Isolated acute hemorrhagic edema of the eyelids

Abstract

Perivascular epithelioid cell tumors (PEComas) are rare tumors which can arise in almost every body site. Little is still known about these tumors because of the limited number of cases described by far and the short follow-ups. Diagnosis, based on histopathological and immunohistochemical features, is not so straightforward owing to their rarity. Prediction of clinical behaviour which can be benign, locally aggressive or purely malignant, is not without flaws. A refinement in the prognostic criteria is desiderable. Even if surgery is considered the mainstay of therapy of both primary PEComa and local recurrences/metastases, doubts still exist regarding the other treatment modalities such as chemotherapy and immunotherapy. The rarity of this neoplasm prompted us to report a case we observed. A left lung PEComa was diagnosed in a 70-year-old lady that we treated with upper left lobectomy. Our aim is to add our experience to the present body of literature and contribute in the understanding of PEComa neoplasms.