Chordomas and cartilaginous tumors at the skull base

Heffelfinger, M. Joan; Dahlin, David C.; MacCarty, Collin S.; Beabout, John W.

doi:10.1002/1097-0142(197308)32:2<410::aid-cncr2820320219>3.0.co;2-s

Cited by 589 publications

(349 citation statements)

References 18 publications

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“…It grows invasively, has a high rate of local recurrence, metastasizes, implants surgically, demonstrates Btumor progression^as it becomes more malignant by accumulating genetic mutations and has a dismal prognosis following recurrence [1][2][3]. For these reasons, chordoma needs to be treated aggressively.…”

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confidence: 99%

“…Owing to their rarity, studies and experience in chordoma are very limited. The landmark article of Heffelfinger et al lists only 55 patients with clival chordomas over a 60-year period drawn to their world referral center [3]. This extreme rarity and the time passed between the different eras of management make it difficult but extremely critical to have a true long-term follow-up.…”

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confidence: 99%

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Chordoma

Al‐Mefty

2017

Acta Neurochir

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Chordoma

Al‐Mefty

2017

Acta Neurochir

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“…2 Treatment options have included extensive surgical resection, external beam radiotherapy alone, or a combination of the two with average survival times of 1.5, 4.8, and 5.2 years, respectively. 2 Complete resection, however, is nearly impossible and because of tumour insensitivity, high radiotherapy doses between 50 and 80 Gy are reported necessary to achieve palliation. 4 Above 50 Gy, postradiation injury to the optic nerves, chiasm, or tracts has been reported.…”

Section: Discussionmentioning

confidence: 99%

Visual complications of proton beam therapy for clival chordoma

Bowyer

Natha

Marsh

et al. 2003

Eye

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Purpose Combining maximal surgical resection with high-dose proton radiation therapy is reported to be currently the best management of patients with clival chordoma. Method Since 1991, four consecutive patients from our institution with this tumour have been referred for postoperative proton beam radiotherapy.Result We have experienced an unusually high complication rate (50%) of delayed radiation optic neuropathy. This has resulted in profound, bilateral visual loss at 1 and 2 years postproton beam treatment. Conclusion It has served as a reminder that proton beam therapy is not an innocuous treatment option and this devastating complication should be taken into account when discussing the management of clivus chordoma. We postulate whether the optic apparatus is particularly sensitive to proton vs photon damage.

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“…Furthermore, unlike the adults, the chondroid subtype does not seem to have a favorable impact on prognosis. 7,56 In the case of a local-regional failure, re-irradiation is increasingly considered a reasonable option, especially in the context of modern technologies. It still remains complex due to the subtle balance between a tumor dose level associated with a prolonged local control expectancy and a relatively safe dose to critical organs.…”

Section: Review Of Literaturementioning

confidence: 99%

Reprint of “Chordoma in children: Case-report and review of literature”

Habrand

Datchary

Bolle

et al. 2016

Reports of Practical Oncology & Radiotherapy

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Sarcoma Protontherapy Children a b s t r a c tWe report an exceptional case of a very late local failure in a 9-year-old boy presenting with a chordoma of the cranio-cervical junction. The child was initially treated with a combination of surgical resection followed by high dose photon-proton radiation therapy. This aggressive therapy allowed a 9-year remission with minimal side-effects. Unfortunately, he subsequently presented with a local failure managed with a second full-dose course of protons. The child died one year later from local bleeding of unclear etiology.© 2015 Greater Poland Cancer Centre. Published by Elsevier Sp. z o.o. All rights reserved. IntroductionChordomas ( with the brachyury gene, a gene involved in the notochord development. 5,6 In the adults, they are located typically in the sacrococcygeal (SC) (50%), the intra cranial (IC), at the skull base (SB) (35%), and the intermediate spinal (S) regions (15%). Only 5% of cases have been described in the pediatric age, with unclear specificities concerning presentation, and outcome. We report in this paper a clinical case of skull base CH http://dx

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Chordomas and cartilaginous tumors at the skull base

Cited by 589 publications

References 18 publications

Chordoma

Chordoma

Visual complications of proton beam therapy for clival chordoma

Reprint of “Chordoma in children: Case-report and review of literature”

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