10063 Background: Intracranial germ cell tumors (iGCT) constitute 1% of all malignant neoplasms in children. The aim of this study is to evaluate the demographic characteristics, therapy, and long term outcome of children with iGCT in a single institution. Methods: 16 children (9 female, 7 male) with a median age of 11 years (10 months-18 yrs) treated for iGCT between 1990 and 2008 were evaluated retrospectively. They were treated according to our institutional protocol. Patients > 13 years old with germinomas recieved radiotherapy alone (2520 cGy craniospinal/1980 cGy local boost), patients <13 yrs old recieved 2 courses of etoposide, cisplatinum followed with cranial radiotherapy. Nongerminoma patients recieved 3 courses of bleomycine, etoposide, cisplatinum followed by 3600 cGy craniospinal / 1980 cGy local boost RT. Results: Presenting clinical features were diabetes insipidus (7), raised intracranial pressure (12), visual changes-oculomotor palsies (6), hypopituitarism (4). 8 had germinomas and 8 nongeminomatous GCT. Ten were suprasellar and 6 pineal. AFP and BHCG were evaluated at diagnosis and during therapy both in serum and CSF. Three had seeding in the spinal axis in MRI. One had widespead systemic metastasis. Three had a total resection, 12 had a biopsy, 1 was clinically/radiologically diagnosed, 5 had a shunt. Two nongerminamatous iGCT patients died: 1 of widespread disease in the intensive care unit at diagnosis, the other with progressive disease 18 months after diagnosis. Fourteen patients are alive at a median follow-up of 12 years (1–17 years) from diagnosis. One that had motor and mental retardation before diagnosis needs special care. All others have a moderate/good quality of life. One is married and has a child. Six are at school (2 in university). Five are employed. The patients are also followed up in the endocrinology clinic. One is recieving growth hormone. Conclusions: Cisplatin based chemotherapy and radiotherapy is successful and well tolerated in children with iGCT. The possibility of an early clinical diagnosis based on MRI and tumor markers and the use of modern neurosurgical techniques increases the chance of cure, gives a chance to reduce acute morbidity and further decrease late effects. No significant financial relationships to disclose.
Intracranial germ cell tumors: a single-institution experience
