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In the ampulla of Vater, carcinomas with "diffuse-infiltrative"/"signet ring cell" morphology, designated as "poorly cohesive carcinoma" (PCC) in the WHO classification, are very rare and poorly characterized. Nine cases with a classical PCC morphology constituting >50% of the tumor were identified. Mean age was 64.8 years (vs 64.6 in ampullary carcinomas [ACs]) and 6 were males, 3 females. The mean invasive tumor size was 2.5 cm (vs 1.9 in ACs). Other morphologic patterns displayed included cord-like infiltration (n=2), plasmacytoid cells (n=2), and microglandular component (n=4), including goblet cell adenocarcinoma-like foci. None of the cases were associated with dysplasia. By immunohistochemistry, the carcinomas did not show intestinal differentiation (CDX2 0/9, CK20 1/9, MUC2 3/9), MUC1 was positive in 4/9, MUC5AC was positive in 7/8. E-cadherin loss was noted in 4/9. All cases were advanced stage (6/9-pT3, 3/9-pT4) (vs 43% in ACs). Lymph node metastases were identified in 44% (vs 45% in AC). Six patients (67%) died of disease at a median of 25 months, 3 were alive at 13, 15, and 60 months. Overall median survival was significantly worse than that of intestinal-type ACs (26 vs 122 months, P =.006) and trended toward worse than pancreatobiliary type (26 vs 42 months, P =.1). In conclusion, PCCs constitute 2.45% of all ACs. These present as advanced tumors and express upper-gastrointestinal immunoprofile with frequent MUC5AC labeling, which may be helpful in identifying subtle infiltration in the surface mucosa since MUC5AC is not expressed in the ampullary mucosa. Patients have poor prognosis. [ABSTRACT FROM AUTHOR]

Background. Mucins are high-molecular-weight glycoproteins that play important roles in carcinogenesis or tumor invasion. The authors investigated the expression of mucins in ampullary cancer. Methods. MUC1 and MUC2 expressions were examined using immunohistochemistry. Tissue samples were obtained from 32 patients with ampullary cancer who underwent resection at Yamagata University Hospital, Japan. The authors classified the cases with ampullary cancer into 2 subtypes—pancreatobiliary type (PB type) and intestinal type (I type)—using H&E, MUC1, and MUC2 staining. Then, the authors made a comparison of the clinicopathologic data of the 2 subtypes. Results. Fourteen patients (44%) were classified as PB type and 18 patients (56%) as I type. The PB-type group had significantly worse histopathologic characteristics than the I-type group in nodal involvement (PB type 57% vs I type 22%; P = .04), perineural invasion (PB type 50% vs I type 17%; P = .04), duodenal invasion (PB type 100% vs I type 33%; P = .01), and pancreatic invasion (PB type 71% vs I type 33%; P = .03). The PB-type group had significantly worse outcome than the I-type group (5-year survival: PB type 40% vs I type 72%; P = .03). Conclusion. PB-type ampullary cancers were more aggressive than I-type carcinomas. MUC1 and MUC2 expression was useful for classification as PB or I type. [ABSTRACT FROM PUBLISHER]

Context. Subtyping of periampullary adenocarcinoma into intestinal and pancreatobiliary subtypes has emerged as an important prognostic factor with potential therapeutic implications. This distinction on morphology alone is often difficult with significant interobserver variability. Objective. To analyze the usefulness of a panel of immunohistochemistry (IHC) markers as an aid to morphologic subtyping of periampullary adenocarcinoma. Design. A total of 172 periampullary adenocarcinomas were classified morphologically by 3 study pathologists. Interobserver agreement was assessed in each case. Cases were then typed using a predetermined IHC panel (comprising CK7, CK20, MUC1, and CDX2). Results. Morphologically, 66 (38.4%) cases were intestinal, 56 (32.6%) pancreatobiliary, 25 (14.5%) mixed, 16 (9.3%) poorly differentiated, 6 (3.5%) mucinous, and 3 (1.7%) signet ring cell adenocarcinoma. Concordant diagnosis was reached in 138 cases (80.2%) with moderate overall interobserver agreement (κ = 0.47). Concordance was higher in morphologically distinct mucinous (100%; κ = 0.94) and signet ring cell subtypes (100%; κ = 1.0) than in intestinal (84.6%; κ = 0.47) and pancreatobiliary (82.1%; κ = 0.43) types. Concordance was poor for mixed (64%; κ = 0.27) and poorly differentiated (68.8%; κ = 0.76) tumors. IHC subtyped 79 cases (46%) as pancreatobiliary, 73 (42.4%) as intestinal, and was inconclusive in 20 cases (11.6%). IHC helped classify 21 out of 25 (84%) mixed and 10 out of 16 poorly differentiated (62.5%) adenocarcinomas. Combination of histology and IHC classified 161 of the total 172 cases (93.6%). Conclusion. Use of an IHC panel aids in subtyping of periampullary adenocarcinomas, especially in tumors with mixed morphology and poor differentiation. [ABSTRACT FROM AUTHOR]

