Infrantentorial ganglioglioma in a child-Case report

Introduction: Ganglioglioma is a very rare primary neoplasm comprising up to 10% of primary pediatric CNS neoplasms. Posterior fossa gangliogliomas represent less than 15% of these cases. Gross-total resection in the infratentorial region is often not possible and is associated with important morbidity. Regardless tumor's location the postoperative adjuvant therapy following subtotal resection of gangliogliomas is still controversial. Herewith, we present a case of a posterior fossa low-grade ganglioglioma with an unfavorable outcome along with a brief review of current literature on operative and adjuvant therapy. Material and Methods: A 9-month-old infant was admitted with a large posterior fossa tumor and marked hydrocephalus. The patient was operated on twice with partial and gross total resection, respectively. The radiological follow-up including MRI and PET-scan following both operations was very unfavorable with a vast progression of a lesion. PET-scan demonstrated hypermetabolism. Pathological examination following both operations revealed a ganglioglioma (WHO Grade I) brain tumor with an index of proliferation less than 1% (anti-KI67). The postoperative course was marked with extremely high progression of a lesion and a clinical deterioration. The patient died two weeks after the second operation. Conclusions: Ganglioglioma is an uncommon neoplasm of the CNS that rarely presents in the posterior fossa. Even though, in the majority of cases the course of the disease is benign, it may present very unfavorable outcome as in the presented case. The adjuvant therapy following subtotal resection is still controversial and there is certainly a need for further randomized studies in this field.