Radhi J M, Lukie B E
Department of Pathology, College of Medicine, University of Saskatchewan, Royal University Hospital, Saskatoon, Canada.
J Clin Pathol. 1998 Nov;51(11):865-7. doi: 10.1136/jcp.51.11.865.
Ductal adenocarcinoma is the most common type of pancreatic carcinoma while squamous, carcinosarcoma, sarcoma, giant cell carcinoma, and clear cell types are all rare. Hepatocellular fibrinogen storage disease is also an uncommon disorder which may be associated with hepatocellular carcinoma. Two cases of pancreatic carcinoma were encountered in a family with fibrinogen storage disease, further raising the possibility of a predilection to malignancy in this unusual disorder. The tumour in one case was of the rare clear cell type. These two cases are the basis for this report.
Sections were cut from retrieved paraffin embedded tissue and stained for routine histology. Immunohistochemistry using the avidin-biotin technique was applied for the expression of the markers p53 (D07), carcinoembryonic antigen (CEA), c-erbB-2, epithelial membrane antigen (EMA), and alpha-fetoprotein (AFP).
Both cases were adenocarcinoma of pancreatic ductal origin. The tumour in one case showed features of a clear cell carcinoma. The tumour cells expressed p53, CEA, and EMA immunoreactivity and were negative for c-erbB-2 and AFP.
Hepatocellular fibrinogen storage disease is rare and has been described in association with chronic hepatitis, cirrhosis, and rarely with hepatocellular carcinoma. This represents the first report of its association with carcinoma outside of the liver.
导管腺癌是胰腺癌最常见的类型，而鳞状细胞癌、癌肉瘤、肉瘤、巨细胞癌和透明细胞型均较为罕见。肝细胞纤维蛋白原贮积病也是一种不常见的疾病，可能与肝细胞癌有关。在一个患有纤维蛋白原贮积病的家族中发现了两例胰腺癌，这进一步增加了这种罕见疾病易患恶性肿瘤的可能性。其中一例肿瘤为罕见的透明细胞型。这两例病例是本报告的基础。
从回收的石蜡包埋组织中切片，进行常规组织学染色。采用抗生物素蛋白-生物素技术进行免疫组织化学检测，以检测p53（D07）、癌胚抗原（CEA）、c-erbB-2、上皮膜抗原（EMA）和甲胎蛋白（AFP）等标志物的表达。
两例均为导管起源的胰腺腺癌。其中一例肿瘤表现为透明细胞癌特征。肿瘤细胞表达p53、CEA和EMA免疫反应性，而c-erbB-2和AFP为阴性。
肝细胞纤维蛋白原贮积病罕见，已被描述与慢性肝炎、肝硬化相关，很少与肝细胞癌相关。这是其与肝外癌症相关的首次报告。
