoscopic hemostasis challenging. Dynamic contrast-enhanced CT showed active bleeding from the pancreatic tumor. Subsequently, emergency angiography was performed via the superior mesenteric artery, successfully embolizing vessels supplied by the inferior pancreaticoduodenal artery to achieve hemostasis. Due to concerns about rebleeding, we performed pancreaticoduodenectomy 1 month after the emergency angiography, during which we awaited the improvement of the patient’s overall condition. Microscopic findings of the tumor showed multinodular proliferation with hematoxylin-eosin staining, revealing cells with clear cytoplasm and abundant capillaries and dilated branching vessels within the nests. Immunohistochemical analysis demonstrated positivity for alpha-inhibin and S100, with partial positivity for carbonic anhydrase IX, leading to a diagnosis of pancreatic hemangioblastoma.

CONCLUSIONS: This paper reports a rare case of pancreatic hemangioblastoma arising in a patient with VHL disease. It is crucial to consider the possibility of pancreatic hemangioblastoma when treating pancreatic tumors in VHL disease patients.

INTRODUCTION: Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm that most commonly originates in the pleura but can also occur at extrapleural sites, including the abdominal cavity. Among these, primary SFT of the stomach is exceptionally rare. Due to overlapping clinical, endoscopic, and radiologic characteristics, distinguishing SFT from gastrointestinal stromal tumor (GIST) can be particularly challenging. Misdiagnosis may result in inappropriate treatment, such as unnecessary administration of imatinib. Therefore, accurate preoperative differentiation is essential for appropriate management.

CASE PRESENTATION: A 74-year-old man was incidentally found to have a submucosal gastric tumor during a routine health checkup and was referred to our hospital for further evaluation and treatment. Upper gastrointestinal endoscopy revealed a 30-mm subepithelial lesion on the greater curvature of the gastric fundus. Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) demonstrated isolated and clustered cells with ovoid to spindle-shaped nuclei. Although not definitive, the combined endoscopic and cytological findings led to a preoperative diagnosis of suspected GIST, and laparoscopy and endoscopy cooperative surgery (LECS) was subsequently performed for local resection. Histopathological examination of the resected specimen revealed an irregular proliferation of spindle cells and nuclear immunopositivity for signal transducer and activator of transcription 6 (STAT6), leading to a final diagnosis of SFT of the stomach. According to Demicco’s risk stratification model, the tumor was classified as low risk. The patient underwent complete resection via LECS and has remained free of recurrence for more than 2.5 years postoperatively.

CONCLUSIONS: This case highlights the difficulty in differentiating SFT from GIST preoperatively and underscores the importance of obtaining sufficient tissue samples to allow for immunohistochemical analysis, particularly STAT6 staining. Recognizing gastric SFT as part of the differential diagnosis is critical to avoid misdiagnosis and ensure appropriate therapeutic decision-making.

INTRODUCTION: Glycogen storage disease type IX (GSD type IX) is caused by a deficiency in phosphorylase b kinase (PHK) and is classified into hepatic (IXa–c) and muscular (IXd) subtypes. GSD type IXd leads to exercise intolerance, rhabdomyolysis, and myoglobinuria owing to impaired glycogen breakdown. It is a rare and mild metabolic disorder, with only 19 reported cases of PHKA1 mutations. To the best of our knowledge, this is the 1st report on the perioperative management of a patient with GSD type IXd.

CASE PRESENTATION: A 61-year-old male presented with a right inguinal hernia requiring surgical repair. He had experienced muscle weakness since the age of 53, which progressed to severe neck muscle atrophy by the age of 58. Genetic testing confirmed a PHKA1 mutation, leading to the diagnosis of GSD type IXd. He had previously undergone multiple surgeries without any complications. Given his underlying muscle weakness, totally extraperitoneal (TEP) inguinal hernia repair was performed to minimize postoperative pain and muscle damage. Postoperative monitoring revealed no rhabdomyolysis or myoglobinuria, and the patient was discharged without complications on POD 7.

CONCLUSIONS: We successfully managed a patient with GSD type IXd perioperatively, without complications. Although this disease can cause rhabdomyolysis, the symptoms are often mild and may remain undiagnosed. Therefore, in patients with muscle weakness or elevated creatine kinase levels, careful surgical planning and perioperative monitoring are essential.

