Recurrence of retroperitoneal localized perivascular epithelioid cell tumor two years after initial diagnosis: case report

Abstract

CONTEXT: Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors. Adjuvant radiotherapy and/or chemotherapy are administered according to the patient’s clinical characteristics. CASE REPORT: A 42-year-old female patient was operated to treat a retroperitoneal mass. The diagnosis was established as PEComa with benign behavior. Two years after the diagnosis, chest and abdominal computed tomography scans showed intra-abdominal recurrence and lymphangioleiomyomatosis in the lung. Treatment with everolimus was started. The disease stabilized in the third month of treatment, according to the response evaluation criteria in solid tumors. CONCLUSION: PEComas are tumors with unpredictable behavior. Therefore, these patients require long-term follow-up, even in cases of correct diagnosis and benign PEComa.

Perivascular epithelioid cell tumor (PEComa) of the pterygopalatine fossa

“…Perivascular epithelioid cell tumors have previously been associated with tuberous sclerosis, although they have been reported to occur in the absence of this syndrome . Perivascular epithelioid cell tumors are typically benign but are known to recur, and malignant behavior has been reported from a number of primary sites . Commonly reported sites of origin include abdominal and gynecologic soft tissue, although cases in the head and neck region have been previously described .…”

Pecoma De Colo Uterino: Um Raro Relato De Caso

“…São encontrados mais frequentemente no retroperitônio, em região abdominopélvica, no trato gastrointestinal e no trato ginecológico, podendo ser encontrada também em tecidos moles e ossos 7,8 . Os sintomas apresentados podem ser assintomáticos ou inespecíficos, tais como náuseas, anorexia e queixas álgicas 9,10 .…”