Recent advances in the diagnosis and treatment of central nervous system germ-cell tumours.

Primary germ-cell tumors of the central nervous system are rare neoplasms that are seen primarily in the pediatric age group. Because of their frequent location in the pituitary and suprasellar regions, they present with typical neuro-ophthalmologic and neuroendocrine symptoms. Sophisticated imaging and surgical biopsy allow precise anatomic definition, but only allow an approximate guess of the tumor histopathology. Tumor markers in the serum and cerebrospinal fluid are extremely helpful in the diagnosis and monitoring of response to treatment when they are detectable. Because of the deleterious effects of irradiation on neurocognitive and neuroendocrine functioning, we have looked at strategies that either reduce or eliminate radiation exposure. Large, randomized, prospective, cooperative trials in the future will be the only way to identify subgroups of patients that may benefit from particular treatment strategies.