To investigate the diagnosis, treatment and clinical efficacy of perivascular epithelioid cell tumor (PEComa) of the liver.

Clinical characteristics, imaging manifestations, histopathological findings, diagnosis and treatment of a case of PEComa of the liver admitted to the First Affiliated Hospital of Harbin Medical University were retrospectively analyzed, and literature review was performed. The 46-year-old female patient reported chief complaint of "intermittent abdominal distension for 1 month" and was admitted to our hospital on January 19, 2022. No positive signs were found in abdominal examination. No abnormality was found in liver function. Routine blood test: red blood cell count was 2.43×10¹²/L, indicating the sign of anemia. CT scan of the liver prompted multiple space-occupying lesions with abundant blood supply in the liver, accompanied with partial cystic changes. The possibility of malignant tumors could not be excluded. Plain CT scan of the kidney and lung showed multiple mixed densities in bilateral kidneys and multiple cystic lesions in both lungs. The informed consents of the patient was obtained and the local ethical committee approval was received.

After consultation by Department of Hematology and Department of Rheumatology Immunology, preliminary diagnosis suggested that anemia was correlated with PEComa of the liver. To further confirm the diagnosis, PET-CT examination showed hematoma in partial liver lesions. On January 25, 2022, the patient underwent left lateral lobe resection, partial right lobe resection and cholecystectomy. The operation was successfully completed, and the patient was properly recovered without fever or abdominal pain. She was discharged at postoperative 5 d. Pathological diagnosis indicated PEComa of the liver with slight cell atypia, rare mitosis and local necrosis. Immunohistochemical staining revealed HMB-45(+), MelanA (weakly +), SMA (lesions +), Ki-67 5%(+), CD34 (vessels +) and CD68 (weakly +). As of the submission date, no recurrence has been reported at postoperative 18 months.

PEComa of the liver is a rare condition with non-typical clinical features. The diagnosis mainly depends on pathological examination. Surgery is the optimal solution. Extensive attention should be paid to postoperative follow-up.