Hepatocellular carcinoma in association with bile duct hamartomas: report on 2 cases and review of the literature.

Biliary hamartomas are hepatic lesions related to defects in the ductal plate and are part of the spectrum of adult polycystic disease. Microscopically, the lesions are small and consist of irregular bile ducts amidst a fibrous stroma. There are reports on the malignant transformation of biliary hamartomas, particularly on cholangiocarcinoma. Case 1: a 19-year-old woman presented with increased abdominal volume, lumbar pain, jaundice, choluria, cachexia, and progression to hepatic insufficiency. At autopsy, the liver weighed 8850 g and showed a 20.5-cm mass in the right lobe and nodules in the left lobe. Case 2: a 39-year-old man presented with an asymptomatic nodule in the right hepatic lobe. A 6.5-cm liver segment containing a 3.0-cm nodule was excised. Histopathology of both cases revealed hepatocellular carcinoma associated with multiple bile duct hamartomas (von Meyenburg complex). Bile duct hamartoma is generally an incidental finding at laparotomy and autopsy because its course is asymptomatic. The literature has documented its possible progression to malignant neoplasia. However, this relationship has only been established with cholangiocarcinomas. We report herein for the first time on the association of the lesion with hepatocellular carcinoma.

Fibroistiocitoma angiomatóide com metástase ao diagnóstico: relato de caso e revisão da literatura / Angiomatoid fibrous histiocytoma presenting metastasis: case report and literature review

O fibroistiocitoma maligno variante angiomatóide constitui entidade rara e geradora de controvérsia, especialmente quanto a sua histogênese. Apresenta como peculiaridades o acometimento preferencial da faixa etária pediátrica e o comportamento biológico indolente, tendo baixas taxas de metastatização. No presente caso, paciente feminina de 6 anos apresentou, em decorrência de lesão em extremidade inferior, metástase linfonodal inguinal.

Angiomatoid malignant fibrous histiocytoma is a rare and controversial entity, particularly as to its histogenesis. The tumor affects mostly the pediatric group, with an indolent clinical course and low rate of metastatization. In this report, the patient presented a lesion in the right foot and metastasis in an inguinal lymph node.