Intraductal papillary mucinous neoplasms (IPMNs) are noninvasive lesions of the pancreas and classified as intraductal papillary mucinous adenomas (IPMAs), borderline IPMNs, and intraductal papillary mucinous carcinomas (IPMCs). Expression patterns of the specific genes alter during IPMN progression. Based on the evidence that signal transducers and activators of transcription (STAT) 5 play important roles in tumor development, we tested STAT5 expression in IPMAs, borderline IPMNs, and IPMCs byimmunohistochemical method. STAT5 frequently expressed in the nuclei of tumor cells of borderline IPMNs or IPMCs but was not observed in those of IPMAs. Nuclear expression of STAT5 protein correlated to the Ki-67 labeling index of the examined IPMNs. STAT5 protein could contribute to the progression and proliferation of IPMNs. [ABSTRACT FROM AUTHOR]

Gastric heterotopia within the biliary system is extremely rare. Moreover, the combination of gastric heterotopia in the bile duct with cholangiocarcinoma has not been reported. We describe a case of heterotopic gastric mucosa in the common bile duct with cholangiocarcinoma. An 80-year-old male was admitted with abdominal pain. Abdominal computed tomography revealed wall thickening from the hilar duct to the distal common bile duct. Biopsy from the distal bile duct showed only benign gastric foveolar-type epithelium and fundic glands. Although the diagnosis of the biopsy was benign, malignancy was strongly suspected from the radiologic findings, and excision of the bile ducts was performed. Microscopically, the resected specimen showed poorly formed malignant glands and gastric heterotopia also identified in the common bile duct. Three months later, the patient’s state worsened due to recurrence, and he died. To our knowledge, this is the first report of gastric heterotopia in the bile duct accompanying cholangiocarcinoma. [ABSTRACT FROM AUTHOR]

Introduction. Mixed adenoneuroendocrine carcinoma (MANEC) has recently been defined by the World Health Organization in 2010. These are rare tumors and MANECs of ampullary region are even rarer. Only 19 cases have been reported in literature. We present 3 cases; the largest series, second case of amphicrine tumor and first case associated with chronic pancreatitis. Methods. Retrospective review of 3 patients who were diagnosed to have ampullary MANEC. Results. All 3 patients were diagnosed preoperatively as neuroendocrine carcinoma and underwent margin negative pancreaticoduodenectomy. The histopathology revealed MANECs of small cell, mixed type in 2 patients and large cell, amphicrine type in 1 patient. The neuroendocrine component was grade 3 in all, the tumor was T3 in 2 and T2 in 1 and all had nodal metastases. Two patients received adjuvant chemotherapy and 2 of them had recurrence at 13 and 16 months. The median survival was 15 months. Conclusion. Ampullary MANECs are rare tumors. They are diagnosed on histopathologic examination of the resected specimen. Clinical presentation, management, and prognosis is similar to ampullary adenocarcinoma in literature. [ABSTRACT FROM AUTHOR]

Introduction: Our study attempts to characterize mesothelin and GPR30 / estrogen receptor (ER) staining in pancreatic pathology. Materials and Methods: Immunohistochemical staining for mesothelin, GPR30, and ER was performed on a variety of pancreatic lesions. Results: 24 of 42 (57%) adenocarcinomas stained for mesothelin, while 0 of 16 non-carcinomas (0%) stained (p = 0.0000784). 35 of 39 (90%) adenocarcinomas stained for GPR30, while only 4 of 15 (27%) non-carcinomas stained (p = 0.0000036). Apart from stromal staining in one case of mucinous cystic neoplasm, no cases stained for ER. 27 of 37 (73%) adenocarcinoma fine needle aspirates were positive for mesothelin. Discussion: GPR30 is more sensitive, but less specific than mesothelin for pancreatic adenocarcinoma. Mesothelin is detected in most adenocarcinoma fine needle aspirates. ER is rarely detected in pancreatic lesions. [ABSTRACT FROM PUBLISHER]

The article presents a case study of an 86-year-old woman who was suffering from intestinal bleeding and anemia. Topics discussed include presence of polypoid mass in small intestine revealed from abdominal computed tomography, dialation of submucosal glands and proliferation of stroma was observed from histological testing, and diagnosis of adenomyoma along small bowel was reported.