Multicentric Chordoma With Initial Resection by Bilateral Transcondylar Approach: 2-Dimensional Operative Video.

Abstract

Chordoma is a rare skull base tumor with malignant behavior.1-3 It invades locally with high recurrences, metastasizes distally, and seeds after interventions.1-4 Chordoma exemplifies the malignant progression doctrine as it accumulated genetic mutations. The natural history of untreated disease is 2.4 yr on average survival.5 Best tumor control is achieved by radical resection, followed by high doses radiation. Multicentric chordoma is an ill-defined challenging entity extremely rare in the literature. However, chordoma is known for distal metastasis, particularly to the lungs, iatrogenic cerebrospinal fluid (CSF) dissemination with drop metastasis, or surgical implantation. A subset of patients present with synchronous or metachronous regional or distal neuraxial lesions associated with the initial chordoma. Patients presenting with multicentric bony axial lesions and no extra-axial metastases point toward the multicentric chordoma concept rather than local, hematogenous, or CSF spread.6-12 Biopsy of these multicentric lesions can show a spectrum of abnormalities ranging from benign notochordal tumor to chordomas confirming the multicentric hypothesis.9 We present a patient who underwent a bilateral transcondylar approach for giant craniovertebral junction chordoma and then treated with radiation and a second lesion at the C6 transverse foramen. Six years later, she presented with a chordoma at the petrous apex. The patient consented to surgery and to the publications of her image. The participants and any identifiable individuals consented to publication of his/her image. Image at 1:39 reprinted with permission from Al-Mefty O, Operative Atlas of Meningiomas. Vol 1, © LWW, 1998.