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

• Autor(a) principal: Cihan, Yasemin Benderli
• Data de Publicação: 2019
• Outros Autores: Kut, Engin, Koç, Ali
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: São Paulo medical journal (Online)
• Texto Completo: https://periodicosapm.emnuvens.com.br/spmj/article/view/831
• topic: Perivascular epithelioid cell neoplasms; Retroperitoneal neoplasms; Antineoplastic protocols

CONTEXT: Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors. Adjuvant radio-therapy 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, ac-cording 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.