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Giant cell tumors (GCTs) are rare lesions of the cervical spine, with only 14 previously reported pediatric cases in the literature, all occurring in females. The authors present the case of a 15-year-old boy with neck pain who was found to have a lytic GCT of the odontoid process. Following resection, recurrent disease was treated with radiotherapy and chemotherapy and then a final resection. He has remained tumor free for more than 10 years. The rarity of GCTs can make their diagnosis difficult in the cervical spine. Because of their aggressive behavior and relative resistance to adjuvant therapy, GCTs must be monitored diligently and treated aggressively.

Central nervous system medulloepitheliomas are extremely rare and malignant (World Health Organization Grade IV) primitive neuroectodermal tumors (PNETs) that arise in childhood. Unlike other PNETs, medulloepitheliomas have a dismal prognosis, with only 2 reported cases in the literature in which the patient survived beyond 5 years after treatment. The authors report on the third known case of a child who survived longer than 5 years. A review of all the published cases of medulloepithelioma is also presented, and alternative treatment strategies for PNET tumors, including high-dose chemotherapy with stem-cell rescue, are discussed.