Primary retroperitoneal PEComa: an incidental finding

Perivascular epithelioid cell neoplasm (PEComa) is a rare type of tumour, and primary retroperitoneal PEComa is rarer still. Although pulmonary lymphangioleiomyomatosis (LAM), angiomyolipomas and clear cell 'sugar' tumours of the lung are well described, relatively little is known about ot...

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Bibliographic Details
Published in:	BMJ case reports Vol. 15; no. 11; p. e250466
Main Authors:	Marinho, Bárbara Monteiro, Canha, António Gâmboa, Silva, Donzília Sousa, Silva, José Davide Pinto
Format:	Journal Article
Language:	English
Published:	England BMJ Publishing Group LTD 11-11-2022 BMJ Publishing Group
Subjects:	Adrenal glands Angiomyolipoma Case reports Case Reports: Rare disease Chest tubes Emergency medical care Female Genes Humans Incidental Findings Lymphangioleiomyomatosis - pathology Lymphangiomyoma Medical imaging Melanoma Middle Aged Perivascular Epithelioid Cell Neoplasms - diagnostic imaging Perivascular Epithelioid Cell Neoplasms - surgery Pneumothorax Smooth muscle Thoracic surgery Transcription factors Tumors Pneumothorax Lung function Surgical oncology General surgery
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Summary:	Perivascular epithelioid cell neoplasm (PEComa) is a rare type of tumour, and primary retroperitoneal PEComa is rarer still. Although pulmonary lymphangioleiomyomatosis (LAM), angiomyolipomas and clear cell 'sugar' tumours of the lung are well described, relatively little is known about other members of the PEComa family. We describe a case of an asymptomatic retroperitoneal PEComa, lymphangioleiomyoma type, which appeared in a previously healthy middle-aged woman as an incidental finding, in a CT scan performed in the context of spontaneous pneumothorax. The patient underwent surgical excision of the tumour and the histopathological and immunohistochemical analysis of the surgical specimen made the definitive diagnosis. Although rare, reports of isolated retroperitoneal lymphangioleiomyoma and primary retroperitoneal PEComas NOS (not otherwise specified) are described in the literature, normally associated with pulmonary LAM. The patient's pulmonary imaging was normal. Short-term re-examination did not detect any recurrence. We also provide a literature review of this rare group of tumours.
Bibliography:	ObjectType-Case Study-2 SourceType-Scholarly Journals-1 ObjectType-Feature-4 content type line 23 ObjectType-Report-1 ObjectType-Article-3
ISSN:	1757-790X 1757-790X
DOI:	10.1136/bcr-2022-250466