RESUMO
The Perivascular Epithelioid Cell tumours (PEComas) are rare mesenchymal neoplasms recognized as entity by the World Health Organization. The tumour cells have an uncertain origin and are characterized by distinctive histological and immunohistochemical features. We report a case of PEComa occurring as intraorbital lesion in a 47-year-old man. We found only two other cases described in the literature and we considered all three cases together in order of histology, immunohistochemistry, and clinical outcome. We found a strict histological overlapping and quite similar immunohistological results. All three cases showed a favourable clinical course probably related to small size of tumours (<5 cm), low mitotic rate (<2 mitoses in 50 HPF), and absence of necrosis.
RESUMO
El Angiomixoma agresivo es una neoplasia de diferenciación incierta descrita por primera vez por Steeper y Rosai (1983), que tiene predilección por la región pélvica y perineal de mujeres entre la 3ra y la 6ta década de la vida, afectando con menor frecuencia al sexo masculino, con casos descritos de localización escrotal. En Venezuela hasta la fecha, no hay casos informados de este tumor en pacientes masculinos. Presentamos un caso de localización pélvica, confirmado mediante estudios de inmuno-histoquímica en un varón de 27 años de edad con una recidiva luego de 3 años de la exéresis inicial.
Aggressive angiomyxoma is a neoplasm of uncerta in differentiation first described by Steeper and Rosai (1983), which has a predilection for the pelvic and perineal region of females between the 3rd and 6th decade of life, affecting males less frequently, with reported cases of scrotal location. In Venezuela, to date, no cases were reported in male patients. A case of pelvic location we presented, confirmed by immunohistochemistry studies in a male of 27 years-old with a relapse after three years of the initial excision.
