Stereotactic Radiosurgery for Chordoma: A Report From the North American Gamma Knife Consortium

Department of Neurological Surgery, and Radiation Oncology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA.
Neurosurgery (Impact Factor: 3.62). 11/2010; 68(2):379-89. DOI: 10.1227/NEU.0b013e3181ffa12c
Source: PubMed


Although considered slow-growing, low-grade malignancies, chordomas are locally aggressive and destructive tumors with high recurrence rates.
To assess patient survival, tumor control, complications, and selected variables that predict outcome in patients who underwent Gamma Knife stereotactic radiosurgery (SRS) as primary, adjuvant, or salvage management for chordomas of the skull base.
Six participating centers of the North American Gamma Knife Consortium identified 71 patients who underwent SRS for chordoma. The median patient age was 45 years (range, 7-80 years). The median SRS target volume was 7.1 cm³ (range, 0.9-109 cm³), and median margin dose was 15.0 Gy (range, 9-25 Gy).
At a median follow-up of 5 years (range, 0.6-14 years) after SRS, 23 patients died of tumor progression. The 5-year actuarial overall survival after SRS was 80% for the entire group, 93% for the no prior fractionated radiation therapy (RT) group (n = 50), and 43% for the prior RT group (n = 21). Younger age, longer interval between initial diagnosis and SRS, no prior RT, < 2 cranial nerve deficits, and smaller total tumor volume were significantly associated with longer patient survival. The 5-year treated tumor control rate after SRS was 66% for the entire group, 69% for the no prior RT group, and 62% for the prior RT group. Older age, recurrent group, prior RT, and larger tumor volume were significantly associated with worse tumor control.
Stereotactic radiosurgery is a potent treatment option for small sized chordomas, especially in younger patients and as part of a multipronged attack that includes surgical resection when possible.

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    • "Delivery of newer photon-based radiotherapy techniques, especially intensity-modulated radiation therapy (IMRT), has produced encouraging results in skull base chordomas, either alone or in combination with heavy particle therapy.[3191] For smaller sized tumors, stereotactic radiosurgery may also play a role, and early results have suggested comparable outcomes to other radiation modalities for residual or recurrent cases.[40] "
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    ABSTRACT: Chordomas of the skull base are rare locally aggressive neoplasms with a predilection for encapsulating critical neurovascular structures, bony destruction and irregular growth patterns, and from which patients succumb to recurrence and treatment failures. A review of the medical literature is performed, using standard search engines and identifying articles related to skull base chordomas, surgery, radiation therapy, chemotherapy, molecular genetics, and prospective trials. A synthesis of the literature is presented, including sections on pathology, treatment, molecular genetics, challenges, and future directions. Beyond an understanding of the current treatment paradigms for skull base chordomas, the reader gains insight into the collaborative approach applied to orphan diseases, of which chordomas is a prime exemplar.
    No preview · Article · Jun 2013 · Surgical Neurology International
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    • "Nowadays, the improvements in surgical techniques allow more radical resection of these tumours, while frequently providing small residual lesions, which can be suitable for SRS. Again, very few clinical data have been published on this issue with promising preliminary results showing that SRS at marginal doses of 14–16 Gy could represent a valuable treatment option for small-sized chordomas residual after surgery or relapsing [65-68]. "
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    ABSTRACT: Stereotactic radiosurgery (SRS) is an important treatment option for intracranial lesions. Many studies have shown the effectiveness of photon-SRS for the treatment of skull base (SB) tumours; however, limited data are available for proton-SRS. Several photon-SRS techniques, including Gamma Knife, modified linear accelerators (Linac) and CyberKnife, have been developed and several studies have compared treatment plan characteristics between protons and photons. The principles of classical radiobiology are similar for protons and photons even though they differ in terms of physical properties and interaction with matter resulting in different dose distributions. Protons have special characteristics that allow normal tissues to be spared better than with the use of photons, although their potential clinical superiority remains to be demonstrated. A critical analysis of the fundamental radiobiological principles, dosimetric characteristics, clinical results, and toxicity of proton- and photon-SRS for SB tumours is provided and discussed with an attempt of defining the advantages and limits of each radiosurgical technique.
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    ABSTRACT: The optimal treatment of chordomas is maximal safe resection followed by radiation therapy. Data have shown that the use of protons has increased the local control of chordomas. Because of their physical properties, proton therapy has a sharp decline at its distal range, thereby minimizing collateral damage. However, this choice of radiation therapy has been limited based on the availability of resources. Given the high cost of proton facilities and improved techniques for other forms of radiation therapy, this article evaluates whether proton therapy is still superior to other radiation techniques in the treatment of chordomas.
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