Cutaneous PEComa: diagnostic challenge of an infrequent neoplasm

Authors

  • Juliana Cabrera Cisneros Central Hospital of San Isidro Dr. Melchor Ángel Posse, Province of Buenos Aires, Argentina
  • Cynthia Laura Rossi Central Hospital of San Isidro Dr. Melchor Ángel Posse, Province of Buenos Aires, Argentina
  • María Laura Trotta Central Hospital of San Isidro Dr. Melchor Ángel Posse , Province of Buenos Aires, Argentina
  • Andrea Vanesa Giuliani Central Hospital of San Isidro Dr. Melchor Ángel Posse, Province of Buenos Aires, Argentina

DOI:

https://doi.org/10.47196/da.v29i2.2378

Keywords:

cutaneous PEComa, perivascular epithelioid cell tumor, clear cells

Abstract

Perivascular epithelioid cell tumors (PEComas) are a rare group of mesenchymal neoplasms characterized histologically by proliferating cells with clear cytoplasm, epithelioid appearance, and perivascular arrangement, which have the ability to co-express melanocytic and myogenic immunohistochemical markers. Primary cutaneous PEComas are infrequent, accounting for about 8%.

We present a case of cutaneous PEComa to be considered as a differential diagnosis of diseases that share similar histopathologic and immunohistochemical features such as amelanotic melanoma or metastasis of clear cell renal carcinoma.

Author Biographies

Juliana Cabrera Cisneros, Central Hospital of San Isidro Dr. Melchor Ángel Posse, Province of Buenos Aires, Argentina

Fifth Year Programmatic Concurrent Dermatology Physician, Dermatology Service, Central Hospital of San Isidro Dr. Melchor Ángel Posse

Cynthia Laura Rossi, Central Hospital of San Isidro Dr. Melchor Ángel Posse, Province of Buenos Aires, Argentina

Dermatologist, Dermatology Service, Central Hospital of San Isidro Dr. Melchor Ángel Posse

María Laura Trotta, Central Hospital of San Isidro Dr. Melchor Ángel Posse , Province of Buenos Aires, Argentina

Staff Dermatologist, Dermatology Service, Central Hospital of San Isidro Dr. Melchor Ángel Posse

Andrea Vanesa Giuliani, Central Hospital of San Isidro Dr. Melchor Ángel Posse, Province of Buenos Aires, Argentina

Head of Service, Dermatology Service, Central Hospital of San Isidro Dr. Melchor Ángel Posse

References

I. Stuart LN, Tipton RG, DeWall MR, Parker DC, et ál. Primary cutaneous perivascular epithelioid cell tumor (PEComa): five new cases and review of the literature. J Cutan Pathol. 2017;44:713-721.

II. Charli-Joseph Y, Saggini A, Vemula S, Weier J, et ál. Primary cutaneous perivascular epithelioid cell tumor: a clinicopathological and molecular reappraisal. J Am Acad Dermatol. 2014;71:1127-1136.

III. Kneitz H, Mentzel T, Wobser M, Goebeler M. Cutaneous PEComa of the right lower leg. J Dtsch Dermatol Ges. 2015;13:815-817.

IV. Ieremia E, Robson A. Cutaneous PEComa: a rare entity to consider in an unusual site. Am J Dermatopathol 2014;36:198-201.

V. Odoño EIG, Tan KB, Tay SY, Lee VKM. Cutaneous "fibroma-like" perivascular epithelioid cell tumor: a case report and review of literature. J Cutan Pathol. 2020;47:548-553.

VI. Haiges D, Kurz P, Laaff H, Meiss F, et ál. Malignant PEcoma: case report. J Cutan Pathol. 2018;45:84-89.

VII. Greveling K, Winnepenninckx VJL, Nagtzaam IF, Lacko M, et ál. Malignant perivascular epithelioid cell tumor: a case report of a cutaneous tumor on the cheek of a male patient. J Am Acad Dermatol. 2013;69(5):e262-e264.

VIII. Ueberschaar S, Goebeler M, Kneitz H. CD10-positive cutaneous PEComa: an extremely rare skin tumour. Case Rep Dermatol. 2020;12:192-198.

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Published

2023-08-08

Issue

Vol. 29 No. 2 (2023): May-August 2023

Section

Clinical Cases

License

Copyright (c) 2023 on behalf of the authors. Reproduction rights: Argentine Society of Dermatology

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