Perivascular epithelioid cell tumor (PEComa) of the kidney: an overview of its management and outcomes

• Published in: Journal of international medical research Vol. 48; no. 10; p. 300060520961223
• Main Authors: Yang, Yu, Yu, Xiaohong, Lu, Bo, Shao, Zhiqiang
• Format: Journal Article
• Language: English
• Published: London, England SAGE Publications 01.10.2020; Sage Publications Ltd; SAGE Publishing
• Subjects: Carcinoma, Renal Cell - diagnostic imaging; Carcinoma, Renal Cell - surgery; Case Report; Case reports; Humans; Immunohistochemistry; Kidney - diagnostic imaging; Kidney - surgery; Kidney cancer; Kidney Neoplasms - diagnostic imaging; Kidney Neoplasms - surgery; Kidneys; Male; Neoplasm Recurrence, Local; Perivascular Epithelioid Cell Neoplasms - diagnostic imaging; Perivascular Epithelioid Cell Neoplasms - surgery; Tumors; recurrence; kidney; case report; Perivascular epithelioid cell tumor; immunohistochemistry; retroperitoneum
• ISSN: 0300-0605; 1473-2300
• DOI: 10.1177/0300060520961223

Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor with unique histological and immunohistochemical characteristics. Malignant renal cell carcinoma is even rarer and its biological behavior is still not clear. We report here a case of malignant PEComa in a male patient. The first symptom of the patient was intermittent hematuria. Ultrasound and computed tomography scan showed a solid mass in the left kidney. Fibrosarcoma was initially diagnosed after radical resection of the tumor. Six years later, the patient had a tumor recurrence and underwent a second operation, through which the diagnosis of renal malignant PEComa was made. Since then, the patient received chemotherapy, a third operation, and interventional treatment because of recurrence and metastasis of the tumor, which prolonged the life of the patient for another 6 years. Malignant PEComa involving the kidney is very rare; it seems to develop slowly but will recur and metastasize. At present, surgical resection remains the best treatment.