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.
Keywords: CEUS; Hepatic angiomyolipoma; Hepatic perivascular epithelioid cell tumor; Ultrasound.
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