A rare case of extraluminally pedunculated gastrointestinal stromal tumor with postoperative metastasis to pancreas.

The gastrointestinal stromal tumor (GIST) is the most common type of sarcomatous tumor of the gastrointestinal tract. Many GISTs appear as submucosal tumors with intraluminal protrusion. GISTs with malignant features have a high risk of postoperative recurrence or metastasis, usually to the liver or peritoneum. We present a case of gastric GIST with double rarities: arising completely extraluminally with a pedicle and postoperative metastasis to the pancreas. A woman in her seventies diagnosed with a large extraluminal gastric GIST underwent complete removal of the tumor. Nine months later, a solitary metastatic tumor in the pancreas was detected. Imatinib controlled metastasis well for four years before the tumor became resistant. The patient then had a partial pancreatectomy with splenectomy. She is currently free from recurrence. We genetically analyzed the primary and metastatic GISTs and found known mutations related to poor prognosis and imatinib resistance.

A case of intraductal tubulopapillary neoplasm of pancreas with severe calcification, a potential pitfall in diagnostic imaging.

We experienced a case of intraductal tubulopapillary neoplasms (ITPN) of the pancreas with severe calcification, which complicated image diagnosis. A pancreas head tumor was detected in a Japanese female in her 50s. Early enhancement by contrast-enhanced CT and coarse calcification suggested a neuroendocrine tumor, although the obstruction and dilation of the main pancreatic duct appeared to be an intraductal tumor. An endoscopic ultrasound-guided fine needle aspiration biopsy specimen revealed adenocarcinoma tissue. Pancreaticoduodenectomy was performed, and the patient has been well without evidence of recurrence for over 10 months. Pathological examination on the resected specimen revealed that the tumor showed papillary and tubulo-cribriform growth patterns. Together with typical immunohistochemical results, the final diagnosis of ITPN was made. Characteristically, this case showed extensive calcification of both psammoma body-type and non psammoma body-type with foamy macrophage aggregation. This is the first report of ITPN with two types of calcification and macrophage. Since calcification might be one of the characteristic histological findings in ITPN as shown in our case, the possibility of ITPN should be also considered when calcification is detected in pancreatic lesions by various imaging modalities.

[A case of synchronous cancer of the gall bladder, common bile duct, and the papilla of vater].

A 58-year-old man was diagnosed with liver dysfunction during a health exam and subsequently visited a doctor. Abdominal ultrasonography revealed space-occupying lesions in the gall bladder and bile duct, and he was hospitalized for further examination and treatment. Computed tomography (CT), endoscopic retrograde cholangiopancreatography (ERCP), and magnetic resonance cholangiopancreatography (MRCP) revealed double cancer of the gall bladder and bile duct with pancreaticobiliary maljunction (PBM), and we performed a pancreatoduodenectomy. Pathological examination revealed gall bladder and bile duct cancer, and severe dysplasia of the papilla of Vater. We diagnosed synchronous triple cancer because none of the cancers had continuity or vascular invasion. Each cancer was at Stage I, and the patient has survived for 2 years and 6 months without recurrence and no additional treatment. PBM is a mutation of the junction of the pancreatic and bile ducts outside of the duodenal wall, and is a known complication of biliary tract cancer due to the reflux of pancreatic juice and bile. Because K-ras and p53 gene mutations occur in the biliary tract mucosal epithelium, PBM increases the risk of developing multicentric cancer. It is important to consider the existence of double cancer when biliary tract cancer is detected in a PBM patient.

[A case of postpancreaticoduodenectomy local recurrence successfully treated with extended resection].

A 68 -year-old man underwent a pancreaticoduodenectomy after being diagnosed with primary duodenal cancer. The postoperative pathological diagnosis was tub2, SE, ly1, v1, panc3, pn+, N0. Although adjuvant chemotherapy was administered, local recurrence in the portal region was detected 18 months later. The recurrent tumor pressed against the region of the bile duct anastomosis, which caused obstructive jaundice. After serum bilirubin levels were reduced, resection of the recurrent tumors was performed. This required resection of the transverse colon, parts of the portal vein, and the inferior vena cava. The bile duct anastomotic region, which had been infiltrated by the tumor, was excised and rebuilt. The postoperative pathological diagnosis was tub2. The patient continued to receive adjuvant chemotherapy and showed no signs of recurrence 9 months after surgery. Extended resection for local recurrences of primary duodenal cancer may be an effective means of disease control.

[A case of extragastrointestinal stromal tumor with peritoneal dissemination].

Here, we present the case of a 60-year-old man in whom abdominal computed tomography showed a solid abdominal tumor (11 cm in diameter) in the pelvic space, with widely disseminated nodular lesions. Emergency surgery was performed following the rapid onset of intense abdominal pain. Peritoneal disseminations were widespread and the tumor was confirmed to be in the pelvic space. The tumor was not connected to any segment of the intestinal tract but rather to the retroperitoneum. Immunohistochemical staining was positive for c-kit (exon 11 mutation) and CD34 but negative for S-100 protein. Careful postoperative examination did not reveal any lesions in the upper or lower alimentary tract. On the basis of these findings we diagnosed the tumor as an extragastrointestinal stromal tumor (EGIST) originating from the retroperitoneum. After surgery, intravenous infusion of imatinib was started at a full dose of 400mg/day; however, owing to strong adverse effects, the dose was reduced to 200mg/day. Despite halving the dose, the patient has remained lesion-free according to computed tomography for 36 months after the operation. Low-dose imatinib chemotherapy remained efficacious in controlling progression in this case.

[Russell body gastritis].

A 63-year-old woman who presented with chest and back pain underwent an upper gastrointestinal endoscopy which revealed elevated legion in the antrum mucosa. Histologic examinations of gastric biopsies were showing monoclonal proliferation plasma cells containing Russell bodies. Differential diagnosis from B-cell lymphoma and plasmacytoma is difficult, because of monoclonality. Molecular analyses of immunoglobulin heavy chain (IgH) gene demonstrated that gene rearrangement was negative. Thus, diagnosis of Russell body gastritis was made. The Giemsa stains were also showing infection of Helicobacter pylori (H.pylori). After eradication therapy for H.pylori, follow-up upper gastrointestinal endoscopy was performed. She then recovered.

[A case of unresectable advanced gastric cancer with peritoneal dissemination responding to UET/low-dose CDDP combination chemotherapy].

We report the case of a 69-year-old female with unresectable gastric cancer (T3, N2, P3, H0, Stage IVb) accompanied by peritoneal dissemination, diagnosed on laparotomy. UFT/low-dose cisplatin (CDDP) combination chemotherapy was performed after surgery. UFT 300 mg/day was administered orally every day, and CDDP 10 mg was injected intravenously every week. Chemotherapy was continued for ten months with a total dose of CDDP of 380 mg, but was stopped after oral mucositis developed as a side effect. Seven months after the chemotherapy was started, endoscopy revealed that the gastric cancer tumors had disappeared and the gastric mucosa was intact. Gastric cancer recurrence occurred 2 years and 2 months after chemotherapy was started. Low-dose CDDP/5-FU chemotherapy and TS-1 chemotherapy were performed, but no effects were observed. The patient died 3 years and 6 months after the start of initial chemotherapy, and was treated as an outpatient for 3 years while maintaining a good quality of life. UFT/low-dose CDDP combination chemotherapy offers promise as an effective tool in the clinical management of advanced gastric cancer with peritoneal dissemination.