INTRODUCTION: Thoracic vertebral fractures are commonly associated with aortic injury at the time of injury. However, delayed thoracic aortic injury during intensive care management of thoracic fractures has not been reported.

CASE PRESENTATION: An 83-year-old man was transported to our hospital after a collision with a car while riding a motorized bicycle. A fracture of the 10th thoracic vertebra was identified and treated with thoracic fusion on day 24. On day 34, a new pseudoaneurysm was found in contact with the vertebral fracture. Thoracic endovascular aortic repair (TEVAR) was performed on day 36, and disappearance of the pseudoaneurysm was confirmed on day 38.

CONCLUSIONS: The possibility of delayed thoracic aortic pseudoaneurysm should be considered when thoracic vertebral fractures are near the descending aorta. Early TEVAR may be effective for delayed aortic injury.

INTRODUCTION: There are no reports of patients undergoing McKeown esophagectomy for esophageal cancer after undergoing pancreaticoduodenectomy for pancreatic cancer. We report the case of a patient who underwent subtotal esophagectomy and colon reconstruction after pancreaticoduodenectomy using the mesenteric approach.

CASE PRESENTATION: A 71-year-old male was diagnosed with advanced esophageal cancer. Four years prior to diagnosis, he underwent subtotal stomach-preserving pancreaticoduodenectomy using the mesenteric approach for pancreatic surgery, followed by Child’s reconstruction surgery. After undergoing 3 cycles of neoadjuvant chemotherapy with docetaxel, cisplatin, and 5-fluorouracil, the patient was scheduled for a subtotal esophagectomy. The middle colic artery was transected using the mesenteric approach, and the upper jejunum was utilized for Child’s reconstruction surgery. A 2-stage procedure involving McKeown esophagectomy and left-sided colon reconstruction was planned. The 1st stage of the procedure involved robot-assisted subtotal esophagostomy in the prone position, followed by cervical esophagostomy and gastrostomy. The patient underwent the 2nd stage of the surgery after approximately 1 month of parenteral nutrition via a gastrostomy tube. The transverse colon was mobilized and transected at the hepatic flexure. The left side of the mesocolon, which is fed by the left colic artery, was then pulled up through the antethoracic route. The right internal thoracic artery and vein were anastomosed to the marginal artery and vein of the transverse colon, respectively, for supercharge and superdrainage. Reconstruction involved esophago-colonic and colonic–gastric anastomoses. The patient was discharged without postoperative complications, and no signs of recurrence were observed at the 2-year postoperative follow-up.

CONCLUSIONS: Subtotal esophagectomy for esophageal cancer after subtotal stomach-preserving pancreaticoduodenectomy using a mesenteric approach and colon reconstruction can be safely performed in 2 stages. The optimization of pancreaticoduodenectomy for pancreatic cancer could improve the long-term survival of patients with 2nd primary esophageal cancer, for which radical esophagectomy is necessary.

INTRODUCTION: Osler–Weber–Rendu syndrome, or hereditary hemorrhagic telangiectasia (HHT), is a rare autosomal dominant genetic vascular disease characterized by arteriovenous malformations, vascular wall fragility, dilatation, and rupture of the vessels with hepatic symptoms. As HHT with hepatic symptoms is recognized as the primary etiology for liver transplantation, liver transplantation with liver grafts from donors affected by HHT is extremely rare. Herein, we report a successful liver transplantation in a patient with biliary atresia who received a whole-liver graft from a young brain-dead donor with HHT.

CASE PRESENTATION: The patient was a 15-year-old girl with decompensated liver cirrhosis who underwent Kasai surgery for biliary atresia at 3 months of age. The donor was a female in her teens, diagnosed with brain death due to cerebral hemorrhage. Although the donor was diagnosed with Osler disease, she had no hepatic symptoms and normal liver function. CT did not reveal any apparent vascular malformations in the liver. A whole-liver transplant was performed using the donated liver. The patient recovered well in terms of liver function, without any hepatic-related symptoms.

CONCLUSIONS: Our experience with this patient may have important implications for liver transplantations from donors with HHT.

INTRODUCTION: Brain metastasis from gastric cancer is rare (0.5%) and often occurs with metastasis to other organs. We herein describe a very rare patient with a solitary brain metastasis from residual gastric cancer with no metastasis to other organs.

