Congenital neuroblastoma: Report of an autopsy case

Neuroblastoma (NB) is the most common malignancy of infancy. The perinatal tumors are usually associated with an excellent prognosis, although lethal respiratory distress may occur in newborns with stage 4S disease due to large metastatic liver. We describe an autopsy case of a male newborn that died on the second day because of the Pepper syndrome's complications. He had massive hepatomegaly, splenomegaly, and hypertrophic cardiomyopathy, but he did not show any malformation. Histology and immunohistochemical studies confirmed a NB of undifferentiated subtype invading the liver and the pancreas. The primary tumor could not be localized in the adrenal medulla or anywhere along the sympathetic ganglion chain. Accordingly, stage 4S NB with massive hepatomegaly may be of pancreatic origin, raising major diagnostic and therapeutic challenges.