Clivus chordoma

Clivus chordomas are slow-growing yet aggressively invasive and destructive tumors, arising from the embryonic rests of the notochord.

Chordomas belong to the sarcoma family of tumors.

The location distribution of chordomas is 50% sacral, 35% (skull base chordoma), and 15% occur in the vertebral bodies of the mobile spine (most commonly the C2 vertebrae followed by the lumbar then thoracic spine) 1).

Poorly differentiated chordoma with SMARCB1/INI1 loss: a distinct molecular entity with dismal prognosis 2).

The most common presenting symptoms of clivus chordoma are headache, diplopia, (abducens nerve palsy), dysphagia and dysarthria, and facial sensory changes 3).

see Chordoma diagnosis.

The anterior meningeal artery arises at the body of C2 (axis), may feed chordomas or foramen magnum meningiomas, may also act as collateral in vascular occlusion.

A systematic search of the literature was done by Labidi et al. in March 2016 using EMBASE and PubMed for articles published between January 2006 and March 2016 to identify surgical series of clivus chordomas. Only articles describing chordomas cases arising from the clivus of craniocervical junction were included in the analysis.

Twenty-seven articles were included in this systematic review, amounting to a total of 1050 patients. The weighted mean rate of GTR was 39.9% (range 0-78.3%) in this patient population. The surgical approaches were described in 16 papers, with 6 series reporting on surgeries done exclusively through the midline corridor (116 patients). In the remaining 10 series (495 patients), the anterior midline approach (AMA) was used in a mean of 56.8% of cases. In studies including patients operated solely through an AMA, a higher GTR rate was obtained (60.7% vs. 42.0%). Postoperative complications were also different between the two cohorts, with lower cranial nerves deficits and CNS infections but higher incidence of Cerebrospinal fluid fistula in the AMA group than in mixed surgical series. In a weighted mean follow-up time of 52.1±21.9 months, recurrences were observed in 38.2% of the total population of patients. Among 423 patients, the weighted 5-year PFS was 49.9±12.1% and the 5-year OS was 73.9±11.2% (N.=391). A random effects model was performed, combining data from studies reporting recurrence rates in GTR and non-GTR (N.=610), with a total odds ratio of having a recurrence for patients who had GTR vs non-GTR of 0.289 (95CI 0.184-0.453).

In this systematic review and meta-analysis of studies published in the last decade, an estimated 5-year PFS of 49.9% and 5-year OS of 73.9% were obtained. The weighted mean GTR rate in the included study was 39.9%, with a significantly reduced occurrence of recurrence in complete resections. Although anterior midline approaches may allow for higher GTR rates and fewer neurological morbidity than traditional transcranial routes, their impact of long-term survival and disease control remains largely unknown 4).

Tenny S, Varacallo M. Chordoma. 2022 Feb 12. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan–. PMID: 28613596.
Cha YJ, Hong CK, Kim DS, Lee SK, Park HJ, Kim SH. Poorly differentiated chordoma with loss of SMARCB1/INI1 expression in pediatric patients: A report of two cases and review of the literature. Neuropathology. 2017 Aug 15. doi: 10.1111/neup.12407. [Epub ahead of print] PubMed PMID: 28812319.
Harbour JW, Lawton MT, Criscuolo GR, Holliday MJ, Mattox DE, Long DM. Clivus chordoma: a report of 12 recent cases and review of the literature. Skull Base Surg. 1991;1(4):200-6. PubMed PMID: 17170837; PubMed Central PMCID: PMC1656331.
Labidi M, Watanabe K, Bouazza S, Bresson D, Bernat AL, George B, Froelich S. Clivus chordomas: a systematic review and meta-analysis of contemporary surgical management. J Neurosurg Sci. 2016 Dec;60(4):476-84. PubMed PMID: 27303859.
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