Congenital Neuroblastoma of the Adrenal with Metastasis to Liver, Contralateral Adrenal and Pituitary: Report of an autopsy case

Myong, Na Hye; Song, Sang Yong; Chi, Je G

Abstract

Neoplasms presenting at birth or within the first month of life are defined as congenital tumors. The principal components of this congenital tumors are neuroblastoma, leukemia, brain tumors and sarcomas. The neuroblastoma is the most common accounting for 15~50% of all tumors in this group. It most often presents with an abdominal mass due to adrenal-retroperitoneal primary or hepatomegaly resulting from extensive metastasis. Most often the primary site is adrenal but other loci include the retroperitoneum, mediastinum, pelvis, etc. This 2-day-old female presented with hepatomegaly and a left adrenal mass at birth, first detected by ultrasonography. On the first day, she suffered from hematemesis and bradycardia. She died on the second day. Postmortem examination revealed massive metastatic tumor nodules in the liver and a well-demarcated round mass, 4 cm, in the left adrenal, with necrosis and hemorrhage. Microscopic findings revealed largely undifferentiated neuroblastoma with focal neuronal differentiation and areas of necrosis and calcification in the background of fine fibrovascular stroma. Other metastatic foci were detected in the right adrenal and pituitary gland.