CASE PRESENTATION: The patient was an 82-year-old man who visited a previous institution for anemia. Upper gastrointestinal endoscopy revealed a type 2 tumor extending from the esophagogastric junction to the upper part of the residual gastric body. Biopsy revealed tubular differentiated adenocarcinoma, and he was referred to our institution. He had a history of distal gastrectomy for a gastric ulcer in his 30s. After contrast-enhanced CT, we diagnosed residual gastric cancer (cT4aN + M0 cStage III). After three courses of preoperative chemotherapy with S-1 plus oxaliplatin, the patient underwent open total resection of the residual stomach, lower esophagectomy, D2 dissection, and Roux-en-Y reconstruction and was discharged without postoperative complications. Six months after surgery, thoracic and abdominal contrast-enhanced CT showed no apparent recurrence. However, 1 month later, he began to experience speech difficulties and mobility issues, and head CT revealed a 3-cm tumor in the left frontal lobe. After whole-body contrast-enhanced CT and PET-CT, the brain tumor was confirmed as a solitary lesion with no metastasis to other organs. The patient underwent open brain tumor resection, and pathology diagnosed brain metastasis from residual gastric cancer. Postoperatively, he underwent radiation therapy (40 Gy in 8 fractions) to the tumor cavity. At the time of writing, 24 months have passed since the gastrectomy and 16 months have passed since the removal of the brain tumor, with no significant neurological damage or other evidence of distant metastasis.

CONCLUSIONS: We experienced an extremely rare case of a solitary brain metastasis after residual gastric cancer surgery. Our findings suggest that aggressive local treatments for brain metastasis, including surgical resection and radiotherapy, may contribute to improvements in symptoms and prognosis.

INTRODUCTION: Liver transplantation for polycystic liver disease (PLD) poses significant intraoperative risks due to the presence of a massively enlarged liver. We report a rare case of intraoperative pneumothorax and pneumatocele formation during total hepatectomy, which was successfully managed with a non-operative approach.

CASE PRESENTATION: A female patient in her 40s with a history of autosomal dominant polycystic kidney disease presented with progressive liver cyst enlargement (Gigot type III, Qian classification Grade 4), which led to decreased activities of daily living and intracystic hemorrhage. The patient underwent a deceased-donor liver transplantation. During mobilization of the liver from the right side of the diaphragm, the patient experienced sudden onset of pneumothorax. Incision of the diaphragm revealed a cystic structure containing a hematoma, suggesting pneumatocele formation. The pneumatocele was not resected during the ongoing operation; instead, thoracic drainage was performed as the primary intervention. Postoperatively, no air leakage was observed, and the thoracic drain was successfully removed on POD 12. The pneumatocele, which measured approximately 10 × 10 × 7 cm, showed no signs of infection, and was monitored without additional surgical intervention. On POD 19, a fever prompted further evaluation, and CT-guided cyst aspiration for culture was performed, which revealed no evidence of infection. Acute T-cell-mediated rejection was observed on POD 27, and a steroid pulse was administered, but even after that, the pneumatocele gradually decreased in size without any signs of infection.

CONCLUSIONS: A pneumatocele is an uncommon but important consideration during liver transplantation for PLD, potentially resulting from barotrauma related to abrupt changes in intrathoracic pressure during hepatectomy and mechanical ventilation. Considering the risk of infection in immunosuppressed patients, close monitoring is essential. On the contrary, surgical resection also carries the risk of pulmonary or bronchial fistulae; therefore, careful consideration is required. This case demonstrates that non-operative management with careful observation can be an effective strategy in selected patients.

INTRODUCTION: Prosthetic valve endocarditis following aortic root replacement (ARR) typically necessitates redo-ARR, which involves complete graft removal, extensive aortic root dissection, and coronary reimplantation. This highly invasive procedure carries substantial surgical risk, including high operative mortality. In select high-risk patients without evidence of prosthetic graft infection, alternative surgical strategies may reduce procedural complexity and improve outcomes.

