Hypothalamic chordoma

Commins, D. J.; Baran, Gregg A.; Molleston, Michael C.; Vollmer, Dennis G.

doi:10.3171/jns.1994.81.1.0130

Cited by 10 publications

(8 citation statements)

References 7 publications

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“…It was a pineal chordoma with abdominal metastasis, due to previous ventriculoperitoneal shunt placement. The origin of such intraparenchymal tumours is not clear and it has been suggested that they arise from ecchordosis physaliphora (EP) or directly from notochord remnants that subsist within brain tissue [8,10]. The cases described suggest ectopic notochordal remnants may be located in different areas, even away from the neuroaxis.…”

Section: Discussionmentioning

confidence: 81%

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Intradural chordoma mimicking a lateral sphenoid wing meningioma: a

Kunert¹,

Dziedzic²,

Matyja³

et al. 2012

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Section: Discussionmentioning

confidence: 81%

“…The majority of these lesions were located in the prepontine space. Intradural extraosseous chordomas have occasionally been found within the brain, in hypothalamic and pineal regions [8,10]. Intradural chordomas are believed to be less aggressive and have a better prognosis for patients.…”

Section: Introductionmentioning

confidence: 99%

Intradural chordoma mimicking a lateral sphenoid wing meningioma: a

Kunert¹,

Dziedzic²,

Matyja³

et al. 2012

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“…[2] Ecchordosis physaliphora is a small, well-circumscribed intradural lesion usually found on the ventral surface of the brainstem, attached to the dorsum sellae and mostly discovered incidentally at autopsy. [7] Ecchordosis is thought to originate from migration of hamartomatous notochordal remnants to the intradural space via a small opening in the dura.…”

Section: Discussionmentioning

confidence: 99%

“…[7] Ecchordosis is thought to originate from migration of hamartomatous notochordal remnants to the intradural space via a small opening in the dura. [7] Despite the fact that it presents the same histological characteristics of chordoma, it is not a neoplasm[2] and its clinical behavior differs substantially from that of typical chordomas,[7] even though the possibility that intradural extraosseous chordomas may simply be giant ecchordosis physaliphora has also been pointed out. [2]…”

Section: Discussionmentioning

confidence: 99%

Ectopic pineal chordoma

Figueiredo

Tavares²,

Welling³

et al. 2011

Surg Neurol Int

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Background:Chordomas are rare tumors that arise from the remnants of embryonic notochord anywhere along the neuroaxis. Even though they may occur in an extraosseous intradural location, the most common sites include the sacrococcygeal and clivus regions. The authors report a unique presentation encompassing the pineal region with metastasis to the peritoneum after a ventriculoperitoneal (VP) shunt procedure and review the current knowledge about their pathophysiology and management. The presentation and clinical history endorse the idea that intradural extraosseous chordomas may be distinct from ecchordosis physaliphora and probably do not derive from it.Case Description:An 18-year-old male with previous history of VP shunt presented to the emergency room with pain and abdominal distension. Computed tomography (CT) scans revealed a mass in the pineal region and in the abdominal cavity. Histopathologic exams showed chordoma in both abdominal and cranial samples. The patient died due to systemic complications.Conclusion:The authors hypothesized that notochord remnants may subsist within the brain and occasionally may generate a neoplastic lesion.

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“…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. If they are IC, cranial nerve palsies are seen in 60% or so.…”

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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Hypothalamic chordoma

Abstract: The case is reported of a 51-year-old woman found to have a hypothalamic mass with the histological features typical of chordoma. This represents the first description of a chordoma arising within the brain.

Cited by 10 publications

References 7 publications

Intradural chordoma mimicking a lateral sphenoid wing meningioma: a

Intradural chordoma mimicking a lateral sphenoid wing meningioma: a

Ectopic pineal chordoma

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

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