Background: It has been generally accepted while leiomyoma is generally assumed to be the predominant esophageal submucosal tumor, the incidence of esophageal gastrointestinal stromal tumor (GIST) appears to be increasing, particularly following the standardization of GIST diagnosis. Methods: We retrospectively reviewed 13 cases of patients undergoing esophageal submucosal tumor resection between 1997 and 2008, then evaluated the GIST incidence after immunohistochemical rediagnosis. We also looked at the post-enucleation outcome. Results: Among our 13 subjects, initially diagnosed with GIST, 3 (23%) were, 6 with leiomyoma, 2 with leiomyosarcoma, and 1 each malignant lymphoma or granular cell tumor. Following histochemical reevaluation, 2 initially diagnosed with leiomyoma were rediagnosed with GIST, 5 (38%) of the 13 were confirmed to have GIST, 4 to have leiomyoma, 2 to have leiomyosarcoma, and 1 each to have malignant lymphoma or granular cell tumor. Among the 5 with GIST, 4 patients underwent tumor enucleation alone, and had no recurrence. We looked at the post-enucleation outcome in these 4 cases and in 12 other reported cases. Post-enucleation, recurrence occurred in 4 of these 16 patients (25%), but none in those with tumors of less than 4 cm and classified in the very low or low-risk group. Conclusions: Our results indicated that esophageal GIST may be initially misdiagnosed as leiomyoma, meaning that the incidence of GIST is actually not rare among esophageal submucosal tumors. Enucleation for esophageal GIST is recommended in patients whose tumors are small. Careful follow-up is required due to the high possibility of recurrence in cases with high mitosis and a high MIB-1 index.

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A 62-year-old man undergoing endoscopic mucosal resection (EMR) for gastric adenoma followed by annual upper gastrointestinal endoscopy was found in a blood test to have CEA of 75 ng/dl. A diagnosis of gastric cancer based on signet ring cell carcinoma detected by an EMR scar biopsy and scheduled surgery was halted, however, when he suddenly developed cervical pain, shoulder pain, and headache, necessitating emergency surgery following a complex epileptic seizure. Spinal fluid test on admission showed elevated intrathecal pressure higher than exceeding 35 cmH2O. Decompression dramatically relieved symptoms. Signet ring cells were detected in the spinal fluid, yielding a diagnosed of carcinomatous meningitis with increased intracranial pressure, necessitating subdural-peritoneal lumbar shunting. Following chemotherapy with TS-1 and CDDP, he returned to regular life. About 2 months after discharge, however, he was found lying in the bathroom at 06: 00 and taken to hospital by ambulance. He had reportedly taken his usual morning and evening walk the day before admission. He did not regain consciousness and died 1 week after rehospitalization. His intrathecal pressure was normal and the shunt tube presented no apparent problem.

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Primary double cancers of the duodenum and pancreas are rare, and we found only one case described previously when we surveyed the literature. We report the case of a 61-year-old man with primary double cancers of the duodenal 3^rd portion and pancreatic body. Abdominal CT showed a duodenal tumor and a separate tumor in the pancreatic body, necessitating a pancreatoduodenectomy. Histologically, the duodenal tumor was adenocarcinoma and the pancreatic tumor was invassive ductal carcinoma. These tumors were not continuous macroscopically, in pathological examination. Cytokeratin 7 found on immunohistological examination showed that the duodenal cancer origin was differed from the pancreatic cancer. Based on these findings, the definitive diagnosis was synchronous double cancers of the duodenum and pancreas. This is, to our knowledge, the first such confirmed case confirmed in immunohistological examination with Cytokeratin 7.

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We report a peculiar case of portal vein thrombus in a 50-year-old woman who underwent upper gastric resection and splenectomy for intractable esophagogastric varices with cirrhosis in July 2004 and then was hospitalized in December 2004 due to consciousness disturbance, melena, evidencing symptoms of shock, marked liver disorder and anemia. Enhanced computed tomography indicated widespread portal vein thrombus from the right branch to superior mesenteric vein together with multiple hemorrhagic erosions of the remaining stomach found in upper gastrointestinal endoscopy. Based on a diagnosis of acute liver failure and upper gastrointestinal bleeding caused by portal vein thrombus, we started intensive treatment without thrombolytic therapy because gastric hemorrhaging was difficult to control endoscopically. One week later, the thrombus diminished and her general condition gradually improved. Seven weeks later, the thrombus had disappeared and she has had no recurrence. It is very rare for a portal vein thrombus causing acute liver failure to disappear so quickly and of its own accord.

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We report a rare case of cholangiocellular carcinoma associated with Wilson's disease (WD). A 39-year-old man diagnosed with WD at age 11 and administrated a copper chelating agent was hospitalized for further examination and treatment at age 39 after a tumorous lesion 40 mm in diameter was found. A series of imaging tests and percutaneous needle biopsy indicated well-differentiated adenocarcinoma. Following percutaneous transhepatic portal embolization, he underwent right hepatic lobectomy and lymph node dissection. The definitive histopathological diagnosis was moderately-differentiated cholangiocellur carcinoma with tumor formation.

