title: Giant petroclival primary intradural chordoma: case report and systematic review of the literature.
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.
authors.affiliation: Division of Neurosurgery, Department of Radiation Oncology, McGill University, Jewish General Hospital, Montreal, Quebec, Canada ; Department of Neurosurgery, National Neuroscience Institute, King Fahad Medical Center, Riyadh, Saudi Arabia.
authors.forename: Fahad
authors.initials: F
authors.lastname: AlOtaibi
authors.affiliation: Department of Neuropathology, McGill University, Montreal Neurological Institute, Montreal, Quebec, Canada.
authors.forename: Marie-Christine
authors.initials: MC
authors.lastname: Guiot
authors.affiliation: Department of Radiation Oncology, McGill University, Jewish General Hospital, Montreal, Quebec, Canada.
authors.forename: Thierry
authors.initials: T
authors.lastname: Muanza
authors.affiliation: Division of Neurosurgery, Department of Radiation Oncology, McGill University, Jewish General Hospital, Montreal, Quebec, Canada.
authors.forename: Salvatore
authors.initials: S
authors.lastname: Di Maio
keywords: brainstem neoplasms; chordoma; notochord; skull base neoplasms
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