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Micropapillary carcinoma (MPC) has been recently reported as a variant of invasive breast carcinoma. MPC is also known to be an aggressive variant of adenocarcinoma, and it is associated with poor prognosis and a high propensity for lymphovascular invasion and lymph node metastases. MPC of the breast, urinary bladder, and lung has been reported relatively frequently; however, there have been few reports on gastrointestinal MPC. Furthermore, MPC of the small bowel has not been reported yet. Here we report a case of MPC of the jejunum. A female septuagenarian was admitted because of anorexia and weight loss. We established a diagnosis of primary jejunal cancer and resected the duodenum and a part of the jejunum. Histologically, the tumor consisted of adenocarcinoma with moderate to poor differentiation. Among the carcinoma cells, approximately 10% formed small papillary neoplastic cell clusters surrounded by clear spaces, suggesting a diagnosis of MPC. We present the details of the case along with a review of relevant literature.

A woman in her 80s had two episodes of ileus, which led to the diagnosis of advanced jejunal cancer. She was diagnosed with Lynch syndrome when she was in her 60s, for which she underwent annual follow-up with computed tomography for 8 years. Unfortunately, she died from the recurrence of jejunal cancer and liver metastases. Jejunal cancer is relatively rare in Lynch syndrome, and no surveillance strategy has been established for small bowel cancer. In patients with unexplained abdominal complaints, small bowel cancer should be considered.

We report a case of 72-year-old man found to have a primary malignant melanoma in the jejunum. The patient was noted to be anemic and had lower abdominal pain on his visit to the Department of Gastroenterology. However, an upper gastrointestinal series and colonofiberscopic examination revealed no abnormalities. After clinical examinations, the radiological workup, which included CT, X-ray of the small intestine and single-balloon enteroscopy, revealed an intraluminal polypoid tumor, with a patchy light gray and black pattern. Pre-operative biopsy specimens revealed a malignant melanoma. Segmental intestinal resection with regional lymph node dissection was performed. The tumor size was 7.0×9.5×5.8cm. Nodal metastasis was seen only in the mesenteric node draining from the tumor-bearing intestinal segment (stage IIIa). Adjuvant chemotherapy with dacarbazine, nimustine hydrochloride and vincristine sulfate was performed, and the patient was able to recover his level of activity of daily living for 6 months.

Between 1989 and 2009, 10 patients with small bowel adenocarcinoma were treated in our hospital. These tumors appeared in the jejunum in 6 patients and in the ileum in the remaining 4 patients. All patients had some symptoms. The median size of the tumors was 50mm(30-110mm). All tumors were advanced type 2 lesion with severe stricture. Histologically there were 8 well, 1 moderately and 1 poorly differentiated adenocarcinomas. There were 8 tumors invading the serosa and 2 tumors invaded other organs. Positive lymph nodes were identified in 6 cases. Liver metastasis and peritoneal dissemination were identified in 3 and 4 cases, respectively. Eight cases were diagnosed as small bowel adenocarcinoma preoperatively by double balloon endoscopy. The 4 patients with stage II tumor and 2 patients with stage III tumor underwent curative-intent surgery. The 4 patients with stage II tumor are all surviving without evidence of disease now.

The patient was a 75-year-old woman who had undergone resection of a transverse colon cancer two years before. She had anemia and intestinal obstruction, and a diagnosis of multiple metastases to the small intestine was made by double balloon enteroscopy. Eleven metastatic foci were resected by partial resection of the jejunum and ileum. Adjuvant FOLFOX chemotherapy was given, achieving a 26-month disease-free survival. The double balloon enteroscopy was useful in the definitive diagnosis of this case, and aggressive resection with adjuvant chemotherapy contributed to the good outcome.

A 63-year-old male was admitted to our department for further examination of hypergastrinemia. Secretin provocation test and calcium infusion test suggested Zollinger-Ellison syndrome and percutaneous transhepatic portal venous sampling (PTPVS) demonstrated gastrinoma in the jejunum, although CT, ultrasonography and angiography could not accurately detect the location of the gastrinoma. Laparotomy findings showed a solid tumor 1.5 cm in diameter in the jejunal mesentery 5 cm distal to the ligament of Treitz, and primary gastrinoma was confirmed in the submucosa of the jejunum immediately adjacent to this tumor. An immunohistochemical study using the PAP method revealed gastrin secreting cells in the tumor. In addition to this case of jejunal gastrinoma, a review of literature in Japan and other countries was presented.

An autopsy case of malignant fibrous histiocytoma (MFH) of the mediastinum in a 25-year-old Japanese man is described. He initially complained of general fatigue and intermittent tarry stool, and was radiographically revealed to have a huge mass within the mediastinum as well as multiple nodules within the abdominal cavity, respectively. The mediastinal tumor could not be resected because of direct tumor invasion into surrounding tissues. Within the abdominal cavity, three distinct tumor nodules were discovered in the jejunum, which could be resected surgically. Histopathologically, the components of both lesions were very similar, and the present case was interpreted as multiple metastases of mediastinal MFH to the intestinal wall. In spite of various kinds of treatment, the mediastinal tumor rapidly enlarged, causing SVC syndrome. Brain CT findings suggested multiple metastases which were considered to be the cause of death. Autopsy confirmed that the main tumor nodule was located within the mediastinum, with brain metastases. The present case of mediastinal MFH is considered to be the youngest case as well as the first case presenting intestinal metastases.

To obtain characteristic findings of gastrointestinal aberrant pancreas by endoscopic ultrasonography (EUS), we made comparison between endoscopic ultrasonograms and histological findings in 5 cases (6 lesions) of resected gastric aberrant pancreas and 1 case (1 lesion) of small intestinal one. By EUS, gastrointestinal aberrant pancreases were delineated as hypoechoic masses with blurred boundary containing fine scattered hyperechoic spots, and also there were thickening of the fourth layer below the mass. Occasionally, there were some duct-like structures within the mass or marginal lobular structures. Moreover, the ultrasonographic patterns of aberrant pancreases were classified into 2 types, that is, M and S types. On histological examination, M type cases, in which the mass attached to the thickened fourth layer by EUS, were corresponded to acinar type and the pancreatic acinar cells penetrated into the proper muscle layer of the gastrointestinal wall, whereas S type cases, in which the mass was separated from the fourth layer, to ductal or mixed type. We conclude, therefore, that not only making the accurate diagnosis of aberrant pancreas but also drawing an inference of its histological type might become possible by EUS.