Klimstra D S, Rosai J, Heffess C S
Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, New York 10021.
Am J Surg Pathol. 1994 Aug;18(8):765-78. doi: 10.1097/00000478-199408000-00002.
Acinar cell carcinoma is a rare pancreatic neoplasm that may contain scattered endocrine cells in as many as 40% of cases. In addition, unusual tumors exist in which the acinar and endocrine components each constitute a significant proportion (> 25%) of the neoplasm; we propose to designate them as "mixed acinar-endocrine carcinomas." In a study of five such cases, we found one case with segregated areas of acinar and endocrine cells that were identifiable in routinely stained sections and four cases with morphologically uniform cell populations where the divergent differentiation was only detected immunohistochemically. The tumors occurred in adults (age range, 48-81; mean, 68); there were two men and three women. None of the patients presented with symptoms related to either enzyme or hormone liberation. Histologically, the tumors were very cellular; various combinations of solid, trabecular, acinar, and glandular growth patterns were noted. The cells contained d-PAS-positive granules and showed immunohistochemical positivity for pancreatic enzymes (trypsin, chymotrypsin, and lipase) and endocrine markers (chromogranin and synaptophysin); specific endocrine hormones were found in two cases. Double immunohistochemical staining for acinar and endocrine markers showed that most cells expressed only one line of differentiation. Ultrastructural study of two cases showed two populations of granules. Two of the patients died of their tumors (mean survival, 10.5 months), one with widespread metastases. Two patients were alive with disease at 12 months after diagnosis, and one patient was lost to follow-up after 3 months. This rare type of pancreatic neoplasm provides further evidence of the close histogenetic relationship between the exocrine and endocrine components of this organ.
腺泡细胞癌是一种罕见的胰腺肿瘤，多达40%的病例中可能含有散在的内分泌细胞。此外，还存在一些特殊肿瘤，其中腺泡和内分泌成分在肿瘤中各占相当大的比例（>25%）；我们建议将它们命名为“腺泡-内分泌混合癌”。在一项对5例此类病例的研究中，我们发现1例在常规染色切片中可识别出腺泡和内分泌细胞分离的区域，4例细胞形态均匀，仅通过免疫组化检测到不同的分化。肿瘤发生于成年人（年龄范围48 - 81岁，平均68岁）；2例男性，3例女性。所有患者均未出现与酶或激素释放相关的症状。组织学上，肿瘤细胞丰富；可见实性、小梁状、腺泡状和腺管状生长模式的各种组合。细胞含有d-PAS阳性颗粒，对胰腺酶（胰蛋白酶、糜蛋白酶和脂肪酶）和内分泌标志物（嗜铬粒蛋白和突触素）呈免疫组化阳性；2例发现了特定的内分泌激素。腺泡和内分泌标志物的双重免疫组化染色显示，大多数细胞仅表达一种分化谱系。2例病例的超微结构研究显示有两种颗粒群。2例患者死于肿瘤（平均生存期10.5个月），1例有广泛转移。2例患者在诊断后12个月仍患有疾病存活，1例患者在3个月后失访。这种罕见的胰腺肿瘤进一步证明了该器官外分泌和内分泌成分之间密切的组织发生关系。
