A case of nonfunctioning pancreatic endocrine tumor with atypical imaging findings due to prominent fibrosis of the tumor stroma.

The patient, a 56-year-old woman, was found during routine checkup to have a disorder of hepatic function. Abdominal ultrasonography showed an ill-defined hypoechoic mass in the head and body of the pancreas; however, no blood-flow signal was observed within the tumor on Doppler ultrasonography. Abdominal computed tomography showed a low-density area in the arterial and portal venous phases. The lesion was visualized as an area of low signal intensity on both T1- and T2-weighted magnetic resonance images, whereas fluorodeoxyglucose positron emission tomography showed fluorodeoxyglucose accumulation in the tumor. Although a preoperative diagnosis was difficult to make, a rapid cytologic examination revealed evidence of a pancreatic endocrine tumor, and subtotal stomach-preserving pancreaticoduodenectomy with portal vein resection was performed. Histopathological examination showed tumor cell nests scattered in abundant fibrotic tissue; the tumor cells had proliferated in a cord-like fashion and showed immunostaining for chromogranin A. Staining for fibroblast activation protein α was seen in the fibroblastic cells contained within the fibrous stroma surrounding the tumor cell nests, whereas both the fibroblastic cells in the tumor and those in the stroma showed a high rate of staining for thrombospondin. We presume that tumor-associated fibroblasts were involved in the fibrosis of the tumor stroma.

A case of nonfunctioning pancreatic endocrine tumor with atypical imaging findings due to prominent fibrosis of the tumor stroma.

The patient, a 56-year-old woman, was found during routine checkup to have a disorder of hepatic function. Abdominal ultrasonography showed an ill-defined hypoechoic mass in the head and body of the pancreas; however, no blood-flow signal was observed within the tumor on Doppler ultrasonography. Abdominal computed tomography showed a low-density area in the arterial and portal venous phases. The lesion was visualized as an area of low signal intensity on both T1- and T2-weighted magnetic resonance images, whereas fluorodeoxyglucose positron emission tomography showed fluorodeoxyglucose accumulation in the tumor. Although a preoperative diagnosis was difficult to make, a rapid cytologic examination revealed evidence of a pancreatic endocrine tumor, and subtotal stomach-preserving pancreaticoduodenectomy with portal vein resection was performed. Histopathological examination showed tumor cell nests scattered in abundant fibrotic tissue; the tumor cells had proliferated in a cord-like fashion and showed immunostaining for chromogranin A. Staining for fibroblast activation protein α was seen in the fibroblastic cells contained within the fibrous stroma surrounding the tumor cell nests, whereas both the fibroblastic cells in the tumor and those in the stroma showed a high rate of staining for thrombospondin. We presume that tumor-associated fibroblasts were involved in the fibrosis of the tumor stroma.

Adenocarcinoma of the minor duodenal papilla: report of a case.

We report a rare case of adenocarcinoma of the minor papilla of the duodenum treated with transduodenal minor papillectomy. A 64-year-old woman was treated for an asymptomatic duodenal tumor detected on gastroduodenoscopy. Endoscopy showed a 15-mm sessile mass in the descending duodenum proximal to the major papilla. The major papilla was a villous 24-mm-diameter polypoid tumor. Histopathologic examination of the biopsy specimen showed tubular adenoma with moderate epithelial atypia. Transduodenal major and minor papillectomies were performed. The orifice of the duct of Santorini and the pancreatic duct were re-approximated to the duodenal wall to prevent acute pancreatitis caused by scarring and stenosis of the duct orifice. Histological findings were consistent with well-differentiated adenocarcinoma limited to the minor duodenal papilla, without infiltration of the duodenal wall submucosa, and confirmed complete resection. The patient had an uneventful postoperative course and has remained asymptomatic, without evidence of tumor recurrence or stenosis of the pancreatic duct orifice, for 4 years.

Giant epidermoid cyst of the spleen with elevated CA 19-9 production managed laparoscopically: report of a case.

True splenic cysts are uncommon and are associated with elevated serum and intracystic tumor marker CA 19-9 levels. A 33-year-old woman presented to our hospital with a chief complaint of epigastralgia. Computed tomography of the abdomen showed a 10-cm cystic lesion in the spleen. The serum carbohydrate antigen (CA) 19-9 level was 3,347 U/mL (normal, <37 U/mL). Total laparoscopic splenectomy was performed, and the serum level of CA 19-9 had normalized 2 weeks later. Pathological examination showed a benign true epidermal cyst of the spleen with strong immunohistological staining for CA 19-9. Splenic epidermoid cysts most often occur in young women, and laparoscopic surgery to remove cysts of this type is minimally invasive. Thus, laparoscopic surgery should be the method of first choice for most cases of splenic benign true cyst.

