Wilentz R E, Albores-Saavedra J, Hruban R H
Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, MD 21287, USA.
Semin Diagn Pathol. 2000 Feb;17(1):31-42.
Since their initial description, mucinous cystic neoplasms have been difficult to classify. This article attempts to clarify histological, clinical, and genetic criteria so that the pathologist can categorize each mucinous cystic neoplasm into 1 of 4 possible categories. Mucinous cystadenomas contain a single layer of mucin-producing, columnar epithelium lacking significant atypia. Borderline mucinous cystic neoplasms contain cells with moderate atypia. Mucinous cystic neoplasms with in situ carcinoma show significant architectural and cytological atypia. When invasive carcinoma is present in association with a mucinous cystic neoplasm, then the diagnosis of invasive mucinous cystadenocarcinoma should be made. The categorization of mucinous cystic neoplasms into these groups is essential because it accurately predicts outcome, provided that the tumor has been sampled and examined thoroughly. Completely removed mucinous cystadenomas, borderline mucinous cystic neoplasms, and mucinous cystic neoplasms with in situ carcinoma follow benign courses. Partial resection should be avoided as evidence suggests that mucinous cystic neoplasms can progress from adenomas to borderline lesions to carcinomas in situ to invasive carcinomas over time; partial resection should be avoided if possible. Modern molecular genetic techniques are helping to unravel the origins of rare variants of mucinous cystic tumors, such as the mucinous cystic tumor with an associated osteoclast-like giant cell tumor and the mucinous cystic tumor with sarcomatous stroma.
自首次描述以来,黏液性囊性肿瘤一直难以分类。本文试图阐明组织学、临床和遗传学标准,以便病理学家能够将每个黏液性囊性肿瘤归入4种可能类别中的1种。黏液性囊腺瘤由单层产生黏液的柱状上皮组成,缺乏明显异型性。交界性黏液性囊性肿瘤含有中度异型性细胞。伴有原位癌的黏液性囊性肿瘤表现出明显的结构和细胞学异型性。当黏液性囊性肿瘤伴有浸润性癌时,则应诊断为浸润性黏液性囊腺癌。将黏液性囊性肿瘤分为这些组至关重要,因为只要对肿瘤进行了充分取材和检查,就能准确预测其预后。完全切除的黏液性囊腺瘤、交界性黏液性囊性肿瘤和伴有原位癌的黏液性囊性肿瘤预后良好。应避免部分切除,因为有证据表明黏液性囊性肿瘤可能会随着时间的推移从腺瘤发展为交界性病变、原位癌再到浸润性癌;如果可能,应避免部分切除。现代分子遗传学技术有助于揭示黏液性囊性肿瘤罕见变异型的起源,如伴有破骨细胞样巨细胞瘤的黏液性囊性肿瘤和伴有肉瘤样间质的黏液性囊性肿瘤。