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纖毛黏液結節性乳突狀瘤/細支氣管腺瘤

摘要


纖毛黏液結節性乳突狀瘤(ciliated muconodular papillary tumors, CMPT)是一種罕見的肺腫瘤。它最常見於年紀50至70多歲的患者,通常透過電腦斷層掃描意外發現。典型微觀特徵是纖毛柱狀、粘液和基底細胞所構成,具有乳突狀結構。然而,後續的研究發現這類腫瘤存在更廣泛的形態表現,第5版世界衛生組織胸腔腫瘤分類採用細支氣管腺瘤(bronchiol aradenoma, BA)的嶄新名稱,以總括擁有支氣管管腔細胞及基底細胞雙層分化的所有腫瘤。近來針對CMPT/BA的研究上發現了一系列相關分子變化包括:BRAF、EGFR、AKT1、KRAS和HRAS基因突變,以及ALK基因重排。此類腫瘤的臨床病程和緩,接受手術切除的病例中沒有復發或轉移的發生。本文中我們將回顧CMPTs/BAs的臨床,放射學,病理特徵和分子特徵。

參考文獻


Zheng Q, Luo R, Jin Y, et al. So-called "non-classic" ciliated muconodular papillary tumors: a comprehensive comparison of the clinicopathological and molecular features with classic ciliated muconodular papillary tumors. Hum Pathol 2018;82:193-201.
Lau KW, Aubry MC, Tan GS, et al. Ciliated muconodular papillary tumor: a solitary peripheral lung nodule in a teenage girl. Hum Pathol 2016;49:22-6.
Lu YW, Yeh YC. Ciliated Muconodular Papillary Tumors of the Lung. Arch Pathol Lab Med 2019;143:135-9.
Liu L, Aesif SW, Kipp BR, et al. Ciliated Muconodular Papillary Tumors of the Lung Can Occur in Western Patients and Show Mutations in BRAF and AKT1. Am J Surg Pathol 2016;40:1631-6.
Shao K, Wang Y, Xue Q, et al. Clinicopathological features and prognosis of ciliated muconodular papillary tumor. J Cardiothorac Surg 2019;14:143.

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