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

J Int Med Res. 2020 Oct;48(10):300060520961223. 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.

Keywords: Perivascular epithelioid cell tumor; case report; immunohistochemistry; kidney; recurrence; retroperitoneum.

Publication types

  • Case Reports

MeSH terms

  • Carcinoma, Renal Cell* / diagnostic imaging
  • Carcinoma, Renal Cell* / surgery
  • Humans
  • Kidney / diagnostic imaging
  • Kidney / surgery
  • Kidney Neoplasms* / diagnostic imaging
  • Kidney Neoplasms* / surgery
  • Male
  • Neoplasm Recurrence, Local
  • Perivascular Epithelioid Cell Neoplasms* / diagnostic imaging
  • Perivascular Epithelioid Cell Neoplasms* / surgery