Cervical Chordoma in Childhood Without Typical Vertebral Bony Destruction

In Brief Study Design. Case report. Objective. We present a giant cervical chordoma without typical vertebral bony destruction in an 11-year-old girl. Summary of Background Data. Chordomas are rare malignant bone tumors that arise at both the cranial and the caudal ends of the axial skeleton, characteristically destroying the bone. Cervical chordomas comprise only 3% to 7% of all chordomas. To our knowledge, there is no case of cervical chordoma in a child, presenting without vertebral body involvement, in English literature. Methods. Discussion on the patient’s clinical, radiologic history, and histopathologic diagnosis of the resected tumor, with a review of the relevant background literature. Results. We report the first case of cervical chordoma in a child without typical vertebral bony destruction, the diagnosis of which was difficult to confirm before and after operation. Conclusion. Giant notochordal rest and benign notochordal cell tumors (BNCTs) need to be recognized for differential diagnosis of this atypical chordoma. A long-term follow-up might be necessary for the diagnosis of this nontypical patient. Chordomas display bone destruction and intralesional calcification, which are regarded as radiologic hallmarks. It is a tumor that occurs commonly in older patients, and there is a generally progressive increase in incidence with age. Chordomas in children are rare and unpredictable.