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J Neurol Surg Rep 2014; 75(01): e160-e169
DOI: 10.1055/s-0034-1378157
Case Report
Georg Thieme Verlag KG Stuttgart · New York

Giant Petroclival Primary Intradural Chordoma: Case Report and Systematic Review of the Literature

Fahad AlOtaibi
1   Division of Neurosurgery, Department of Radiation Oncology, McGill University, Jewish General Hospital, Montreal, Quebec, Canada
2   Department of Neurosurgery, National Neuroscience Institute, King Fahad Medical Center, Riyadh, Saudi Arabia
,
Marie-Christine Guiot
3   Department of Neuropathology, McGill University, Montreal Neurological Institute, Montreal, Quebec, Canada
,
Thierry Muanza
4   Department of Radiation Oncology, McGill University, Jewish General Hospital, Montreal, Quebec, Canada
,
Salvatore Di Maio
1   Division of Neurosurgery, Department of Radiation Oncology, McGill University, Jewish General Hospital, Montreal, Quebec, Canada
› Author Affiliations
Further Information

Publication History

12 February 2014

09 April 2014

Publication Date:
26 June 2014 (online)

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Abstract

Background Chordomas are rare, locally aggressive neoplasms thought to arise from notochordal remnants in the axial skeleton. Primary intradural chordomas are considered to be extremely rare. In this article a giant intradural petroclival chordoma is presented, and a synthesis of the available literature is performed to measure overall survival (OS) and recurrence-free survival (RFS) and to identify prognostic factors.

Methods A systematic Medline review yielded 47 patients with purely intradural tumors from 38 publications including 39 chordomas, 8 cases of ecchordosis physaliphora, and 1 case with features of both. The 5-year OS and RFS were calculated based on the Kaplan-Meier method. Risk factors for progression or mortality were analyzed using binomial logistic regression.

Results Maximal tumor diameter varied from 1.5 to 6.0 cm (mean: 3.2 cm). Tumors were located predominantly in the prepontine area (66.7%). Combined 5-year Kaplan-Meier OS and RFS were 77% ± 11% and 74% ± 11%, respectively. Incomplete surgical resection, larger tumor diameter, and an elevated Ki-67 index were statistically more frequent in cases of recurrence and mortality.

Conclusions Based on a systematic literature review, the behavior of primary intradural chordomas may be closer to typical chordomas than was previously thought.

Keywords

chordoma - notochord - skull base neoplasms - brainstem neoplasms
 

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