Intracranial Germ Cell Tumors: A Comprehensive Update of the European Data

TLDR

Intracranial germ cell tumors are rare childhood and adolescent tumors, mainly germinomas, occurring in midline sites such as the pineal and suprasellar regions, with rising incidence and poor prognosis for non‑germinomatous types. The study aims to improve survival of patients with these tumors by evaluating chemotherapy added to conventional surgery and radiotherapy. The authors review international efforts, including the 1993 SIOP working group, to develop combined chemotherapy, surgery, and radiotherapy protocols. Modern protocols achieve about 90 % cure of pure germinomas with radiotherapy alone.

Abstract

Intracranial germ cell tumors are rare tumor entities in childhood and adolescents. Extra- and intracranial germ cell tumors are identical in their histologic pattern and occur in preferential midline localizations such as the pineal and the suprasellar region. Germ cell neoplasms show increasing incidence rates over the last 30 years. The majority of intracranial germ cell neoplasms are germinomas. About 90% of the patients with pure germinomas can be salvaged by radiotherapy alone according to modern protocols. Non-germinomatous malignant CNS-germ cell tumors are considered to have a poor prognosis. In order to improve the survival of patients affected by these tumors different treatment approaches adding chemotherapy to conventional surgery and radiotherapy have been initiated by various study groups throughout the world. Due to the rarity of these neoplasms only a very limited number of patients has been enrolled in each study. In 1993 an international working group on these tumors was established by the International Society of Pediatric Oncology (SIOP).