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We report an extremely rare case of a primary angiomyolipoma arising in the spleen. A 80-year-old woman diagnosed with gastric adenocarcinoma was found in abdominal computed tomography (CT) to have an irregular 14-cm mass in the left upper quadrant with potential infiltration into the pancreas. She had no kidney or liver abnormalities and no clinical evidence of tuberous sclerosis. Abdominal angiography showed that the mass corresponded to a hypervascular tumor in the spleen, radiologically interpreted as malignant tumor and necessitating splenectomy. The resected 14×12×9-cm spleen containing the tumor weighed 444 g and contained yellow nodules and irregular cysts in the splenic parenchyma without necrosis or bleeding foci. Histopathologically, the tumor consisted of mature adipose tissue, dilated blood vessels associated with wall thickening, and atypical smooth muscle cells associated with marked morphological pleomorphism. Immunohistochemically, tumor cells were positive for HMB-45, α-SMA, and desmin and negative for CD68. The Ki67/MIB1 labeling index was 1-3%, yielding a definitive diagnosis of angiomyolipoma arising in the spleen based on histological and immunohistochemical findings. Only one case of angiomyolipoma arising in the spleen has been reported in the global literature.

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A 73-year-old man with acute abdomen was found in abdominal enhanced Computed Tomography (CT) to have a thrombosis of the SMV. Because of a lack of symptomatic intestinal necrosis, we started systemic thrombolytic therapy with heparin and urokinase. Successful thrombolytic therapy, ileus and abscess formation due to cicatricial ileum stenosis and ileum penetration were occurred. Ileus and the abscess were controlled with long-tube decompression and antibiotic administration. We then conducted partial intestine and sigmoid colon resection. The postoperative course was good and no recurrence was seen in subsequent warfalin therapy. SMV thrombosis is comparatively rare disease, but sometimes ileum stenosis may occur in conservative therapy like our case. Our case comorbid abscess formation, but we controlled ileus and infection conservatively to conduct elective surgery.

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We report a case of appendiceal adenoendocrine cell carcinoma. A 75-year-old man reporting fever and general fatigue consulted a general practitioner was referred to our hospital and found to have a tumor without tenderness in the right lower abdomen. He was found in abdominal computed tomography (CT) to have appendiceal swelling with wall thickening and two liver tumors with low-density contrast were detected at S7 and S8, yielding a diagnosis of appendiceal cancer with suspected multiple liver abscesses, necessitating ileocecal resection. The resected 90×52 mm appendiceal tumor showed a rough villous pattern with no internal mucus retention. Pathological findings showed partial neuroendocrine differentiation in ordinarily differentiated adenocarcinoma. Immunohistochemically, tumors positively expressed synaptophysin. The primary appendiceal tumor was diagnosed as adenoendocrine cell carcinoma and liver tumors were diagnosed as abscesses due to their disappearance postoperatively. Such adenoendocrine cell carcinoma of the appendix is rare and only 5 cases have been reported in the literature in Japan.

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A 34-year-old man with anal and cutaneointestinal fistulas since summer 2007 referred in late January 2008 was found in gastrointestinal tract examination to have the strictures of the anal canal and sigmoid and ascending colon (hepatic flexure). Diagnosed with Crohn's disease, he underwent Hartmann's operation involving sigmoid and ascending colon resection, simoid-percutaneous fistulectomy and descending colon colostomy in late February 2008. His postoperative course was uneventful. Postoperative pathological examination showed adenocarcinoma in the resected sigmoid colon and dysplasias in the sigmoid and ascending colon. Given the man's age and rather short symptom duration, he may have had colitis-associated colon cancer possibly due to latent long-term Crohn's disease well before symptom onset.

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We report a case of cecal cancer with a large abdominal wall abscess communicating with the colon at several uncancerous sites. A 74-year-old man with appetite loss and abdominal pain to have a very large abdominal wall abscess and cecal wall thickening was found in computed tomography (CT), yielding a diagnosis of abdominal wall abscess due to cecal cancer. After abscess drainage and general improvement in the man's condition, we conducted right hemicolectomy with lymph node dissection but left the abdominal wall abscess as is due to its size. Pathologic findings indicated moderately differentiated adenocarcinoma of the cecum (pT4, pN2, sH0, sP0, sM0, fStage IIIb). In abdominal wall treatment, colon cancer should be considered as a potential underlying cause. CT is very useful in diagnosing abdominal wall abscess due to colon cancer. Cytology at abscess drainage, however, appears to be unreliable.

