Division of Gastroenterologic and General Surgery, Mayo Clinic, Rochester, MN 55905, USA.
J Am Coll Surg. 2012 Jan;214(1):27-32. doi: 10.1016/j.jamcollsurg.2011.09.025. Epub 2011 Nov 23.
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.
胆管内乳头状黏液性肿瘤（B-IPMN）已被提出为一种具有独特临床病理特征的疾病，尽管其广泛接受仍存在争议。最近的一次共识会议根据形态学表现和黏蛋白（MUC）染色特性将胰腺上皮内瘤变（P-IPMN）分为 4 个亚型（即胃型、肠型、胰胆管型、嗜酸细胞型）。本研究旨在确定 B-IPMN 是否具有与 P-IPMN 相似的组织病理学和免疫表型亚型。
对 19 例经组织学诊断为 B-IPMN 的患者标本进行 MUC1、MUC2 和胰腺癌细胞缺失基因 4 （deleted for pancreas cancer, locus 4，DPC4）的特异性免疫染色。B-IPMN 的免疫染色模式与组织病理学相关联。
根据组织病理学，确定了以下 B-IPMN 亚型：胰胆管型 9 例（47%）、肠型 8 例（42%）、嗜酸细胞型 2 例（11%）和胃型 0 例（0%）。胰胆管型和嗜酸细胞型 B-IPMN 对 MUC1 呈阳性，对 MUC2 呈阴性，而肠型 B-IPMN 对 MUC2 呈阳性，对 MUC1 呈阴性。19 例 B-IPMN 中有 13 例与浸润性癌相关；在 13 例浸润性成分中的 6 例和 19 例非浸润性成分中的 3 例中发现 DPC4 缺失。接受 B-IPMN 和浸润性癌切除术的患者 5 年生存率为 38%，与接受 P-IPMN 和浸润性癌切除术的患者相似。
B-IPMN 的组织病理学亚型和特定的 MUC 表达模式与 P-IPMN 相似。在 B-IPMN 中发现了与 P-IPMN 侵袭性特征相关的 MUC1 表达和/或 MUC2 表达缺失，这与侵袭性 B-IPMN 相关。DPC4 的缺失与在 P-IPMN 中观察到的结果平行。这些发现进一步支持 B-IPMN 是一种与主胰管 P-IPMN 具有相似性的独特实体。
