• Canbin Lin, Shulin Liang, Yongxing Wang, Aidi Liang, Weiting Qin, Jiapeng Huang, Hao Meng, Hong Liu, Ming Chen, and Lei Meng.
• Department of Urology, The First Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou, Guangdong, PR China.
• Medicine (Baltimore). 2022 Sep 23; 101 (38): e30653.

RationalePerivascular epithelioid cell tumor (PEComa) is a mesenchymal tumor that arises from perivascular epithelioid cells and can differentiate into melanocytes and smooth muscle cells. Malignant renal perivascular epithelioid cell tumor is extremely rare. Due to the lack of specific clinical manifestations and imaging features, diagnosing PEComa depends on postoperative pathology and immunohistochemistry. Surgery is the primary treatment for malignant PEComa because the efficacy of radiotherapy and chemotherapy is uncertain. There is still a lack of unified diagnostic criteria and treatment guidelines for renal malignant PEComa, especially with vascular invasion. Hence, the treatment experience depends on a small number of cases reported worldwide.Patient ConcernsA 68-year-old woman was admitted to our hospital due to intermittent hematuria for over 8 months. The color Doppler ultrasound and computed tomography scan revealed a mass in the lower middle part of the left kidney.DiagnosisRare renal malignant perivascular epithelioid cell tumor with renal vein cancerous thrombosis.InterventionsA laparoscopic radical left nephroureterectomy in the oblique supine lithotomy position was performed.OutcomesThe operation process went smoothly, and no pulmonary embolism occurred after the operation. The final pathological diagnosis was a renal malignant perivascular epithelioid cell tumor. After a 12-month follow-up, no recurrence or metastasis was found.LessonsRenal malignant PEComa is an extremely rare mesenchymal tumor diagnosed mainly based on pathology. Surgery is currently the effective treatment for malignant PEComa. For the surgical treatment of malignant renal PEComa with vascular invasion, laparoscopic radical nephroureterectomy in the oblique supine lithotomy integrative position has many benefits, as exemplified by our current case.Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

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