Pineal germ cell tumors.

Intracranial germ cell tumors (GCTs), especially pineal tumors have attracted the special attention of neuropathologists and neurosurgeons because of their unique growth sites, characteristic subtypes with different histology, and high incidence in Japan and other Asian countries. This chapter describes the general clinical features of pineal GCTs and current treatment of intracranial GCTs. Despite excellent long-term results for patients with germinoma treated with radiation therapy, the potential for late effects makes the treatment controversial. Most patients with nongerminomatous tumors treated by conventional treatment with surgery and radiation therapy failed to survive longer than 3 years. After combination chemotherapy with cisplatin was confirmed to be effective in gonadal GCTs, GCTs of the brain became candidates for chemotherapy. For germinoma, a trial with chemotherapy alone failed with a high rate of recurrence, but Japanese and European trials with chemotherapy and reduced dose and volume of radiation therapy demonstrate good event-free survival rates. Ongoing phase II studies with combined chemotherapy and radiation therapy for nongerminomatous tumors will result in a 5-year survival rate of >50%, which is better than that by radiation therapy alone.