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Case Report
A case report on malignant perivascular epithelioid cell neoplasm of the thigh, a rare presentation in the skin
1 Department of Internal Medicine, The Brooklyn Hospital Center, 121 De Kalb Avenue, Brooklyn, NY 11201, USA
2 Division of Hematology-Oncology, The Brooklyn Hospital Center, 121 De Kalb Avenue, Brooklyn, NY 11201, USA
3 Department of Pathology, The Brooklyn Hospital Center, 121 De Kalb Avenue, Brooklyn, NY 11201, USA
Address correspondence to:
Samridhi Sinha
Department of Internal Medicine, The Brooklyn Hospital Center, 121 De Kalb Avenue, Brooklyn, NY 11201,
USA
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Article ID: 100083Z10SS2021
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Sinha S, Rathnakumar GR, Guevara E, Baqui A. A case report on malignant perivascular epithelioid cell neoplasm of the thigh, a rare presentation in the skin. J Case Rep Images Oncology 2021;7:100083Z10SS2021.ABSTRACT
Perivascular epithelioid cell neoplasms (PEComa) are a family of mesenchymal tumors characterized by perivascular epithelioid cells with co-expression of melanocytic and muscle markers and can occur in any part of the body. These tumors are almost always positive for HMB-45 and smooth muscle actin (SMA) immunochemistry. Perivascular epithelioid cell neoplasms tend to arise in middle age, and there is a marked female predominance. The tumors most commonly occur in the gynecologic tract, pelvis, and retroperitoneum. They are of varying malignant potential and are occasionally associated with tuberous sclerosis complex, a rare tumor driven by tuberous sclerosis complex gene mutations TSC1/2, causing up-regulation of mTOR. We present a case of malignant perivascular epithelioid cell neoplasm of the thigh, which is an exceedingly rare presentation in the skin.
Keywords: Epithelioid features, HMB-45, Melan-A, Mesenchymal tumors, PEComa, Perivascular, Perivascular epithelioid cell neoplasms, Wide resection
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Author Contributions
Samridhi Sinha - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Final approval of the version to be published
Geethapriya Rajasekharan Rathnakumar - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Elizabeth Guevara - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Aam Baqui - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Guaranter of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2021 Samridhi Sinha et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
