Pediatric cervical chordoma: report of two cases and a review of the current literature

Abstract

The majority of chordomas occur between the fifth and seventh decades of life and are thus extremely rare in children, which account for less than 5% of all spinal chordoma cases. We report on the development of this rare condition in two boys aged 7 and 10 years. One patient presented with a palpable neck mass and dysphagia, while the other presented with posterior neck pain. Radiological studies revealed an extensive mass in the cervical vertebrae and paravertebral soft tissue of both patients. The tumors were subtotally removed in an attempt to improve the success of adjuvant proton beam radiotherapy. Pathological examination, which included immunohistochemical staining, revealed chordoma of the cervical spine in both patients. Although en bloc resection is the ideal modality for treatment of chordoma, such a procedure is often associated with a significant risk of surgical morbidity due to the tumor location. Therefore, piecemeal resection followed by postoperative adjuvant radiotherapy, including proton radiotherapy or radiosurgery, should be considered in such cases.