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We report a case of rectal GIST penetration associated with Fournier's gangrene during imatinib administration. A 59-year-old man reporting anal pain and constipation was found in computed tomography (CT) to have a 12-cm mass in the pelvic cavity diagnosed by percutaneous needle biopsy as a rectal gastrointestinal stromal tumor (GIST). Two months after treatment was started with imatinib of 400 mg/day, the tumor had shrunk to 7 cm, but the man was admitted four months later due to gluteal pain. Digital examination at admission showed no abnormality except for tenderness at the tumor site. CT on day 4 after admission showed the tumor had shrunk to 5 cm but a gas shadow was found surrounding the rectum, suggesting rectal GIST penetration. The next day, the man was found to have scrotal swelling and tenderness of the perineum, followed by redness associated with crepitations in the groin spreading to the abdominal wall, leading to a diagnosis of Fournier's gangrene. We immediately removed necrotic tissue and conducted incision and drainage. The man's subsequent course was good and he was discharged on postoperative day 58. During GIST treatment with imatinib, consideration should be given for possible perforation of the gastrointestinal tract due to imatinib's dramatic antitumor effect.

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We report a case of early rectal cancer with malignant peritoneal mesothelioma. A 87-year-old man admitted for severe constipation and abdominal fullness was found in colonoscopy to have a hemicircumferential type II tumor in the lower rectum, diagnosed as well differentiated adenocarcinoma. Barium enema showed rectal stenosis for about 10 cm induced by wall thickening. Based on a diagnosis of diffuse infiltrative rectal cancer, we conducted abdominoperineal resection of the rectum. Intraoperative findings showed slight yellow ascites and severe thickening of the mesorectum. Histopathologically, the rectal lesion was early rectal cancer, but the mesorectal lesion suspected of invasion of rectal cancer was malignant mesothelioma with metastatic lymph nodes. Differential diagnosis between peritoneal invasion of diffuse infiltrative rectal cancer is very difficult. We must keep malignant peritoneal mesothelioma in mind when finding peritoneal thickening.

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A 40-year-old man admitted for multiple pelvic bone fractures and undergoing exploratory laparotomy twice for suspect intraperitoneal hemorrhage and bowel perforation suffered small bowel obstruction. 3 days after the second laparotomy, small bowel obstruction was necessitating long tube insertion. 6 days after the second laparotomy, this conservative therapy failed, necessitating surgery. Dense adhesions found involving the jejunum required about 1 meter of small bowel to be resected. Histologically, the resected specimen showed ossification within the fibrous septa and thickening such as heterotopic ossification of soft tissue, definitively diagnosed as heterotopic mesenteric ossification. Postoperatively, the man was treated for heterotopic ossification of soft tissue using NSAIDs and cimetidine to prevent recurrence. He was remained disease free in the three years since surgery.

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We report a case of pediatric idiopathic intraabdominal hemorrhage due to anomalous congenital band originated from the mesenterium. A 14-year-old male admitted with severe lower abdominal pain was found in computed tomography (CT) to have ascites in the pelvic space and an appendix that was not identifiable. Emergency laparoscopy found mild appendicitis and bloody ascites. Abnormal string structure between the ileal mesenterium and the cecum thought to have caused abdominal bleeding necessitated appendectomy and resection of the string. The patient was discharged on postoperative day 8. Histopathological examination showed blood vessels and nerve plexi, which indicated anomalous congenital band. We report this case due to the rarity of pediatric idiopathic intraabdominal bleeding caused by anomalous congenital band.

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A 72-year-old woman admitted for right inguinal hernia and found in abdominal computed tomography (CT) to have an incarcerated right femoral hernia containing the vermiform appendix in the hernia sac could not be aided manually. Because past of a long time from appearance of disease and suggestive of gangrenous appendicitis, so we required surgery. We first exfoliated the hernia sac and repaired the hernia with mesh in the preperitoneal space using totally extraperitoneal preperitoneal repair (TEPP), followed by laparoscopic appendectomy. She had no complications and was discharged on postoperative day4. It is extremely rare case for the appendix to be incarcerated in a femoral hernia.

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We report a case of spontaneous splenic rupture secondary to metastatic choriocarcinoma. A 34-year old woman confirmed with a positive pregnancy test and referred for sudden severe abdominal pain. Initially, it was diagnosed with intraabdominal hemorrhage due to ruptured ectopic pregnancy. Physical examination on admission showed shock with severe abdominal distension. Abdominal computed tomography (CT) showed massive free fluid collection in the upper and lower abdominal cavity. Chest CT showed multiple nodules in both lung fields, suspected to be malignant tumor metastasis and necessitating surgery. That comfirmed spontaneous splenic rupture due to metastatic choriocarcinoma, pathologically confirmed to be metastatic choriocarcinoma. Her postoperative splenectomy course was uneventful. Although this condition is rare, it should be borne in mind that spontaneous splenic rupture is an important differential diagnosis in patients with hemorrhagic acute abdomen.

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