Soft tissue
Perivascular epithelioid cell
PEComa - general

Author: Vijay Shankar, M.D. (see Authors page)

Revised: 22 September 2016, last major update November 2012

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: PEComa [title] soft tissue

See also: sclerosing PEComa

Cite this page: PEComa - general. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/softtissuepecgeneral.html. Accessed December 6th, 2016.
Definition / General
  • Mesenchymal tumor with perivascular clear cell and epithelioid features that coexpresses melanocytic and muscle markers
  • Concept first proposed by Bonetti (Am J Surg Pathol 1992;16:307)
  • Tumor family includes angiomyolipoma (renal and extrarenal), clear cell "sugar" tumor (lung and extrapulmonary) and lymphangioleiomyomatosis; these tumors are relatively common and are associated with tuberous sclerosis
  • Family also includes tumors of falciform ligament / ligamentum teres, skin (Histopathology 2005;46:498), uterus (Mod Pathol 2005;18:1336) and other viscera and soft tissue; these tumors are rare, and are not associated with tuberous sclerosis
  • No known normal counterpart to the perivascular epithelioid cell
Epidemiology
Prognostic Factors
  • Usually benign, but some cases have malignant behavior and simulate high grade sarcoma (Pathology 2006;38:415)
  • Poor prognostic factors: tumor size > 5 - 8 cm, infiltrative growth pattern, high nuclear grade, > 1 mitotic figure / 50 HPF or atypical mitotic figures, coagulative cell necrosis (Am J Surg Pathol 2005;29:1558)
Case Reports
Treatment
  • Excision is usually curative if tumors are benign
Clinical Images

Images hosted on PathOut servers:

Cutaneous, courtesy of Mark R. Wick, M.D.

Gross Images

Images hosted on PathOut servers:

Buttocks, courtesy of Mark R. Wick, M.D.



Images hosted on other servers:

Uterus: polypoid gray-white mass

Uterus: polypoid mass

Micro Description
  • Perivascular tumor cells may have radial arrangement around lumen; epithelioid cells (closest to vessel) and spindle cells (remote from vessel) with clear to granular eosinophilic cytoplasm; small, central, round / oval nuclei with small nucleoli; often multinucleated giant cells; may have malignant features with marked atypia, mitotic activity and necrosis
  • Pecosis: continuous layer of perivascular clear cells remote from tumor, transitioning to invasive nests and PEComa; cells are in apposition to and in direct contact with abluminal surface of capillary basal lamina (Virchows Arch 2007;450:463)
  • Pecomatosis: nests of perivascular clear to eosinophilic cells (World J Surg Oncol 2004;2:35); may simulate mesothelioma (Ann Diagn Pathol 2006;10:352)
Micro Images

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Images hosted on PathOut servers:
Soft tissue (Case of the Week #283):



Alpha smooth muscle actin

HMB45 - low power

HMB45 - high power



Soft tissue courtesy of Mark R. Wick, M.D.:

Angiomyolipoma



Cutaneous



Images hosted on other servers:
Bladder:

Epithelioid cells

MelanA+

Smooth muscle actin+



Cervix:

Mass with central circular core

Infiltrative border

Spindled to epithelioid areas

Degenerative atypia

Pecomatosis



Ovarian hila

HMB45+



Head and neck:

Nasal PEComa

Larynx



Uterus:

Malignant epithelioid cells

Actin+ (red) and HMB45+ (brown)

HMB45+ recurrent tumor

Positive Stains
Negative Stains
Electron Microscopy Description
  • Abundant cytoplasmic glycogen, premelanosomes, thin filaments with occasional dense bodies, hemidesmosomes, poorly formed cellular junctions
Molecular / Cytogenetics Description
  • Gross chromosomal aberrances in most / all cases; most frequent imbalances are 19-, 16p-, 17p-, 1p-, 18p-, X+, 12q+, 3q+, 5+, 2q+; 16p- indicates loss of TSC2 gene (Hum Pathol 2006;37:606)
Differential Diagnosis
  • Clear cell / oxyphilic carcinoma: cytokeratin+
  • Epithelioid / clear cell smooth muscle tumors: HMB45-
  • Melanoma: strong S100+
  • Undifferentiated / high grade sarcoma