RESUMO
Hepatic epithelioid hemangioendothelioma (HEHE) is a vascular tumor with a low incidence rate. We report a case of a 26-year-old man who was referred to our hospital with a misdiagnosis of liver cirrhosis. On physical examination, ascites was noted. Chest and abdominal computer tomography scans showed coalescent lesions involving the peripheral liver with heterogeneous contrast enhancement and portal vein dilation due to portal hypertension. Extrahepatic metastasis was not observed. The biopsy with immunohistochemical stains suggested HEHE (Factor VIII, CD31, and CD34). This report describes an uncommon case of HEHE with non-cirrhotic portal hypertension.
El hemangioendotelioma epitelioide hepático (HEHE) es un tumor vascular con una tasa de incidencia baja. Presentamos el caso de un varón de 26 años que fue remitido a nuestro hospital con un diagnóstico erróneo de cirrosis hepática. En el examen físico se observó ascitis. La tomografía computarizada de tórax y abdomen mostró lesiones coalescentes que afectaban al hígado periférico con realce heterogéneo de contraste y dilatación de la vena porta por hipertensión portal. No se observó metástasis extrahepática. La biopsia con tinciones inmunohistoquímicas sugirió HEHE (Factor VIII, CD31 y CD34). Este informe describe un caso poco común de HEHE con hipertensión portal no cirrótica.
RESUMO
Hemangioendothelioma epithelioid is a rare tumor that originates in soft tissues. Imaging evaluation with conventional modalities (tomography and magnetic resonance) is difficult. Novel radiotracers which capably evaluate angiogenesis may have a higher impact on the therapeutic decisions. A 45-year-old man underwent workup for thrombosis and was diagnosed with hemangioendothelioma epithelioid based on the results of liver pathology and immunohistochemistry. The decision of the multidisciplinary board was to begin with thalidomide. After 4 months, progression of disease was documented and right hepatectomy was performed. A 68Ga-DOTA-E-[c(RGDfK)]2 positron emission tomography-computed tomography scan showed residual lesions. After documented angiogenesis by 68Ga-DOTA-E-[c(RGDfK)]2 positron emission tomography-computed tomography, nintedanib was administrated. And 1 year later, progression of the disease was documented by positron emission tomography-computed tomography. Ipilimumab plus nivolumab was started and partial response and excellent clinical response were documented. Molecular imaging with 68Ga-DOTA-E-[c(RGDfK)]2 positron emission tomography-computed tomography is a good biomarker of the response of hemangioendothelioma epithelioid, and ipilimumab plus nivolumab therapy demonstrated a good response.
RESUMO
El hemangioendotelioma epitelioide hepático es una neoplasia poco común, de origen vascular y comportamiento variable, y potencialmente maligno. Se presenta el caso de unamujer de 33 años, quien consultó por dolor en hipocondrio derecho y fiebre, y cuya impresióndiagnóstica extrainstitucional fue absceso hepático; posteriormente fue posible considerareste tumor dentro del diagnóstico diferencial, gracias a sus características imaginológicas,las cuales se confrontan en este reporte de caso con los hallazgos descritos en las mayores series publicadas en la literatura. El diagnóstico se confirmó mediante biopsia de la lesióny estudio histopatológico del hígado explantado. Se presentan, además, los hallazgos en Resonancia Magnética (RM) de otra paciente evaluada en la Unidad de Trasplantes de la Fundación Cardioinfantil y la evidencia acerca del tratamiento de esta entidad mediante trasplante hepático.
Hepatic epithelioid hemangioendothelioma is a rare neoplasm of vascular origin withmalignant potential. We present the case of a 33 year old woman with abdominal pain and fever, initially diagnosed as a hepatic abscess, who was admitted for further study. Imagingfeatures in ultrasound and computed tomography included a peripheral and multifocal liver lesion with capsular retraction, diagnosis was confirmed with a liver biopsy an a pathologicanalisis of the explanted liver. Magnetic Resonance findings from a second patient with a rapidly progressive disease are also reviewed and evidence regarding the surgical treatmentof this condition with liver transplantation are discussed.