Surgical Role in Management of Intracranial Germ Cell Tumors in Pediatric Age Group

Abstract

Introduction: Primary intracranial germ cell tumors (ICGCTs) are rare, histologically diverse, and diagnostically challenging tumors that are usually localized in the pineal and suprasellar regions of the brain. Advanced neurosurgical techniques such as neuroendoscopy and frameless stereotactic biopsy have made diagnosis of newly discovered cases of ICGCTs easier and safer. Material and methods: Seventeen patients with intracranial germ cell tumors operated upon between 2008 to 2012 at the Children's Cancer Hospital Egypt, were retrospectively reviewed and analyzed regarding the surgical decision, clinical outcome and surgical complications. Results: There were 9 cases of germinoma (53%), and 8 cases of non-germinomatous germ cell tumors (47%). Nine cases were in the pineal region, six in the suprasellar, and two in the thalamic region. Ten cases were operated upon initially by open surgery and frozen section with subtotal resection and seven cases were biopsied either endoscopically (3 cases) or by frameless guided stereotaxic (4 cases). Accurate pathology was achieved in all biopsied cases without major complications. In the germinoma group, the 4-year overall survival and progression free survival rate were 75% for both at a median follow up period of 26 (range 1 -50) months. For the non-germinomatous germ cell tumors group, the 4-year OS and PFS rates were 36.5% and 31.2% at a median follow up period of 11 (range 2-54) months, respectively. Conclusion: In cases of intracranial germ cell tumors with negative tumor markers the role of surgery is important in the establishment of proper histopathological diagnosis. However, in Non Germinomatous Germ Cell Tumors, further investigations should be done regarding the extent of resection owing to the poor long-term outcome.