Primary cutaneous perivascular epithelioid cell tumor (PEComa): Five new cases and review of the literature

Lauren N. Stuart, Russell G. Tipton, Michael R. DeWall, Douglas C. Parker, Christina D. Stelton, Annie O. Morrison, Landon W. Coleman, Scott Fosko, Claudia I. Vidal, Maria Yadira Hurley, Amy H. Deeken, Jerad M. Gardner

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

PEComas represent a family of uncommon mesenchymal tumors composed of “perivascular epithelioid cells” with a distinct immunophenotype that typically shows both myogenic and melanocytic differentiation. The PEComa family includes angiomyolipoma (AML), clear cell “sugar” tumor of the lung and extra pulmonary sites, lymphangioleiomyomatosis and clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres. Very rarely, PEComas may arise in the skin. Primary cutaneous PEComas typically display a dermal proliferation of epithelioid cells with pale, clear, or granular pink cytoplasm arranged in nests and trabecula with an intervening arborizing network of delicate capillaries. Primary cutaneous PEComas have a lower frequency of myogenic marker expression than their deep soft tissue and visceral counterparts. They also often express strong diffuse CD10, leading to potential confusion with metastatic renal cell carcinoma. Most cases behave indolently. We report 5 additional cases of this rare entity. All showed classic histologic features and expression of either HMB-45 and/or Melan-A/MART-1. Four cases were tested for myogenic markers (2 were positive & 2 were negative). Three cases were tested for CD10 (all 3 were positive). All of our cases with clinical follow-up behaved indolently. Table 1 provides a summary of findings for all 5 cases in our series.

Original languageEnglish (US)
Pages (from-to)713-721
Number of pages9
JournalJournal of Cutaneous Pathology
Volume44
Issue number8
DOIs
StatePublished - Aug 1 2017

Fingerprint

Perivascular Epithelioid Cell Neoplasms
Skin
Round Ligaments
MART-1 Antigen
Lymphangioleiomyomatosis
Angiomyolipoma
Epithelioid Cells
Lung
Renal Cell Carcinoma
Ligaments
Cytoplasm

Keywords

  • CD10
  • clear cell tumor
  • cutaneous PEComa
  • myomelanocytic
  • perivascular epithelioid cell tumor

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology
  • Dermatology

Cite this

Stuart, L. N., Tipton, R. G., DeWall, M. R., Parker, D. C., Stelton, C. D., Morrison, A. O., ... Gardner, J. M. (2017). Primary cutaneous perivascular epithelioid cell tumor (PEComa): Five new cases and review of the literature. Journal of Cutaneous Pathology, 44(8), 713-721. https://doi.org/10.1111/cup.12972

Primary cutaneous perivascular epithelioid cell tumor (PEComa) : Five new cases and review of the literature. / Stuart, Lauren N.; Tipton, Russell G.; DeWall, Michael R.; Parker, Douglas C.; Stelton, Christina D.; Morrison, Annie O.; Coleman, Landon W.; Fosko, Scott; Vidal, Claudia I.; Yadira Hurley, Maria; Deeken, Amy H.; Gardner, Jerad M.

In: Journal of Cutaneous Pathology, Vol. 44, No. 8, 01.08.2017, p. 713-721.

Research output: Contribution to journalArticle

Stuart, LN, Tipton, RG, DeWall, MR, Parker, DC, Stelton, CD, Morrison, AO, Coleman, LW, Fosko, S, Vidal, CI, Yadira Hurley, M, Deeken, AH & Gardner, JM 2017, 'Primary cutaneous perivascular epithelioid cell tumor (PEComa): Five new cases and review of the literature', Journal of Cutaneous Pathology, vol. 44, no. 8, pp. 713-721. https://doi.org/10.1111/cup.12972
Stuart, Lauren N. ; Tipton, Russell G. ; DeWall, Michael R. ; Parker, Douglas C. ; Stelton, Christina D. ; Morrison, Annie O. ; Coleman, Landon W. ; Fosko, Scott ; Vidal, Claudia I. ; Yadira Hurley, Maria ; Deeken, Amy H. ; Gardner, Jerad M. / Primary cutaneous perivascular epithelioid cell tumor (PEComa) : Five new cases and review of the literature. In: Journal of Cutaneous Pathology. 2017 ; Vol. 44, No. 8. pp. 713-721.
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