Cuilliere Peggy, Lazure Thierry, Bui Matthieu, Fabre Monique, Buffet Catherine, Gayral François, Bedossa Pierre
Service d'Anatomie Pathologique, CHU de Bicêtre,78 rue du Général Leclerc, 94275 Le Kremlin-Bicêtre, France.
Virchows Arch. 2002 Nov;441(5):519-22. doi: 10.1007/s00428-002-0683-0. Epub 2002 Aug 27.
We report a unique, previously unreported pancreatic tumor with hepatoid differentiation associated with serous microcystic adenoma in a 70-year-old man. These two lesions localized, respectively, at the body and the tail of the pancreas, were found incidentally on abdominal ultrasonography. Serum alpha-fetoprotein was not increased and no hepatic lesion was displayed on computed tomography. A subtotal pancreatectomy with splenectomy was performed. The patient is alive and well 12 months after resection. Pathological examination showed a very unusual encapsulated solid tumor with hepatocytic differentiation, bile production and immunoreactivity for hepatocyte paraffin-1 antibody. The tumor cells were negative for endocrine (neuron-specific enolase, chromogranin A, synaptophysin) and acinar (amylase, trypsin) markers. Ultrastructurally, zymogen and neurosecretory granules were absent. The features of the tumor were almost indistinguishable from those of hepatocellular adenoma; therefore, we believe that this solid hepatoid tumor may represent a variant of pancreatic adenoma. Recognition of this entity is important because the only reported pancreatic hepatoid tumors to date have been malignant. The main differential diagnoses include hepatoid ductal adenocarcinoma, hepatoid acinar cell carcinoma, primitive hepatoid endocrine tumor, and metastatic hepatocellular carcinoma.
我们报告了一例70岁男性患者,其胰腺出现一种独特的、此前未报道过的具有肝样分化且与浆液性微囊性腺瘤相关的肿瘤。这两个病变分别位于胰腺体部和尾部,是在腹部超声检查时偶然发现的。血清甲胎蛋白未升高,计算机断层扫描未显示肝脏病变。实施了胰腺次全切除术加脾切除术。患者术后12个月健在。病理检查显示为一个非常特殊的包膜实性肿瘤,具有肝细胞分化、胆汁生成以及对肝细胞石蜡-1抗体呈免疫反应性。肿瘤细胞的内分泌(神经元特异性烯醇化酶、嗜铬粒蛋白A、突触素)和腺泡(淀粉酶、胰蛋白酶)标志物均为阴性。超微结构检查显示无酶原颗粒和神经分泌颗粒。该肿瘤的特征与肝细胞腺瘤几乎无法区分;因此,我们认为这种实性肝样肿瘤可能代表胰腺腺瘤的一种变体。认识到这一实体很重要,因为迄今为止报道的胰腺肝样肿瘤均为恶性。主要鉴别诊断包括肝样导管腺癌、肝样腺泡细胞癌、原始肝样内分泌肿瘤和转移性肝细胞癌。