Hepatic perivascular epithelioid cell tumor: A case report

BACKGROUND: Perivascular epithelioid cell tumor (PEComa) is a mesenchymal tumor with histologic and immunophenotypic characteristics of perivascular epithelioid cells, has a low incidence, and can involve multiple organs. PEComa originating in the liver is extremely rare, with most cases being benign, and only a few cases are malignant. Good outcomes are achieved with radical surgical resection, but there is no effective treatment for some large tumors and specific locations that are contraindicated for surgery. CASE SUMMARY: A 32-year-old woman was admitted to our hospital with a palpable abdominal mass and progressive deterioration since the previous month. An ultrasound-guided percutaneous liver aspiration biopsy was performed. Postoperative pathological immunohistochemical staining was HMB45, Melan-A, and smooth muscle actin positive. Perivascular epithelioid tumor was diagnosed. The tumor was large and could not be completely resected by surgery. Further digital subtraction angiography revealed a rich tumor blood supply, and interventional embolization followed by surgery was recommended. Finally, the patient underwent transarterial embolization (TAE) combined with sorafenib for four cycles. Angiography reexamination indicated no clear vascular staining of the tumor, and the tumor had shrunk. The patient was followed up for a short period of time, achieved a stable condition, and surgery was recommended. CONCLUSION: Adjuvant combination treatment with TAE and sorafenib is safe and feasible as it shrinks the tumor preoperatively and facilitates surgery.