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

Abstract

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.

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.…”

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

“…Only a few cases have been reported worldwide and can be referred to in diagnosing and treating this disease. [6][7][8][9][10][11][12][13][14][15][16][17] Characteristics of the 12 cases of renal malignant PEcoma from PubMed are shown in Table 1. According to relevant literature reports, the treatment strategy mainly depends on the tumor status, overall health status, and symptoms.…”

“…[4] Renal PEComa mainly includes angiomyolipoma, which is relatively common. [5] However, malignant renal perivascular epithelioid cell tumor is extremely rare, [6][7][8][9][10][11][12][13][14][15][16][17] and only a few cases have been reported worldwide. Surgery is the primary treatment for malignant PEComa because the efficacy of chemoradiotherapy is uncertain.…”

Perirenal Perivascular Epithelioid Cell Tumor (PEcoma) with Pulmonary Micro Invasive Adenocarcinoma: A Case Report and Literature Review

“…Since the first case of PEComa reported in 1996, cases involving various anatomic locations have been reported, such as gastrointestinal tract, 4 kidney, 12 pancreas, 13 liver, 14 ovary, 15 uterus 5 and so on. 16 , 17 According to the recent systematic reviews, 10 , 15 the most commonly affected organs were uterus (including uterine and cervix uteri) and colon, with approximately 65 and 20 cases reported online, respectively.…”