Malignant perivascular epithelioid cell tumor in kidney: A case report and literature review / 肿瘤

ABSTRACT

Objective: To investigate the clinicopathological characteristics, diagnosis, and treatment of malignant perivascular epithelioid cell tumor (PEComa) in kidney. Methods: The cinical diagnosis and treatment outcome of a case of PEComa were reported. The morphological characteristics of renal PEComa were analyzed. The PEComa-related literatures were reviewed, and the diagnosis and treatment strategies of PEComa were summarized. Results: A 21-year-old female patient with a solid mass in the left kidney underwent nephrectomy. The pathological examination revealed PEComa. After 2 years, the computed tomography (CT) scan showed several masses in the lung and bone, which were speculated to be metastases from the kidney lesion. The patient received chemotherapy with recombinant human endostatin and Apatinib. Despite active treatment, the tumor was still progressing, and the patient died of respiratory failure 45 months after the original diagnosis. Literature reviews showed that PEComa patients had not typical clinical symptoms, and the positive immunohistochemical results of human melanoma black-45 (HMB45), melanoma antigen (Melan-A), and smooth muscle actin (SMA) were the key features in the diagnosis of PEComa. Conclusion: PEComa is a kind of rare tumor. The diagnosis and treatment of this disease should be intensified, and the long-term close follow-up is necessary.