Primary Hepatic Neuroblastoma

We present a rare case of primary hepatic neuroblastoma in an 8-year-old boy who was admitted to Mofid Children’s Hospital due to organomegaly which was detected during physical examination. Abdominal sonography revealed a 9.9x9.3 cm well defined mass with low vascularity in Rt lobe of liver. Abdominal Spiral CT scan with IV contrast media showed a 11.1x9.6x8.9 cm right liver mass involving segments VI and VII, causing compression effect on Rt kidney, IVC and head of pancreas. Spiral CT of thorax and the rest of the abdominal cavity, chest X-ray, bone survey , BM biopsy and flow cytometry were normal. The clinical diagnosis was hepatoblastoma.The specimen was composed of an encapsulated spherical mass measuring 11.5x9.5x8 cm and weighing 550 gr with smooth, dark brown external surface. On cutting, it was a well-defined lobular, solid, dark brown mass with multifocal hemorrhagic and necrotic areas. Residual yellow, liver tissue measured 3x3x0.2 cm. Liver tissue revealed involvement by a poorly differentiated neuroblastoma with large amount of neuropil containing neuroblasts, many rosettes, less than 5% ganglion cells and foci of necrosis and hemorrhage. Mitotic-karyorrhectic index (MKI)was low (less than 100 per 5000 tumor cells; less than 2%), tumor calcification and lymphovascular invasion were present. The tumor cells showed immunopositivity for chromogranin and synaptophysin. According to the International neuroblastoma pathology classification (INPC) it had a favorable histology. Non-tumoral liver tissue revealed macro- and micro- vesicular fatty change. FISH analysis of the proto-oncogene N-MYC was negative or N-MYC amplification.This case is presented due to its extreme rarity.

کلیدواژه ها:

Liver ، Neuroblastoma ، Primary