Head and Neck Pathology

, Volume 5, Issue 3, pp 233–240

Perivascular Epithelioid Cell Tumors (PEComas) of the Head and Neck: Report of Three Cases and Review of the Literature

  • Anshu Bandhlish
  • E. Leon Barnes
  • Joseph T. Rabban
  • Jonathan B. McHugh
Original Paper

DOI: 10.1007/s12105-011-0268-9

Cite this article as:
Bandhlish, A., Leon Barnes, E., Rabban, J.T. et al. Head and Neck Pathol (2011) 5: 233. doi:10.1007/s12105-011-0268-9

Abstract

PEComas are a family of neoplastic lesions that share overlapping morphology, immunohistochemistry, and ultrastructure that include angiomyolipoma, lymphangioleiomyomatosis, clear cell “sugar” tumor of the lung as well as similar tumors occurring in a variety of visceral, cutaneous and soft tissue sites throughout the body. The defining histopathological features are epithelioid cells with a perivascular distribution containing clear to pale eosinophilic granular cytoplasm and a round-to-oval centrally located nucleus with an inconspicuous nucleolus. Immunohistochemically, coexpression of melanocytic (HMB-45 and/or Melan-A) and myoid markers are characteristic. In the present study, we describe three PEComas occurring in the head and neck (nasal cavity and larynx) and discuss the behavior of these distinctive tumors and review the literature of head and neck PEComas. The importance of recognizing this entity will ensure its consideration in the differential diagnosis of tumors of the head and neck with a similar morphology. The histogenesis of PEComas still remains elusive and additional cases with a prolonged follow up remain important to accurately determine the behavior of these distinctive tumors. Complete surgical excision still remains the treatment of choice for histologically benign PEComas.

Keywords

Perivascular epithelioid cell tumorPEComaAngiomyolipoma

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  • Anshu Bandhlish
    • 1
  • E. Leon Barnes
    • 2
  • Joseph T. Rabban
    • 3
  • Jonathan B. McHugh
    • 1
  1. 1.University of Michigan Health SystemAnn ArborUSA
  2. 2.University of Pittsburgh Medical CenterPittsburghUSA
  3. 3.University of California San FranciscoSan FranciscoUSA