Cystic Neuroblastoma of Infancy

Cystic neuroblastoma is exceedingly rare; only 29 cases have been reported in the medical literature. The authors treated two children with cystic neuroblastoma. The first was a 2-week-old boy with an adrenal lesion; the second was a 15-month-old girl with solitary thoracic cystic neuroblastoma. Cystic neuroblastoma is located, almost exclusively, in the adrenal gland. In contrast to solid neuroblastoma, cystic neuroblastoma has a benign course. Cystic neuroblastoma is diagnosed earlier than solid neuroblastoma, and the former rarely presents with metastatic lesions. All previous reported cases of cystic neuroblastoma were diagnosed before 21 months of age. The presence of calcification was rare, and only 9.5% had documented elevation of the vanillylmandelic acid or homovanillic acid level. Surgical resection of all gross tumor was accomplished in 86% of children with cystic neuroblastoma, and in this group none of the patients had reported recurrent disease. Both present cases also had surgical resection of the entire gross tumor, and likewise have had no evidence of recurrent disease. This report brings the total number of reported pediatric cases of cystic neuroblastoma to 31; to the authors' knowledge, this is the first report of a child with solitary cystic neuroblastoma in an intrathoracic location.