Purpose: Crohn’s disease (CD) exists in an environment of chronic intestinal inflammation, but rarely develops into cancer. In Japan, cancer associated with CD is usually reported in the anorectal region, while small intestinal cancer is uncommon. Herein, we review small intestinal cancer cases associated with CD from our hospital and other reports in Japan. Materials and Methods: The subjects were 43 patients (52 lesions), including 17 who underwent surgery at our hospital between January 2011 and December 2022, and 26 cases reported elsewhere. The clinicopathological characteristics, diagnosis, and treatment were examined. Result: There were 31 males and 12 females, the mean age at the time of cancer diagnosis was 49 years, and the average time after onset of CD was 18.0 years. The CD type was small intestinal (n=13 cases), small and large intestinal (n=26), and unknown (n=4). Surgical indications were stricture (n=27 cases), fistula (n=11), cancer (n=6), and perforation (n=3). Six cases were diagnosed with small intestinal cancer preoperatively, 4 were diagnosed intraoperatively, and 33 were diagnosed by postoperative pathological examination. One lesion of small intestinal cancer was found in the jejunum, with all others in the ileum. The most common macroscopic lesion was type 5 (n=11 lesions), followed by types 0 and 3 (n=5 each), and types 1, 2 and 4 (n=1 each); with 28 lesions of unknown type. The histological types included well-differentiated adenocarcinoma (n=26 lesions), moderately differentiated adenocarcinoma (n=14), poorly differentiated adenocarcinoma (n=6), signet ring cell carcinoma (n=4), and mucinous carcinoma (n=2). The depth of invasion was pTis+T1 (n=10 cases), pT2 (n=9), pT3 (n=14), and pT4 (n=15). Lymph node metastasis was observed in 3/20 cases, and distant metastasis in 5/38 cases. The 5-year survival rate was 63.8%. Conclusion: Small intestinal cancer associated with CD is difficult to diagnose preoperatively. Thus, in most cases, surgery was performed as a common Crohn’s disease-related stricture or fistula.

Thrombotic microangiopathy (TMA) is a life-threatening syndrome that is characterized by hemolytic anemia, thrombocytopenia, and microvascular thrombosis, and results in severe organ damage. We report a case of cancer-related TMA secondary to gastric cancer. The patient was a man aged 73 who was brought to the emergency department with severe diarrhea. He was diagnosed with TMA induced by gastric cancer based on clinical manifestations, laboratory information and imaging findings. Open total gastrectomy was conducted for gastric cancer with hemodialysis and plasma exchange. After the operation, TMA symptoms were improved, and hemodialysis and plasma exchange were withdrawn. However, the patient experienced protracted fistula healing of esophagojejunal anastomotic leakage, resulting in severe esophagitis and obstruction, and died 7 months after surgery due to aspiration pneumonia. This case shows that surgery for a primary tumor with cancer-related TMA can improve TMA symptoms, but complications such as protracted fistula healing should be considered. There is a need to recognize the characteristics of TMA and determine the treatment strategy, including the surgical indication.

A 71-year-old woman was found to have a 7-cm hepatic cyst with a mural nodule in segment 7 in 2018. She was referred to our department due to an increase in the tumor marker CA19-9 and enlargement of the mural nodule on the cyst wall. At this point, the serum CA19-9 level was elevated to 740.2 U/ml. CT and US showed a hepatic cyst with an enhancing mural nodule on the wall. The cyst was suspected to be a hemorrhagic hepatic cyst or a mucinous cystic neoplasm (MCN) of the liver. Extended right posterior sectionectomy was performed, and a pathological examination ultimately diagnosed a hemorrhagic hepatic cyst. The CA19-9 level normalized immediately after the operation. MCN of the liver is a rare disease, and complete resection can result in a good prognosis. However, cases with invasion or dissemination have a poor prognosis. Hemorrhagic hepatic cysts are often similar to MCN in imaging, making preoperative differentiation difficult. Hepatectomy can be considered as a diagnostic and therapeutic option when malignancy cannot be ruled out.

Hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular origin tumor. About one-third of patients with HEHE have synchronous distant metastases at the time of diagnosis, but there have been few reports of cases with sternal metastasis. The patient was an 80-year-old woman who was admitted with an asymptomatic liver tumor that was incidentally detected on coronary CT. The tumor was 80 mm in diameter with an enhanced margin and was located in the lateral segment of the liver. FDG-PET/CT showed increased FDG uptake in the liver tumor and sternum. The bone lesion was diagnosed as EHE by sternal biopsy, while the liver tumor was suspected to be a resectable intrahepatic cholangiocarcinoma. Laparoscopic left hemi-hepatectomy was performed. Histopathologically, the liver tumor was consistent with the pathology of the sternum lesion, as well as that of a lymph node in the hepatoduodenal ligament that was resected for sampling during surgery. Therefore, the final diagnosis was HEHE with sternal and lymph node metastases. The patient was discharged on the 8th postoperative day. As of 10 months since the operation, there has been no recurrence. Our experience suggests that FDG-PET/CT may be useful for preoperative evaluation for HEHE because distant metastases of HEHE are not unusual and target organs are diverse.

A 19-year-old male was admitted to an emergency department with a chief complaint of abdominal pain. A contrast-enhanced CT scan revealed a 30-mm hypovascular tumorous lesion in the pancreatic body. Solid pseudopapillary neoplasm (SPN) was suspected by endoscopic ultrasound-fine needle aspiration, and the patient was referred to our department for surgical intervention. We performed a central pancreatectomy to conserve the pancreatic function. Histopathologically, the tumor was diagnosed as SPN. The patient was discharged from hospital at 19 days postoperatively without complications. Twelve months after surgery, there has been no recurrence and no evidence of abnormal glucose tolerance. The occurrence of SPN in a young male is exceedingly uncommon, and thus, we present this case as a rare example of this condition.

Littoral cell angioma (LCA) is a rare disease with a splenic origin that is difficult to diagnose preoperatively. A 58-year-old woman presented to our hospital after an ultrasound at a medical checkup revealed splenomegaly and multiple nodules. Abdominal CT and MRI showed malignant lymphoma, hemangioma, and granulomatous lesions as differential diagnoses, but did not lead to a definitive diagnosis. Laparoscopic splenectomy was performed for diagnostic and therapeutic purposes. Intraoperative findings revealed no splenic surface and no evidence of distant metastasis. Histopathological examination showed LCA of the spleen. The patient has been followed up since the operation and there has been no recurrence. We report this case as a rare example of LCA of the spleen and accessory spleen.

A 56-year-old man presented with fever and abdominal pain. Blood tests revealed pancytopenia, and abdominal CT showed a massive splenomegaly with a length of approximately 30 cm extending from the upper abdomen to the pelvis. Bone marrow puncture indicated myelofibrosis and a JAK2 gene mutation, leading to a diagnosis of primary myelofibrosis. The patient underwent splenectomy for improvement of pancytopenia and relief of abdominal distention. On the day before surgery, we embolized the upper and lower branches of the splenic artery and performed an open splenectomy. The volume of intraoperative blood loss was 30 ml, and the resected spleen weighed 3,020 g. The postoperative course was favorable and the patient was discharged on the 9th postoperative day. This case shows that preoperative splenic artery embolization for massive splenomegaly is useful to reduce the risk of intraoperative bleeding and improve the surgical field around the spleen by decreasing splenic tension.

A 55-year-old male presented with lower abdominal pain three days after undergoing an upper gastrointestinal series. The patient was diagnosed with acute generalized peritonitis secondary to sigmoid colon perforation, and a Hartmann’s operation was performed. Postoperatively, despite administration of antibiotics and drainage of an intra-abdominal abscess, the patient had persistent fever and a prolonged inflammatory response. Peritonitis associated with intraperitoneal residual barium was suspected, prompting initiation of steroid pulse therapy, which reduced the inflammatory response. On postoperative day 198, the patient underwent a colostomy reversal without complications. This case suggests that in patients with persistent inflammation despite good infection control, the possibility of peritonitis due to a foreign body reaction should be considered. Steroid pulse therapy may be an effective treatment option for managing prolonged peritonitis associated with intraperitoneal residual barium.