Cranial chordomas in children and adolescents

Borba, Luis A. B.; Al‐Mefty, Ossama; Mrak, Robert E.; Suen, James Y.

doi:10.3171/jns.1996.84.4.0584

Cited by 151 publications

(129 citation statements)

References 44 publications

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“…9 Cranial base chordomas have been reported in all age groups, including children and adolescents. 10,20,49,59,71,90,106 In large series, the median age at diagnosis is 46 years. …”

mentioning

confidence: 99%

Skull base chordomas: overview of disease, management options, and outcome

Lanzino¹,

As²,

Mb³

et al. 2001

FOC

101

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Cranial base chordomas are locally invasive tumors that, from a midline, clival location, extend in different directions and display various patterns of skull base invasion. Although histologically benign, their invasive nature makes true “oncological” resection virtually impossible to achieve in most cases, despite modern skull base surgical techniques. Moreover, because of the tumor's location and proximity to critical neural and vascular structures, surgery-related morbidity can be significant when an aggressive resection is undertaken. Cytoreductive surgery assumes a critical role in the management of these lesions. The choice of surgical approach and the extent of resection are dependent on several factors: location and extension of the tumor, the surgeon's philosophy and familiarity with a specific approach, and the patient's preexisting clinical status. Proton-beam radiotherapy seems to be effective as an adjunct to surgery in achieving local tumor control. The timing of radiation therapy, however, remains controversial. Gamma knife surgery has been proposed as an adjunctive therapy, but the limited experience and short follow-up periods do not permit formulation of meaningful conclusions at this time. Recurrences are common, although in a subset of patients prolonged disease-free survival is demonstrated.

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“…9 Cranial base chordomas have been reported in all age groups, including children and adolescents. 10,20,49,59,71,90,106 In large series, the median age at diagnosis is 46 years. …”

mentioning

confidence: 99%

Skull base chordomas: overview of disease, management options, and outcome

Lanzino¹,

As²,

Mb³

et al. 2001

FOC

101

View full text Add to dashboard Cite

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“…[7][8][9] Mean age varies according to series between 8 and 12 years, most approaching 10 years. 10,11 There are case-reports of newborns affected with CHs. 12 Sex ratio is close to 1, although some series evidence a slight female predominance.…”

Section: Review Of Literaturementioning

confidence: 99%

“…If they are IC, cranial nerve palsies are seen in 60% or so. 10,30 Non specific symptoms of hydrocephalus are present in one-third of cases. 31 Cervical-S sites or extension include also frequently stiff neck and nasal obstruction.…”

Section: Review Of Literaturementioning

confidence: 99%

“…Metastases are more common in the course of the disease with a pediatric predominance compared with adults: 9-58% range vs. 7-40%. 10,31,37 In these situations, they are frequently associated with a local failure. 38 Some patterns of such "combined" failures are more frequently reported such as local + nodal or local + surgical route.…”

Section: Review Of Literaturementioning

confidence: 99%

“…<5 years of age) behave actually less favorably. 10,11,31,37,54 This seems to be related with a higher rate of undifferentiated histologies, and a more rapid pace of the disease, related with upfront or secondary metastases. These tumors are also more frequently located in the SC region with frequent advanced presentation that pose highly challenging surgical decisions (especially in case of S3 invasion).…”

Section: Review Of Literaturementioning

confidence: 99%

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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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