Ceus features of liver pecoma: a case report and literature review.

Abstract

Perivascular epithelioid cell neoplasms (PEComas) and epithelioid angiomyolipomas (EAMLs) are two different denominations for the same "mesenchymal tumor composed of histologically and immunohistochemically distinctive perivascular epithelioid cells". Hepatic PEComa/EAML is a very rare neoplasm, and only 29 case reports of hepatic PEComa and 25 of hepatic EAML have been reported in the current literature. A clear female predominance with a mean age at diagnosis of 42.5 years old can be observed by literature review. Ultrasound (US) examination was the first-line diagnostic technique in most of the cases of hepatic PEComa, but it was documented in very few cases of hepatic EAML. A great variability in the ultrasonographic B-mode, color Doppler and contrast-enhanced ultrasonography (CEUS) features of hepatic PEComa/EAML emerges. Computed tomography and magnetic resonance were the most common used techniques to confirm the nature of the hepatic lesion, even if the anatomo-pathological examination was the only technique to display a certainty diagnosis and to differentiate hepatic PEComa/EAML from benign and malignant hepatic lesions. The great majority of hepatic PEComas/EAMLs are surgically treated without any adjuvant therapy.