Chordoma and Chondrosarcoma

These are rare tumours of the skull base (and other sites in the body- but we will concentrate on skull base here). They tend to present with cranial nerve palsies and sometimes pain. They require staging to ensure no lung/bone mets prior to treatment. There is variability in the natural history of the tumours- I have seen both fast and slow growth. Radical treatment is with surgical resection (maximal safe) then adjuvant high dose radiotherapy. Treatment often results in significant morbidity. Treatment does not differ widely between the two diagnoses- just that chordoma has an overall worse prognosis, has more evidence to support as much as a resection as possible, and requires a higher dose of radiotherapy.

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The papers here cover the overall management of newly diagnosed and recurrent chordoma. In addition, though proton therapy is considered the gold standard for radiotherapy due to the high dose required, the team in Cambridge have published their results of using photon radiotherapy (now that we can be a lot more conformal in the modern era) which are similar to those results using protons. (Similarly PMH, Toronto, have published similarly impressive results using photons). If the residual volume is small, a stereotactic boost may well provide sufficient dose escalation without compromise to OAR. For referral to proton therapy, there need to be at least a 3mm gap to the brainstem and 5mm to the optics, otherwise the proton team will not be able adequately reduce dose to key OAR. To achieve this, the surgical team may need to do a two staged operation to clear disease from different areas.

Chordoma: current concepts, management, and future directions
Brian P Walcott, Brian V Nahed, Ahmed Mohyeldin, Jean-Valery Coumans, Kristopher T Kahle, Manuel J Ferreira
Lancet Oncol 2012; 13: e69–76

Overview of Chordoma diagnosis and management

Tumour Volume and Dose Influence Outcome after Surgery and Highdose Photon Radiotherapy for Chordoma and Chondrosarcoma of the Skull Base and Spine
E.R. Gatfield , D.J. Noble , G.C. Barnett, N.Y. Early, A.C.F. Hoole, N.F. Kirkby , S.J. Jefferies , N.G. Burnet
Clinical Oncology 30 (2018) 243e253

Cambridge series of photon therapy for chordoma and chondrosarcoma which shows good results, and may offer an alternative to proton therapy where this is not available or possile

Best practices for the management of local-regional recurrent chordoma: a position paper by the Chordoma Global Consensus Group
ESMO group, S. Stacchiotti et al
Annals of Oncology 28: 1230–1242, 2017

Overview of treatment options in recurrent chordoma