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.摘要背景：血管周上皮样细胞肿瘤（Perivascular epithelioid cell tumors, PEComas）是一类罕见的间叶源性肿瘤，临床中需依据患者个体特征制定辅助放疗和/或化疗方案。 病例报告：1例42岁女性患者因腹膜后肿物接受手术治疗，术后确诊为良性血管周上皮样细胞肿瘤。确诊两年后，胸腹部计算机断层扫描（computed tomography, CT）检查显示腹腔内复发及肺部淋巴管平滑肌瘤病，遂启动依维莫司治疗。依据实体瘤疗效评价标准，治疗第3个月时患者病情稳定。 结论：血管周上皮样细胞肿瘤的生物学行为难以预测，因此即便确诊为良性血管周上皮样细胞肿瘤，此类患者仍需接受长期随访。