Cystic Papillary Pattern in Pancreatic Ductal Adenocarcinoma: A Heretofore Undescribed Morphologic Pattern That Mimics Intraductal Papillary Mucinous Carcinoma
Cystic Papillary Pattern in Pancreatic Ductal Adenocarcinoma: A Heretofore Undescribed Morphologic Pattern That Mimics Intraductal Papillary Mucinous Carcinoma
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DOI：
10.1097/pas.0b013e318249ce1c
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发表时间：
2012-05-01
影响因子：
5.6
通讯作者：
Deshpande, Vikram
中科院分区：
文献类型：
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作者：
Kelly, Paul J.;Shinagare, Shweta;Deshpande, Vikram
导言：典型的胰腺导管腺癌表现为小口径的腺体位于旺盛的促结缔组织间质反应中。已经描述了许多组织学模式，其中大多数模式在遗传学和生物学上与传统的导管腺癌有关。在这篇报告中，我们描述了一种迄今未被描述的胰腺癌的组织学类型，它在形态和放射学上都与导管内乳头状粘液性癌相似。记录人口学、临床、放射学和结果数据。除了回顾组织学特征外，我们还进行了弹性蛋白染色、部分癌基因和包括Smad4在内的肿瘤抑制基因的免疫组织化学检查。结果：患者平均年龄67岁，其中女性6例，男性4例。大体而言，其中6例的切面可见实性和囊性混合区。乳头状囊性结构与常规腺癌区紧密结合，后者以侵袭性小口径管状结构为特征。所有肿瘤均未表现为纯乳头状囊性改变，但在8例中，乳头状囊性改变占肿瘤的50%。囊和乳头主要由高柱状的高粘液上皮排列。在这些扩张的恶性囊肿和腺体周围没有发现弹性蛋白纤维。瘤内间质细胞稀少，呈玻璃化。10个肿瘤中有7个Smad4呈阴性。大多数病例中囊周弹性蛋白纤维的缺乏和Smad4的丢失与导管内乳头状黏液性肿瘤的病变相矛盾。10个肿瘤均为MUC1染色，1例有灶性MUC2反应。大多数病例MUC5AC(9/10)和MUC6(8/10)阳性。7名患者死于自己的疾病，而1名患者因广泛转移而存活。2例患者失访。结论：本文描述的腺癌是胰腺导管腺癌的一种独特的形态类型。其生物学和遗传学(免疫组织化学估计)与传统的导管腺癌没有什么不同，但与其形态最相似的导管内乳头状黏液癌有明显的不同。
Introduction: The prototypic pancreatic ductal adenocarcinoma shows small-caliber glands that are placed within an exuberant desmoplastic stromal reaction. A number of histologic patterns have been described, and the majority of these patterns are genetically and biologically related to conventional ductal adenocarcinomas. In this report we describe our experience with a heretofore undescribed histologic pattern of pancreatic adenocarcinoma that mimics intraductal papillary mucinous carcinoma, both morphologically and radiologically.Methods: We identified 10 cases of pancreatic adenocarcinoma with large-caliber malignant glands and an intraluminal papillary pattern. The demographic, clinical, radiologic, and outcome data were recorded. In addition to a review of the histologic features we also performed elastin stains, immunohistochemistry for selected oncogenes and tumor suppressor genes including SMAD4. Immunohistochemical staining for MUC proteins was also performed.Results: The median age of the patients was 67 years, and there were 6 women and 4 men. Grossly, the cut surface in 6 of these cases showed an admixture of solid and cystic areas. The papillary cystic architecture was intimately mixed with areas of conventional adenocarcinoma, the latter characterized by invasive small-caliber tubular structures. None of the tumors showed a pure papillary cystic pattern; however, in 8 cases, this was the predominant pattern (> 50% of the tumor). The cysts and papillae were lined predominantly by tall columnar hypermucinous epithelium. Elastin fibers were not identified around these dilated malignant cysts and glands. The intratumoral stroma was paucicellular and hyalinized. Seven of the 10 tumors were negative for SMAD4. The lack of pericystic elastin fibers and loss of SMAD4 in the majority of cases argue against these lesions representing an intraductal papillary mucinous neoplasm. All 10 tumors stained for MUC1; focal MUC2 reactivity was noted in 1 case. The majority of cases were positive for MUC5AC (9/10) and MUC6 (8/10). Seven patients died of their disease, whereas 1 patient is alive with widely metastatic disease. Two patients were lost to follow up.Conclusions: The adenocarcinoma described herein is a unique morphologic pattern of pancreatic ductal adenocarcinoma. The biology and genetics (as estimated by immunohistochemistry) are no different from that of conventional ductal adenocarcinoma but are distinctly different from that of an intraductal papillary mucinous carcinoma, its closest morphologic mimic.