Germinomas and Non-germinomatous Germ Cell Tumors

Intracranial germ cell tumors (iGCTs) are rare neoplasms that predominantly affect children and adolescents, accounting for up to 20% of pediatric germ cell tumors and showing higher incidence in East Asian populations. These tumors commonly arise in the pineal and suprasellar regions, with a subset presenting as bifocal lesions. iGCTs are broadly classified into germinomas and non-germinomatous germ cell tumors (NGGCTs), with germinomas representing two-thirds of cases. Tumor classification reflects their developmental origin from primordial germ cells and is confirmed through histopathology and immunohistochemistry, notably with markers, such as SALL4, PLAP, OCT3/4, and CD117. NGGCTs include embryonal carcinoma, yolk sac tumor, choriocarcinoma, and teratomas and often secrete tumor markers like alpha-fetoprotein (AFP) and beta-hCG, which guide diagnosis and treatment without biopsy. Accurate diagnosis using imaging, markers, with or without histology, followed by risk-adapted therapy, is crucial for optimizing outcomes in this heterogeneous group of CNS tumors Treatment strategies differ significantly between tumor types. Germinomas, highly radiosensitive, are treated with chemotherapy and reduced-field radiotherapy, while NGGCTs require intensive chemotherapy, surgical resection of residual tumor, and high-dose radiotherapy. Prognosis is excellent for germinomas but remains more guarded for NGGCTs. Long-term follow-up is essential due to the risk of recurrence and treatment-related sequelae.