PEComa en mucosa yugal. Presentación de un caso

Resumen

Los tumores de células perivasculares epitelioides (PEComas) son un grupo heterogéneo de tumores mesenquimales muy infrecuentes, de histogénesis y evolución incierta. Se incluyen el angiomiolipoma (AML), la linfangiomiomatosis (LAM), el tumor de células claras de pulmón (TCCP) y una variedad de tumores de vísceras y partes blandas morfológica e inmunofenotípicamente similares, caracterizados por expresar marcadores melanocíticos (HMB45 y/o Melan-A) y de músculo liso (actina y/o desmina). Se han publicado casos en gran variedad de órganos, tejidos blandos y hueso. En la cavidad oral solo ha sido descrito un único caso en el paladar duro. Se presenta el caso de un niño de 8 años con un PEComa en la mucosa oral de la mejilla. No hemos hallado un caso similar en la literatura.

Abstract

PEComas (Perivascular Epithelioid Cells tumours) are a group of rare mesenchymal tumours whose origins (or line of differentiation) and behaviour are not well defined. Angiomyolipoma (AML), lymphangiomyomatosis (LAM), clear cell “sugar” tumour of the lung (CCST), and a variety of lesions arising at both visceral and soft tissue sites with morphologically and immunophenotypically similar features are included in this group. The main characteristics in common are immunoreactivity for both melanocytic (HMB-45 and/or Melan-A) and smooth muscle (Actin and/or Desmin) markers. These tumours show a marked female predominance and are composed of nest and sheets of epithelioid but occasionally spindled cells with clear to granular eosinophilic cytoplasm. They have focal association with blood vessel walls. They have been reported at visceral sites but also in soft tissues and bone. Only one case in oral cavity arising in palatine mucosa has been described so far. We report a jugal mucosa PEComa in a 8-year-old boy. That is an uncommon location and, to our knowledge, it′s the first case reported in this site to date.