Laparoscopic radical nephroureterectomy in the oblique supine lithotomy integrative position for rare renal malignant perivascular epithelioid cell tumor with renal vein cancerous thrombosis: A case report and literature review

Abstract

Rationale: Perivascular 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.

Case report: Urachal perivascular epithelioid cell tumor

“…Similarly, partial nephrectomy or radical nephrectomy are the preferred surgical interventions for renal PEComa. Following surgery, some patients may receive chemotherapy, with a subset experiencing recurrence and metastasis during post-operative follow-up ( 21 , 22 ). Notably, there have been limited reported cases of ureteral PEComa to date.…”