Intracranial Chordoma in Children

Sassin, Jon F.; Chutorian, Abe M.

doi:10.1001/archneur.1967.00470250093010

Cited by 48 publications

(12 citation statements)

References 13 publications

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“…Although these cells usually have an intraosseous location, they occasionally perforate through the dorsal wall of the clivus into the subdural and subarachnoid spaces, which may explain the pathogenesis of EP at the prepontine region. 4,18,28,29,36 With EP, there is often a bony stalk that connects it with the clivus, emphasizing its clival origin. 17,24,32 This potential continuum of cells from the clivus to the intradural space explains the occurrence of lesions anywhere along this path, such as was seen in our case in which the lesion was isolated in the epidural spaces.…”

Section: Discussionmentioning

confidence: 99%

“…Unlike chordomas, which are radically resected and then treated with proton beam radiation given their destructive and invasive behavior, EPs can be managed expectantly with serial MRI follow-up since they have a low propensity for growth. 28,32 Giant symptomatic EPs, however, may require resection. To date, 3 reported cases of giant EPs in adults have been reported, but no case has occurred in a pediatric patient and never one restricted to the epidural space.…”

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Giant ecchordosis physaliphora in an adolescent girl

Krisht

Palmer²,

Osborn

et al. 2013

PED

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The authors describe a rare case of giant ecchordosis physaliphora (EP) in a 16-year-old female patient who presented with diplopia. Magnetic resonance imaging of the brain with Gd contrast revealed a 3.0 × 1.7 × 1.8–cm nonenhancing, extraaxial epidural mass along the dorsal aspect of the clivus that was T2 hyperintense and T1 isointense with intermediate signal on diffusion sequences. Resection via a transnasal transsphenoidal approach to the ventral clival wall resulted in a stable tumor size with no evidence of interval growth after 30 months. Although this case features a strictly extradural EP, this tumor more commonly occurs in the subdural space and requires differentiation from intradural chordoma. Unlike EP, intradural chordoma may enhance with Gd contrast, is more likely to be associated with cranial nerve palsies and brainstem symptoms, and will occasionally have an elevated MIB-1 index. In this paper the authors highlight the different possible midline locations for both EP and chordoma, the difficulty in distinguishing between intradural giant EP and intradural chordoma, and the potential occurrence of these lesions in young people despite their typically slow rate of growth, while also underscoring the need for further investigation into the tumors' cytogenetic behavior.

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

Giant ecchordosis physaliphora in an adolescent girl

Krisht

Palmer²,

Osborn

et al. 2013

PED

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“…The rarity of this neoplasm was pointed out by G hntil and C oley [10], M abrey [ 16], Sassen and C hutorian [20] and F orti [7], According to Mabrey [16|, G ardner and T urner [9] and Kamrin et al [14], 48-55% of all chordomas have a sacrococcygeal localization. 25-30°/o are intracranial and 10-25% in the spine, but recent reports in dicate that they are just as common inside the cranium as in the sacrococ cygeal region [8,11,21], Intracranial chordomas are more frequent in the 3rd and 4th decades of life, while the sacrococcygeal localization predominates in the 5th and 6th decades [1,2,6,11,12], They are very rare in infancy and childhood [3,20], Chordomas are benign neoplasm and characteristically show a slow growth with metastatic potential. Fox et al [8] indicated that these tu mors may metastasize to the regional lymph nodes, liver, lungs, heart, bone, adrenal glands, thyroid, pancreas, kidneys, urinary bladder, perito neum, pleura and skin.…”

Section: Discussionmentioning

confidence: 99%

Unusual Subarachnoid Metastasis of an Intracranial Chordoma in Infancy

Plese¹,

Borges²,

Nudelman³

et al. 1978

Pediatr Neurosurg

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“…7,11,14 But this proportion seems superior in younger children, which could explain, in part, a worse outcome. 15,16 Interestingly, multiple reports concern unusual presentations in youngsters, such as: extra clival cranial, intra cerebral, mediastinal, gluteal, etc. [17][18][19][20][21][22][23][24][25][26][27][28][29] Symptoms at presentation depend on the tumor site, and are not specific to children.…”

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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Intracranial Chordoma in Children

Cited by 48 publications

References 13 publications

Giant ecchordosis physaliphora in an adolescent girl

Giant ecchordosis physaliphora in an adolescent girl

Unusual Subarachnoid Metastasis of an Intracranial Chordoma in Infancy

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

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