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肺错构瘤中发生的肺平滑肌肉瘤:罕见肿瘤中罕见的发生情况。

Pulmonary leiomyosarcoma arising in pulmonary hamartoma: an exceptional occurrence in a rare tumor.

机构信息

Pathology Unit, Department of Mental and Physical Health and Preventive Medicine, Università degli Studi della Campania "L. Vanvitelli", Naples, Italy.

Department of Precision Medicine, Università degli Studi della Campania "Luigi Vanvitelli", Naples, Italy.

出版信息

Pathologica. 2023 Dec;115(6):325-332. doi: 10.32074/1591-951X-941.

DOI:10.32074/1591-951X-941
PMID:38180140
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10767797/
Abstract

A solitary peripheral lung nodule was found in the left lung of a 52-year-old man. It was located in the lower lobe and measured 18.5 cm of major axis on chest computed tomography. A tru-cut core biopsy was obtained and a proliferation of bland, monomorphic, spindle cells in interlacing fascicles was observed. Accordingly, a surgical resection of the neoplasm was subsequently carried out. Macroscopically, the tumor appeared as a well-circumscribed nodule with a firm and whitish cut surface. Histologically, the neoplasm was predominantly composed of bland and monomorphic spindle cells, with a predominantly fascicular growth pattern, in which many tubular and cleft-like spaces of entrapped normal respiratory epithelium were involved. Myxoid change, stromal hyalinization and scattered bizarre mononucleated and multinucleated cells were also observed. Based on clinico-morphological, immunophenotypical and molecular features, we made a diagnosis of malignant transformation of pulmonary adenoleiomyomatous hamartoma into pulmonary leiomyosarcoma. As far as we know, this is the first described case of this exceptionally rare occurrence in an already rare neoplasm.

摘要

一名 52 岁男性的左肺中发现一个孤立性外周肺结节。它位于下叶,胸部计算机断层扫描测量其长轴为 18.5 厘米。进行了 tru-cut 核心活检,观察到交织束状中良性、单形性、梭形细胞的增殖。随后对肿瘤进行了手术切除。大体上,肿瘤呈边界清楚的结节状,切面坚实,呈白色。组织学上,肿瘤主要由良性、单形性梭形细胞组成,以束状生长方式为主,其中涉及许多包裹正常呼吸上皮的管状和裂隙样空间。黏液样变、基质玻璃样变和散在的奇异单核和多核细胞也观察到。基于临床形态学、免疫表型和分子特征,我们诊断为肺腺泡性平滑肌瘤病样错构瘤恶变为肺平滑肌肉瘤。据我们所知,这是首例在已经罕见的肿瘤中如此罕见的情况下发生的这种情况。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/56e2/10767797/3e9153efefd2/pathol-2023-06-325-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/56e2/10767797/ccbdbcc3d05f/pathol-2023-06-325-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/56e2/10767797/7f0e1948351e/pathol-2023-06-325-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/56e2/10767797/dbd548642f30/pathol-2023-06-325-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/56e2/10767797/b736325ff426/pathol-2023-06-325-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/56e2/10767797/3e9153efefd2/pathol-2023-06-325-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/56e2/10767797/ccbdbcc3d05f/pathol-2023-06-325-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/56e2/10767797/7f0e1948351e/pathol-2023-06-325-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/56e2/10767797/dbd548642f30/pathol-2023-06-325-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/56e2/10767797/b736325ff426/pathol-2023-06-325-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/56e2/10767797/3e9153efefd2/pathol-2023-06-325-g005.jpg

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