Dumbbell neuroblastomas

Abstract

Neuroblastic tumors (neuroblastoma [NB], ganglioneuroblastoma) are the most common extracranial solid tumors in children during the first 2 years of life and account for 8% to 10% of all childhood cancers. NBs arising from a paravertebral ganglion have an unusual tendency to grow through the intervertebral foramen, forming a dumbbell-shaped or hourglass-shaped mass. The intraspinal component may cause neurologic symptoms of spinal cord compression with paralysis and/or incontinence. Most patients with dumbbell neuroblastoma (DNB) have an excellent chance for long-term survival. The frequency of spinal epidural extension with NB varies from 6% to 25% in different series and may occur by direct extension from a paraspinal primary tumor or from vertebral bone, metastases. Congenital DNB accounts for about 10% of all DNB cases. DNB is the most common malignant cause of spinal cord or nerve root compression in children. Although chemotherapy is safe and effective, the treatment strategy remains controversial. Commonly asked questions that need to be answered when managing the DNB patients include 1) what studies are necessary for preoperative evaluation of a DNB; 2) what is the preferred treatment regimen, and how aggressive should treatment be; and 3) what is the long-term morbidity from treatment and disease? In this article, we review the clinical features of DN13 and try to provide guidelines for the workup and treatment of the disorder.