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Abstract Intracranial germ cell tumors (iGCTs) are rare central nervous system neoplasms predominantly affecting children, adolescents, and young adults. They exhibit striking geographic and gender predilection, with a higher incidence in East Asian populations and a marked male preponderance. Clinically, iGCTs encompass two broad entities—germinomatous and non-germinomatous tumors, with the latter comprising embryonal carcinoma, yolk sac tumor, choriocarcinoma, immature teratoma, and mixed germ cell histologies. This histopathological distinction has critical prognostic implications and remains the cornerstone for contemporary risk-adapted treatment strategies. Current practice relies on an interplay of serum and cerebrospinal fluid tumor markers, neuroimaging, and selective histopathological confirmation for diagnosis. However, there is a lack of a universally accepted consensus on the diagnostic work-up and management protocols for such tumors owing to their infrequent incidence and divergent cooperative group philosophies. Substantial discordance persists in tumor marker thresholds, indications for biopsy, staging definitions, radiotherapy planning, and the role of neoadjuvant chemotherapy. In this context, we did an intensive literature search for relevant articles on iGCTs over PubMed, Embase, and Google Scholar search engines and formulated the present review, which critically integrates contemporary evidence and major international guidelines, including those from European Oncology Societies (EANO-SNO-EURACAN), the Children's Oncology Group (COG), the International Society of Pediatric Oncology (SIOP), and the Japanese Society of Neuro-Oncology (JSNO), to provide a comprehensive overview of epidemiology, biology, diagnostic paradigms, risk stratification, and multimodality management of iGCTs.
Miglani et al. (Wed,) studied this question.