RESUMO
We present three cases of chronic hepatic porphyria (CHP) in alcoholic patients, in which grayscale ultrasound (US) revealed multiple echogenic masses in the liver, mimicking multinodular hepatocellular carcinoma on alcoholic liver injury. In all cases, contrast-enhanced US (CEUS) showed iso-enhancement of the mass lesions throughout all vascular phases. Additionally, two-dimensional shear wave elastography (2DSWE) (performed in two cases) revealed the mass to have almost the same SWE value as the surrounding parenchyma. When encountering alcoholic patients with multiple echogenic masses in the liver, CHP must be included in the differential diagnosis. CEUS and 2DSWE allow us to increase our diagnostic confidence of CHP.
Assuntos
Fígado Gorduroso Alcoólico/diagnóstico por imagem , Porfirias Hepáticas/diagnóstico por imagem , Alcoolismo/complicações , Carcinoma Hepatocelular/diagnóstico por imagem , Meios de Contraste , Diagnóstico Diferencial , Técnicas de Imagem por Elasticidade/métodos , Fígado Gorduroso Alcoólico/etiologia , Feminino , Humanos , Neoplasias Hepáticas/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Porfirias Hepáticas/etiologia , UltrassonografiaRESUMO
An F-FDG PET/CT was performed on a 43-year-old woman with photosensitive skin rash, abnormal liver function, and pancytopenia, which demonstrated prominent hepatomegaly, splenomegaly, and diffuse liver F-FDG avidity. The liver biopsy revealed intrahepatic cholestasis with biliary fibrosis resulting from the deposition of protoporphyrin. X-linked erythroid-specific 5-aminolevulinate synthase gene analysis proved the diagnosis of X-linked protoporphyria.
Assuntos
Fluordesoxiglucose F18/metabolismo , Fígado/diagnóstico por imagem , Fígado/metabolismo , Porfirias Hepáticas/diagnóstico por imagem , Porfirias Hepáticas/genética , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Adulto , Feminino , Humanos , Porfirias Hepáticas/metabolismoRESUMO
UNLABELLED: Appearance of liver foci in patients with chronic hepatic porphyria has been described in literature several times since 1988. We present two impressive examples of multiple coin-like lesions in the liver of patients with till than unknown porphyria. A 61-year-old women was hospitalized with suspected liver metastases: sonography showed multiple ring-like liver foci up to 2 cm. In a 52-year-old man sonography incidentally detected multiple liver foci, in this case homogeneous hyper-echogenic. In both patients a liver tumor was excluded by biopsy, the histologic examination of the specimens only showed uncharacteristic alterations. DISCUSSION: Sonographic findings in hepatic porphyria most times are only uncharacteristic alterated. But 10% of the patients show multiple hyper-echogenic hepatic foci: these lesions typically have a well marked rim, some times they even appear like a ring. In color doppler examination hepatic vessels are not affected by the lesions. The lesions themselves do not show an increased vascularisation. This allows to discriminate from other liver tumors, especially from liver metastases. Histologic findings are not pathognomonic, laboratory research is subsequently required. Strictly avoiding alcoholic drinks the presented discoveries are in principle reversible.
Assuntos
Fígado/diagnóstico por imagem , Porfirias Hepáticas/diagnóstico por imagem , Diagnóstico Diferencial , Feminino , Humanos , Fígado/patologia , Masculino , Pessoa de Meia-Idade , Porfirias Hepáticas/patologia , UltrassonografiaRESUMO
We present five cases of chronic hepatic porphyria (CHP) without skin lesions, in whom a multinodular fatty change in the liver was incidentally detected by ultrasound. Discovery prompted further examinations, leading to the diagnosis of CHP. Although relatively rare, the possibility of CHP must be considered when encountering patients with irregular (multinodular) fatty infiltration of the liver of unknown cause. We also discuss the differential diagnosis.
