A Primary Spinal Nongerminomatous Germ Cell Tumor With Multiple Intracranial Metastases in a Toddler

• Published in: Journal of pediatric hematology/oncology Vol. 45; no. 3; p. e415
• Main Authors: Ramanathan, Subramaniam, Hill, Rebecca M, Ryles, Jade, Halliday, Gail, Mitra, Dipayan, Bailey, Simon
• Format: Journal Article
• Language: English
• Published: United States 01.04.2023
• Subjects: Biomarkers, Tumor - metabolism; Brain Neoplasms - pathology; Brain Neoplasms - therapy; Central Nervous System Neoplasms - therapy; Child; Child, Preschool; Humans; Male; Neoplasms, Germ Cell and Embryonal - therapy; Testicular Neoplasms
• ISSN: 1536-3678
• DOI: 10.1097/MPH.0000000000002486

Central nervous system germ cell tumors (CNS-GCTs) comprise 4% of all pediatric CNS tumors, with one third being nongerminomatous GCT (CNS-NG-GCT) type. The majority of these tumors arise in the intracranial compartment with 20% having drop metastases in the spine. We present a rare case of a 2-year-old boy with a primary intradural-extramedullary NG-GCT arising from the lumbosacral spine with a trifecta of unfavorable features, that is, young age, alpha-feto protein >1000 ng/mL, and disseminated disease within the cranium. Owing to his young age, he was treated with chemotherapy alone, avoiding radiation. His tumor marker (alpha-feto protein) declined from 8468 to 10 k-U/L over 8 weeks, and he remained in remission at the last follow-up. This atypical presentation of an intradural-extramedullary tumor with cranial dissemination in a childhood NG-GCT has yet to be described in the literature. Here we use this opportunity to highlight the treatment strategies and challenges in this unique clinical case.