Craniovertebral junction neoplasms in the pediatric population

Abstract

The incidence of tumors at the craniovertebral junction in the pediatric population is low. Because of the variable pathology and the rarity of these tumors, ideal therapies are only now being defined. Thirty-eight children with tumors affecting the craniocervical junction were encountered between 1991 and 2006. These comprised neoplasms of osseous origin and neural extramedullary tumors. Chordomas of the clivus and foramen magnum were seen in eight, fibrous dysplasia in four, aneurysmal bone cysts in four, eosinophilic granuloma affecting the atlas and axis vertebra in four, Ewing's sarcoma involving the atlas in two, osteoblastoma in two, neurenteric cysts in four, meningioma in five, schwannoma in two, and plexiform neurofibromas in three. The location of these tumors was predominantly ventral, and a very small number had a lateral or dorsal location. The ventral tumors included chordoma, meningioma, fibrous dysplasia, aneurysmal bone cyst, and osteoblastoma. Plexiform neurofibroma affecting the craniocervical junction was ventral to the clivus and upper cervical spine causing severe kyphosis of the craniocervical region. Pain in the head and neck occurred in 70%. Paresthesias and dysesthesias in the hands were seen in 40% and spastic weakness of extremities in 22%. Cranial nerve palsies were seen in 33%. Twenty-eight percent of children showed dysphagia or dysarthria. The cranial nerves affected were the vagus followed by hypoglossal and glossopharyngeal nerves. This led to dysphagia, slurred speech, repeated aspiration pneumonia, and weight loss. The most common findings for chordomas at the craniocervical junction were isolated hypoglossal nerve palsy. All individuals underwent magnetic resonance imaging, computed tomography, and 3D computed tomography and angiography. Vertebral angiography was used to understand the dynamics of collateral circulation and tumor vascularity. Tumor embolization was performed in chordoma and aneurysmal bone cysts. Our experience and results are presented here.