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Ciliated muconodular papillary tumors of the lung: a clinicopathologic analysis of 10 cases.

肺的纤毛黏液结节性乳头状肿瘤:10例临床病理分析

Kamata T,Yoshida A,Kosuge T,Watanabe S,Asamura H,Tsuta K

Abstract

Ciliated muconodular papillary tumors (CMPTs) are rare peripheral nodules of the lung first described in 2002. Because of their rarity and nonstandardized diagnostic terminology, CMPTs have been poorly recognized among pathologists. To better characterize these lesions, we undertook a detailed clinicopathologic and immunohistochemical study of 10 archival cases. Ten CMPTs occurred in 7 men and 3 women with a median age of 62 years. All were small peripheral nonendobronchial nodules with a mean diameter of 1.0 cm. All but 1 tumor were incidentally detected by computed tomography-based screening, all of which were radiologically interpreted as adenocarcinomas. Although limited surgery treated all but 1 CMPT, they followed a benign course with no recurrence at a mean follow-up of 43 months (range: 2 to 88 mo). Histologically, CMPTs showed glandular and/or papillary architecture, comprising a vaguely organized mixture of nonatypical ciliated columnar cells, mucous cells, and basal cells, often enveloped by copious intra-alveolar mucin. Micropapillary tufts of ciliated cells and seemingly discontinuous growth along alveolar walls were occasionally present, mimicking adenocarcinomas. Ciliated cells and basal cells were immunopositive for TTF-1 and p40, respectively, whereas mucous cells lacked HNF4α expression. CMPTs are rare, likely benign, underrecognized processes of the lung that should be distinguished from adenocarcinomas.

摘要

纤毛黏液结节性乳头状肿瘤(CMPTs)是一种罕见的肺外周结节,2002年首次报道。因为罕见且缺乏诊断标准,病理学家一直未能很好地识别CMPTs。

为了更好地描述这一病变,我们对档案中的10个病例进行了详细的临床病理和免疫组化研究。

10例CMPTs中男性7名,女性3名,中位年龄62岁。全部都是外周非支气管内的小结节,平均直径1.0厘米。除1例外,其余全部是CT检查时偶然发现的,所有的病例影像学诊断为腺癌。尽管除1例外其余全部进行了局部切除,但CMPT表现出良性的生物学行为,平均随访43个月无复发(随访时间范围:2—88个月)。

组织学上,CMPTs显示腺样和/或乳头结构,非典型纤毛柱状细胞、黏液细胞及基底细胞有序混合分布,常被肺泡内大量的黏液包围。偶尔会出现纤毛细胞形成的微乳头簇,沿肺泡壁呈不连续的生长分布,类似于腺癌。纤毛细胞和基底细胞免疫组化染色分别TTF-1阳性和p40阳性,而黏液细胞缺乏HNF4α的表达。

CMPTs是一种罕见、良性可能、没有得到很好识别的肺病变,应该与腺癌区别开来。

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