Yutaka Onishi

Malignant endocrine tumor of the pancreas associated with von Recklinghausen's disease.

We describe a case of malignant endocrine tumor of the pancreas associated with von Recklinghausens disease. A 66-year-old woman with familial von Recklinghausens disease was admitted to our hospital with weight loss and lower abdominal pain. Endoscopy revealed a submucosal tumor in the second part of the duodenum. Contrast-enhanced computed tomography showed a heterogeneous low-density tumor, measuring 20 x 15 mm, in the head of the pancreas, and metastatic lymphadenopathy. Angiography showed faint tumor staining without encasement or apparent increase in vascularity. Pancreatoduodenectomy was performed. Macroscopically, there was a solid tumor, measuring 20 x 18 mm, in the head of the pancreas. Histologically, a malignant endocrine tumor was shown, with direct invasion to the duodenum, and lymph node metastases. This is the thirteenth case of pancreatic malignant tumor and the fourth case of pancreatic endocrine tumor associated with von Recklinghausens disease reported in the world literature.

Large mucosa-associated lymphoid tissue lymphoma simulating multiple polypoid lesions at the cecum and rectum

Herein we describe a case of mucosa‐associated lymphoid tissue (MALT) lymphoma of the cecum and rectum with the Leser–Trélat sign. A 76‐year‐old Japanese woman was admitted to the Harima Hospital of Ishikawajima‐harima Heavy Industries, Health Insurance Society for hematochezia. Colonoscopy showed two large elevated tumors, one in the cecum and the other in the rectum. Biopsy was not diagnostic. Endoscopic snare loop biopsy specimens from both tumors were diagnosed as MALT lymphoma. After staging to IIE, the patient underwent surgery. Macroscopically, the cecal tumor was elevated with a large and deep depressed region, measuring 40 × 35 mm. The rectal lesion was a large elevated tumor, measuring 80 × 70 mm. Histologically, both tumors were diagnosed as MALT lymphoma and there was no lymph node metastasis. The patient received chemotherapy and there is no evidence of recurrence 1 year after surgery. We present this case to show that colorectal MALT lymphoma can present as a large tumor, even in early clinical stages, that multiple lesions should be anticipated and that surgical treatment is needed in these cases.

Nodule‐aggregating lesion of the ileum: Report of a case and a review of the literature

We describe here a rare case of nodule‐aggregating lesion of the terminal ileum detected by colonoscopy. An 82‐year‐old Japanese woman was admitted to our hospital with diarrhea. Colonoscopy revealed a flat elevated tumor with conglomerated nodular surface involving the entire circumference of the terminal ileum, suggesting a nodule‐aggregating lesion. Magnifying the colonoscopic view showed the branch‐like or gyrus‐like pits. On biopsy, the tumor was diagnosed as a tubulovillous adenoma. Retrograde ileogram using a colonoscope revealed an elevated tumor with nodular irregularity, measuring 5 cm in length. Ileocecal resection was performed. Macroscopically, the tumor in the terminal ileum, 8 cm distant from the ileocecal valve, showed a nodule‐aggregating lesion, measuring 44 × 60 × 6 mm in size. Histologically, the tumor showed a focal carcinoma in tubulovillous adenoma. To our knowledge, this is the fifth case of early cancer of the ileum in Japan, and the first case of nodule‐aggregating lesion of the ileum detected by colonoscopy in the world.

DUODENAL ADENOMA OF GASTRIC FOVEOLAR PHENOTYPE IN THE SECOND PORTION OF THE DUODENUM

We describe a rare case of duodenal adenoma of a gastric phenotype in the second portion of the duodenum. An 85‐year‐old Japanese man with a history of left nephrectomy was admitted to our hospital with a cerebral transient ischemia attack. Screening upper gastrointestinal endoscopy revealed a subpedunculated polyp with a reddish, erosive, glossy appearance, and a granular surface just above the papilla of Vater in the second portion of the duodenum, which on biopsy was diagnosed as a hyperplastic polyp. Hypotonic duodenography revealed a subpedunculated polyp with irregular granular surface, measuring approximately 20 mm in size, in the second portion of the duodenum. Endoscopic total resection for biopsy was performed. The histopathological examination of the polypectomied specimens revealed an adenoma with moderate dysplasia mimicking gastric foveolar epithelium or hyperplastic polyp accompanied by abundant mucin production. Immunohistochemistry revealed a complete gastric phenotype, suggesting it had probably arisen from heterotopic gastric mucosa. The present case is the eleventh case of duodenal neoplasia arising from heterotopic gastric mucosa and the sixth case of neoplasia with gastric phenotype found in the second portion of the duodenum reported in the world.

Carcinosarcoma of the Extrahepatic Bile Duct Presenting with Stone-like Radiological Findings

A 73-year-old woman was referred to our hospital due to epigastralgia and jaundice. The radiological findings showed a stone-like tumor in the extrahepatic bile duct. The patient was initially thought to have adenocarcinoma of the bile duct based on the findings of a pathological examination of the bile duct biopsy specimen and underwent pancreaticoduodenectomy; the final diagnosis of the lesion was so-called carcinosarcoma of the extrahepatic bile duct. She died of liver metastasis six months after the surgery. This case suggests that surgical resection is not adequate for achieving a radical cure, and the optimal treatment for extrahepatic bile duct carcinosarcoma should be established immediately.

Primary composite tumor with bipartite differentiation of the esophagogastric junction

We describe a rare case of primary composite tumor with bipartite differentiation of the esophagogastric junction. An 88‐year‐old Japanese man with a history of distal subtotal gastrectomy was admitted to Harima Hospital of Ishikawajima‐harima Heavy Industries with dysphagia of 2 months duration. Endoscopy showed a round, sharply demarcated lesion with a smooth surface in the lower esophagus. Distally, the lesion was irregular and elevated with a deep central depression. The gastric cardia was slightly elevated with a granular mucosa. After surgical resection, histological and histochemical findings showed a primary composite tumor with bipartite differentiation of the esophagogastric junction. There is no evidence of recurrence for 21 months after surgery without adjuvant chemotherapy. The present case indicates the necessity for a careful evaluation for accessory lesions in composite tumors containing small cell carcinoma of the esophagogastric junction, and that resectional surgery might not only be the treatment of choice, but also important in the evaluation of immature composite tumor without distant metastases.