Frequency of Subtypes of Biliary Intraductal Papillary Mucinous Neoplasm and Their MUC1, MUC2, and DPC4 Expression Patterns Differ from Pancreatic Intraductal Papillary Mucinous Neoplasm
ABSTRACT Biliary intraductal papillary mucinous neoplasm (B-IPMN) has been proposed as a unique clinicopathologic disease with distinct histopathologic features, although wide acceptance remains controversial. A recent consensus conference classified pancreatic IPMN (P-IPMN) into 4 subtypes (ie, gastric, intestinal, pancreatobiliary, oncocytic) based on morphologic appearance and mucin (MUC) staining properties. The aim of this study was to determine whether B-IPMN has similar histopathologic and immunologic subtypes to P-IPMN.
Specific immunostaining for MUC1, MUC2, and deleted for pancreas cancer, locus 4 were performed on specimens from 19 patients with a histopathologic diagnosis of B-IPMN. Immunostaining patterns of B-IPMN were correlated with histopathology.
Based on histopathology, the following subtypes of B-IPMN were identified: pancreatobiliary n = 9 (47%), intestinal n = 8 (42%), oncocytic n = 2 (11%), and gastric n = 0 (0%). Pancreatobiliary and oncocytic subtypes of B-IPMN were positive for MUC1 and negative for MUC2, and intestinal subtypes were positive for MUC2 and negative for MUC1. Thirteen of the 19 B-IPMN were associated with invasive carcinoma; loss of deleted for pancreas cancer, locus 4 was found in 6 of 13 invasive components and in 3 of 19 noninvasive components of B-IPMN. Five-year survival for patients with resected B-IPMN and invasive carcinoma was 38%, which is similar to that for resected P-IPMN with invasive carcinoma.
Histopathologic subtypes and type-specific MUC expression patterns of B-IPMN resemble those of P-IPMN. MUC1 expression and/or absence of MUC2 expression, which correlate with aggressive features of P-IPMN, were found in B-IPMN and correlate with invasive B-IPMN. Loss of deleted for pancreas cancer, locus 4 parallels the findings observed in P-IPMN. These findings provide additional support that B-IPMN is a unique entity with similarities to main duct P-IPMN.
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ABSTRACT: Intraduktale papilläre Neoplasien der Gallenwege (IPNB, „intraductal papillary neoplasms of the bile duct“) sind seltene Vorläuferläsionen intra- und extrahepatischer Cholangiokarzinome, die sich über eine Adenom-Karzinom-Sequenz entwickeln. Nach der vorherrschenden Histologie und dem immunhistochemischen Muzinprofil werden 4 Subtypen unterschieden (pankreatobiliär, intestinal, gastrisch und onkozytär), die eine prognostische Relevanz haben können. Die Differenzialdiagnosen umfassen mikropapilläre Läsionen (biliäre intraepitheliale Neoplasien), papillär-zystische Läsionen (intraduktale tubulopapilläre Neoplasien des Gallengangs) und zystische Läsionen (muzinös zystische Neoplasien). Abstract Intraductal papillary neoplasms of the bile duct (IPNB) are rare precursor lesions of intrahepatic and extrahepatic cholangiocarcinoma that follow an adenoma-carcinoma sequence. According to the histomorphology and the distinct immunohistochemical mucin pattern, four different subtypes are recognized: pancreatobiliary, intestinal, gastric and oncocytic. Differential diagnoses include micropapillary lesions (biliary intraepithelial neoplasms), papillary cystic lesions (intraductal tubulopapillary neoplasms) and cystic lesions (mucinous cystic neoplasms).Der Pathologe 11/2013; 34(S2):235-240. DOI:10.1007/s00292-013-1861-3 · 0.64 Impact Factor
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ABSTRACT: Intraductal papillary neoplasm of the bile duct (IPNB) is a variant of bile duct carcinoma that is characterized by intraductal growth and better outcomes compared with common cholangiocarcinoma. IPNBs are mainly found in patients from Far Eastern areas, where hepatolithiasis and clonorchiasis are endemic. According to the immunohistochemical profiles of the mucin core proteins, IPNBs are classified into four types: pancreaticobiliary, intestinal, gastric, and oncocytic. Approximately 40%-80% of IPNBs contain a component of invasive carcinoma or tubular or mucinous adenocarcinoma, suggesting that IPNB is a disease with high potential for malignancy. It is difficult to make an accurate preoperative diagnosis because of IPNB's low incidence and the lack of specificity in its clinical manifestation. The most common abnormal preoperative imaging findings of IPNB are intraductal masses and the involvement of bile duct dilation. Simultaneous proximal and distal bile duct dilation can be detected in some cases, which has diagnostic significance. Cholangiography and cholangioscopy are needed to confirm the pathology and demonstrate the extent of the lesions. However, pathologic diagnosis by biopsy cannot reflect the actual stage in many cases because different foci may be of different stages and because mixed pathologic findings may exist in the same lesion. Surgical resection is the major treatment. Systematic cholangioscopy with staged biopsies and frozen sections is recommended during resection to ensure that no minor tumors are left and that curative resection is achieved. Staging, histologic subtype, curative resection and lymph node metastasis are factors affecting long-term survival.World Journal of Gastroenterology 12/2013; 19(46):8595-8604. DOI:10.3748/wjg.v19.i46.8595 · 2.43 Impact Factor
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ABSTRACT: BACKGROUND: Intraductal papillary mucinous neoplasm of the biliary tract (BT-IPMN) has been increasingly recognized as a biliary counterpart of intraductal papillary mucinous neoplasm of the pancreas (P-IPMN). However, there is limited information regarding whether BT-IPMNs and P-IPMNs behave in a similar fashion. METHODS: We retrospectively compared clinicopathological variables between 9 patients with BT-IPMN and 44 patients with P-IPMN. RESULTS: There was no significant difference in age between patients with BT-IPMN and those with P-IPMN. The male/female ratio was significantly higher in patients with P-IPMN than in those with BT-IPMN (P = 0.012). Clinical presentation with jaundice was more common in patients with BT-IPMN (67%) than in those with P-IPMN (4.5%, P = 0.002). In addition, serum levels of CEA and CA19-9 were higher in patients with BT-IPMN than in those with P-IPMN (P = 0.019 and P = 0.002, respectively). The pathological diagnosis of malignancy was significantly more common in patients with BT-IPMN (89%) than in those with P-IPMN (23%, P = 0.002). The association with invasive carcinoma was significantly more frequent in patients with BT-IPMN (44.4%) than in those with P-IPMN (6.8%, P = 0.008). Furthermore, survival time after surgical resection was significantly shorter in patients with BT-IPMN than in those with P-IPMN (P = 0.002). CONCLUSION: These findings reveal differences in clinicopathological features and prognosis between BT-IPMN and P-IPMN, thereby suggesting distinct biological pathways underlying the pathogenesis of these neoplasms.European journal of surgical oncology: the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology 03/2013; 39(6). DOI:10.1016/j.ejso.2013.02.016 · 2.89 Impact Factor