Galateau-Sallé Françoise, Vignaud Jean Michel, Burke Louise, Gibbs Allen, Brambilla Elisabeth, Attanoos Richard, Goldberg Marcel, Launoy Guy
Department of Pathology CHU Caen, France.
Am J Surg Pathol. 2004 Apr;28(4):534-40. doi: 10.1097/00000478-200404000-00013.
Well-differentiated papillary mesothelioma (WDPM) of the pleura represents a distinct mesothelial tumor presenting with unilateral pleural effusion and superficial spreading of stout papillary formations with myxoid cores, lined by bland, flattened, or epithelioid cells, without or with limited invasion of the submesothelial layer. The majority of cases have been reported in the peritoneum in women of reproductive age with no history of asbestos exposure and also in the tunica vaginalis of men. We report 24 cases of pleural WDPM and compared their histologic, epidemiologic, and clinical features with those of classic mesothelioma. Men and women were equally affected, with a mean age of 60 years. Half of the patients had a history of occupational asbestos exposure. In 11 patients with a minimal follow-up period of 24 months, the survival ranged from 36 to 180 months with an average of 74 months as compared with 9.89 months for 1248 paired patients with diffuse malignant mesothelioma. Ten-year survival was 30.8%. We conclude that WDPM is a rare and unusual mesothelial tumor, characterized by a lack of deep invasion and associated with an indolent clinical course and long survival. For these reasons, WDPM is best considered as a specific clinico-pathologic entity distinct from conventional diffuse malignant mesothelioma.
胸膜高分化乳头状间皮瘤（WDPM）是一种独特的间皮肿瘤，表现为单侧胸腔积液以及带有黏液样核心的粗大乳头状结构的浅表播散，其表面衬覆着形态温和、扁平或上皮样的细胞，不累及或仅有有限的亚间皮层浸润。大多数病例报道于无石棉接触史的育龄期女性的腹膜，以及男性的睾丸鞘膜。我们报告了24例胸膜WDPM病例，并将其组织学、流行病学和临床特征与经典间皮瘤进行了比较。男性和女性受影响程度相同，平均年龄为60岁。半数患者有职业性石棉接触史。在11例随访期最短为24个月的患者中，生存期为36至180个月，平均74个月，而1248例配对的弥漫性恶性间皮瘤患者的生存期为9.89个月。十年生存率为30.8%。我们得出结论，WDPM是一种罕见且不寻常的间皮肿瘤，其特征为缺乏深层浸润，临床病程惰性，生存期长。基于这些原因，WDPM最好被视为一种有别于传统弥漫性恶性间皮瘤的特定临床病理实体。