CASE PRESENTATION: Here, we report 3 cases of prosthetic valve endocarditis following the Bentall procedure, a common ARR technique, in older patients (mean age: 73.7 ± 3.5 years). All preoperative blood cultures were negative, and no signs of prosthetic graft infection were noted on CT. Due to advanced frailty (Clinical Frailty Scale scores of 7 or 8), conventional redo-ARR was deemed prohibitively high-risk. Risk assessment using the JapanSCORE showed a mean predicted mortality of 32.5% ± 21.0%, with combined mortality and morbidity of 63.7% ± 22.9%. Instead of redo-ARR, annular reconstruction using a bovine pericardial patch was performed, followed by redo aortic valve replacement. All patients underwent successful surgery with no postoperative reinfection. One patient required prolonged intensive care and was transferred to another facility for rehabilitation, while the other 2 recovered uneventfully and were discharged. During a mean follow-up of 26.3 ± 17.6 months, 2 patients died due to non-cardiac causes: one from pneumonia and the other from gastric cancer.

CONCLUSIONS: In high-risk patients without clear evidence of prosthetic graft infection, aortic valve replacement with annular patch reconstruction may represent a viable alternative to redo-ARR, particularly in settings where homografts are not readily available. This approach reduces operative complexity while maintaining structural integrity. Further studies are warranted to validate infection control criteria and assess long-term outcomes.

INTRODUCTION: Pulmonary smooth muscle hyperplasia (SMH) is a rare benign tumor that presents CT imaging findings that require differentiation from those of primary lung cancer.

CASE PRESENTATION: The postoperative follow-up chest CT for gastric cancer in a 76-year-old Japanese man revealed an abnormal shadow. A 2.2-cm nodule with an unclear border and showing a tendency to grow was detected in the right lower lobe (S6), and suspected infiltration into the right upper lobe (S2). PET showed minimal accumulation of 18F-fluorodeoxyglucose in the nodule, with a maximum standardized uptake value of 1.0. A transbronchial lung biopsy showed no malignant findings. Due to the tumor’s progressive growth, surgical resection was performed. Intraoperatively, a tumor located in S6 with suspected partial invasion into S2 was observed, and a wedge resection from S6 to S2 was thus performed. A frozen section of the resected specimen revealed irregularly distributed atypical cells forming mildly irregular glandular structures, leading to a diagnosis of “suspected adenocarcinoma.” Robotic-assisted thoracoscopic surgery for a right S6 segmentectomy with combined wedge resection of S2 was performed. However, the final histopathological examination revealed spindle-shaped smooth muscle cells’ proliferation. The immunohistochemical analysis revealed positivity for α-SMA, desmin, and h-caldesmon, leading to a diagnosis of pulmonary SMH.

CONCLUSIONS: SMH is an extremely rare benign disease that can mimic lung cancer and may be considered among the possible differential diagnoses of solitary pulmonary nodules. A careful treatment strategy, including the choice of surgical procedure, is recommended to minimize the possibility of overtreatment.

INTRODUCTION: Abdominal compartment syndrome (ACS) is a serious complication that can occur after endovascular aneurysm repair (EVAR) for ruptured abdominal aortic aneurysm (rAAA). Prompt recognition and appropriate management are crucial to improve patient outcomes.

CASE PRESENTATION: An octogenarian with an 11-cm rAAA underwent emergent EVAR due to cardiovascular instability. Postoperatively, the patient developed ACS, necessitating open abdominal management (OAM) due to intestinal edema and retroperitoneal hematoma. Despite multiple surgical interventions, including aneurysmorrhaphy and removal of retroperitoneal hematoma, the patient experienced prolonged difficulty in abdominal closure. The following procedures were attempted for abdominal closure: 1) Dissection of skin and subcutaneous tissues from the rectus sheath on both sides. 2) Release of the external oblique muscle from the anterior layer of the rectus by longitudinally cutting the superficial fascia. 3) Closure of both rectus sheaths with horizontal mattress sutures and negative pressure wound therapy (NPWT). On POD 20, fluid accumulation from bleeding complicated abdominal closure. However, the abdominal wall was successfully closed by achieving hemostasis and using NPWT. Despite these efforts, the patient developed multiple organ failure, including respiratory and renal failure. Sepsis ultimately led to the patient's death on the 80th POD.

CONCLUSIONS: ACS following EVAR for rAAA significantly impacts patient prognosis. Specific techniques for abdominal closure, as described in this case, may help minimize the duration of OAM in challenging cases.