Intradural cervical chordoma with diffuse spinal leptomeningeal spread: case report and review of the literature.

PURPOSE: Chordoma is a low-grade malignant bone tumor derived from embryonic notochord remnants along the axial skeleton. About 50% of chordomas occur in the sacral vertebrae and 35% in the skull base. Most chordomas are extradural and cause extensive bone destruction. Intradural spinal tumors without bone involvement are rare.
METHODS: We herein describe the clinical features of a patient with a chordoma as well as the imaging and pathological manifestations of the tumor.
RESULTS: We encountered an unusual presentation of a C6 and C7 spinal intradural chordoma in a 23-year-old man. He presented with a 5-day history of discomfort over the lumbosacral region. Magnetic resonance imaging and enhanced scanning of the cervical spine showed an intradural soft tissue mass at C6 and C7 and linear enhancement of the spinal meninges. The tumor was excised because the patient had been previously misdiagnosed with an intraspinal neurogenic tumor with spinal meningitis. Postoperative pathological examination confirmed the diagnosis of chordoma. On postoperative day 7, the patient underwent brain magnetic resonance imaging because of severe headache. The images showed multiple soft tissue nodules in the skull base cistern. To the best of our knowledge, this is the first case report of an entirely extraosseous and spinal intradural chordoma with diffuse spinal leptomeningeal spread. The patient died 2 months postoperatively.
CONCLUSIONS: An intradural spinal chordoma is difficult to distinguish from a neurogenic tumor by imaging. When the lesion is dumbbell-shaped, it is easily misdiagnosed as a schwannoma. In the present case, the tumor was intradural and located at the level of the C6 and C7 vertebrae. Preoperative diagnosis was difficult, and the final diagnosis required pathological examination.