T tube drainage for spontaneous perforation of the extrahepatic bile duct.

BACKGROUND: Spontaneous perforation of the extrahepatic bile duct is very rare. We report a patient with a spontaneous perforation of the left hepatic bile duct who was diagnosed preoperatively. CASE REPORT: A 65-year-old woman was admitted to our hospital complaining of a right upper quadrant pain lasting for two days. She was diagnosed as having a perforated bile duct and peritonitis and underwent a laparotomy. After a cholecystectomy, T-tube drainage of the left hepatic duct was performed. The postoperative course was uneventful. The T tube was removed 25 days after the surgery. CONCLUSIONS: A more noninvasive procedure, such as endoscopic treatment, should play a central role in the management of extra bile duct perforation. For this case, however, we chose to perform a laparotomy based on the patient's general condition and the presence of peritonitis. T tube decompression is effective and a safe and reliable method. The goal of treatment is to stop the bile leakage, resolve the choledocholithiasis and cholangitis, and reconstruct the bile duct.

Two successful curative operations using stomach-preserving distal pancreatectomy with celiac axis resection for the treatment of locally advanced pancreatic body cancer.

Large vessel invasion is a serious factor determining whether an operation for pancreatic body cancer is feasible. The Appleby operation is a radical operation for the treatment of pancreatic body cancer that has infiltrated the celiac axis. Since this procedure includes a total gastrectomy, the operation is associated with a high morbidity, mortality, and deteriorating postoperative quality of life (QOL). We experienced two cases in which radical operations consisting of a stomach-preserving distal pancreatectomy with en bloc resection of the celiac, common hepatic, and left gastric artery were performed. The use of adjuvant chemotherapy in these cases led to a good postoperative QOL.

Four-year survival of a patient with malignant fibrous histiocytoma of the liver treated with surgery and chemotherapy.

Malignant fibrous histiocytoma of the liver is an extremely rare tumor. A 43-year-old woman was admitted with an enlargement of the abdomen and a feeling of general fatigue. A clinical examination revealed a right liver giant cystic tumor (18.5 × 22 cm). The cystic tumor, which contained a solid component, occupied almost the entire right lobe of the liver, and a solid S4 tumor was also observed. A blood examination revealed severe anemia and mild liver dysfunction. During hospitalization intratumor bleeding occurred. A right lobectomy of the liver and lymph node dissection was performed after transcatheter arterial embolization. Histopathologically, the tumor was diagnosed as a primary storiform-pleomorphic-type malignant fibrous histiocytoma of the liver. One year after the radical operation, the patient developed recurrences in other organs. She received 17 cycles of chemotherapy with etoposide, ifosfamide, and cisplatin. The recurrent tumors decreased in size, but new recurrences in other organs occurred. The patient died of respiratory failure 4 years and 2 months after the initial operation.

Effects of BRCA1 transgene expression on murine mammary gland development and mutagen-induced mammary neoplasia.

To characterize the role of BRCA1 in mammary gland development and tumor suppression, a transgenic mouse model of BRCA1 overexpression was developed. Using the mouse mammary tumor virus (MMTV) promoter/enhancer, transgenic mice expressing human BRCA1 or select mutant controls were generated. Transgenic animals examined during adolescence were shown to express the human transgene in their mammary glands. The mammary glands of 13-week-old virgin homozygous MMTV-BRCA1 mice presented the morphology of moderately increased lobulo-alveolar development. The mammary ductal trees of both hemizygous and homozygous MMTV-BRCA1t340 were similar to those of control non-transgenic littermates. Interestingly, both hemi- and homozygous mice expressing a splice variant of BRCA1 lacking the N-terminal RING finger domain (MMTV-BRCA1sv) exhibited marked mammary lobulo-alveolar development, particularly terminal end bud proliferation. Morphometric analyses of mammary gland whole mount preparations were used to measure epithelial staining indices of ~35% for homozygous MMTV-BRCA1 mice and ~60% for both hemizygous and homozygous MMTV-BRCA1sv mice versus ~25% for non-transgenic mice. Homozygous MMTV-BRCA1 mice showed delayed development of tumors when challenged with 7,12 dimethylbenzanthracene (DMBA), relative to non-transgenic and homozygous BRCA1t340 expressing mice. In contrast, homozygous MMTV-BRCA1sv transgenic animals were sensitized to DMBA treatment and exhibited a very rapid onset of mammary tumor development and accelerated mortality. MMTV-BRCA1 effects on mortality were restricted to DMBA-induced tumors of the mammary gland. These results demonstrate in vivo roles for BRCA1 in both mammary gland development and in tumor suppression against mutagen-induced mammary gland neoplasia.