| Popis: |
El hemangioendotelioma epitelioide hepático es un tumor raro, de naturaleza agresiva y comportamiento impredecible, con una incidencia de menos de 1 caso por millón de personas al año. Se presenta especialmente en mujeres jóvenes y con síntomas no específicos como dolor abdominal y pérdida de peso. La presentación radiológica es con múltiples nódulos o masas periféricas y bilaterales, con retracción de la cápsula, en algunos casos con calcificaciones y compromiso vascular. Debe realizarse biopsia hepática para confirmar el diagnóstico y para descartar diagnósticos alternativos como angiosarcoma, hepatocarcinoma, colangiocarcinoma y metástasis. El manejo depende de la extensión al momento de presentación, aunque solo el 10% de los casos es susceptible de resección. En las últimas décadas, el trasplante hepático se ha convertido en una opción de manejo, con series de casos aisladas reportadas por centros de trasplante en el mundo. En este artículo se presenta la experiencia de 2 pacientes con diagnóstico de hemangioendotelioma epitelioide hepático sin posibilidad de resección, confirmado histológicamente, sin compromiso extrahepático y que fueron llevados a trasplante de hígado exitoso y sin recurrencia de la enfermedad a 4 y 6 años después del trasplante. Hepatic epithelioid hemangioendothelioma is a rare, aggressive and unpredictable tumor. Its incidence is less than one case per million people per year. Most commonly, it occurs in young women who exhibit nonspecific symptoms such as abdominal pain and weight loss. Radiologically it presents as multiple bilateral and peripheral nodules or masses with retraction of the capsule of the liver. In some cases there is calcification and vascular compromise. A liver biopsy should be performed to confirm the diagnosis and to rule out alternative diagnoses such as angiosarcoma, hepatocellular carcinoma, cholangiocarcinoma and metastasis. Management depends on the extension at presentation, but only 10% of cases are amenable to resection. Although in recent decades liver transplantation has become a management option, only studies of isolated cases reported by various transplant centers around the world have been published so far. In this article, we report our experience with two patients who were diagnosed with hepatic epithelioid hemangioendothelioma in which there were no possibilities of resection. Diagnoses were histologically confirmed, and since the patients had no extra-hepatic compromises, liver transplantations were performed. Both were successful and without recurrence at 4 and 6 years after transplantation |
