Thompson L D, Becker R C, Przygodzki R M, Adair C F, Heffess C S
Department of Endocrine and Otorhinolaryngic-Head & Neck Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.
Am J Surg Pathol. 1999 Jan;23(1):1-16. doi: 10.1097/00000478-199901000-00001.
Mucinous cystic neoplasms (MCNs) of the pancreas are uncommon tumors. The classification and biologic potential of these neoplasms remain the subject of controversy. Attempts to classify these tumors in a similar manner to ovarian MCNs remains controversial, as even histologically benign-appearing pancreatic MCNs metastasize and are lethal. One hundred thirty cases of MCNs were identified in the files of the Endocrine Pathology Tumor Registry of the Armed Forces Institute of Pathology from the years 1979 to 1993. The pathologic features, including hematoxylin and eosin staining, histochemistry, immunohistochemistry (IHC), cell cycle analysis, and K-ras oncogene determination were reviewed. These findings were correlated with the clinical follow-up obtained in all cases. There were 130 women, aged 20-95 years (mean age at the outset, 44.6 years). The patients had vague abdominal pain, fullness, or abdominal masses. More than 95% of the tumors were in the pancreatic tail or body and were predominantly multilocular. The tumors ranged in size from 1.5 to 36 cm in greatest dimension, with the average tumor measuring >10 cm. A spectrum of histomorphologic changes were present within the same case and from case to case. A single layer of bland-appearing, sialomucin-producing columnar epithelium lining the cyst wall would abruptly change to a complex papillary architecture, with and without cytologic atypia, and with and without stromal invasion. Ovarian-type stroma was a characteristic and requisite feature. Focal sclerotic hyalinization of the stroma was noted. This ovarian-type stroma reacted with vimentin, smooth muscle actin, progesterone, or estrogen receptors by IHC analysis. There was no specific or unique epithelial IHC. K-ras mutations by sequence analysis were wild type in all 52 cases tested. Ninety percent of patients were alive or had died without evidence of disease (average follow-up 9.5 years), irrespective of histologic appearance; 3.8% were alive with recurrent disease (average 10 years after diagnosis); and 6.2% died of disseminated disease (average 2.5 years from diagnosis). Irrespective of the histologic appearance of the epithelial component, with or without stromal invasion, pancreatic MCNs should all be considered as mucinous cystadenocarcinomas of low-grade malignant potential. Pancreatic MCNs cannot be reliably or reproducibly separated into benign, borderline, or malignant categories.
胰腺黏液性囊性肿瘤（MCNs）是罕见肿瘤。这些肿瘤的分类和生物学潜能仍是争议焦点。试图以类似于卵巢MCNs的方式对这些肿瘤进行分类仍存在争议，因为即使组织学上看似良性的胰腺MCNs也会发生转移且具有致命性。在武装部队病理研究所内分泌病理肿瘤登记处1979年至1993年的档案中识别出130例MCNs。回顾了其病理特征，包括苏木精和伊红染色、组织化学、免疫组织化学（IHC）、细胞周期分析以及K-ras癌基因测定。这些发现与所有病例的临床随访结果相关。有130名女性，年龄在20至95岁之间（初始平均年龄44.6岁）。患者有模糊的腹痛、饱胀感或腹部肿块。超过95%的肿瘤位于胰尾或胰体，且多为多房性。肿瘤最大直径在1.5至36厘米之间，平均肿瘤大小大于10厘米。同一病例以及不同病例之间存在一系列组织形态学变化。囊肿壁内衬一层外观温和、产生涎黏蛋白的柱状上皮会突然转变为复杂的乳头结构，伴有或不伴有细胞异型性，伴有或不伴有间质浸润。卵巢型间质是一个特征性且必备的特征。注意到间质有局灶性硬化透明变性。通过IHC分析，这种卵巢型间质对波形蛋白、平滑肌肌动蛋白、孕激素或雌激素受体有反应。不存在特异性或独特的上皮IHC。在所有52例检测病例中，通过序列分析K-ras突变均为野生型。90%的患者存活或死于无疾病证据（平均随访9.5年），无论组织学表现如何；3.8%的患者存活但有复发性疾病（诊断后平均10年）；6.2%的患者死于播散性疾病（诊断后平均2.5年）。无论上皮成分的组织学表现如何，无论有无间质浸润，胰腺MCNs均应全部视为低级别恶性潜能的黏液性囊腺癌。胰腺MCNs无法可靠且可重复地分为良性、交界性或恶性类别。
