Primary central nervous system germ cell tumors in children and young adults: A review of controversies in diagnostic and treatment approach

• Published in: Neoplasia (New York, N.Y.) Vol. 36; p. 100860
• Main Authors: Yeo, Kee Kiat, Nagabushan, Sumanth, Dhall, Girish, Abdelbaki, Mohamed S.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.02.2023; Elsevier
• Subjects: Brain Neoplasms; Central Nervous System - pathology; Central Nervous System Neoplasms - diagnosis; Central Nervous System Neoplasms - therapy; Chemotherapy; Child; Combined Modality Therapy; Germ cell tumor; Germinoma; Humans; Mixed malignant; Molecular; Neoplasms, Germ Cell and Embryonal - diagnosis; Neoplasms, Germ Cell and Embryonal - therapy; Prospective Studies; Radiation therapy; Young Adult; Germ cell tumor; Chemotherapy; Radiation therapy; Molecular; Germinoma; Mixed malignant
• ISSN: 1476-5586; 1476-5586
• DOI: 10.1016/j.neo.2022.100860

Primary central nervous system (CNS) germ cell tumors (GCT) are a rare heterogenous group of cancers, arising most commonly in the second decade of life. Through several clinical trials conducted around the world by various groups, the treatment approach for CNS GCT has advanced substantially with generally improved overall outcomes. In recent years, the goal of clinical trials has been focused on reduction of the radiotherapy burden and minimization of long-term toxicity. This review summarizes the current diagnostic and treatment regimens for CNS GCT, examines the controversies associated with these approaches, gaps in contemporary knowledge, and underscores the challenges we face. We also explore future directions in the management of CNS GCT with the ultimate overall aim of preserving curative outcomes, identifying novel biomarkers, and mitigating neurocognitive, endocrine, and psychological toxicity through prospective clinical studies.