2015
DOI: 10.1097/moo.0000000000000140
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Contemporary management of clival chordomas

Abstract: Clival chordomas are locally aggressive tumors that are challenging to treat because of their unique biology, proximity to key neurovascular structures and poor prognosis. Currently, chordomas are optimally managed with aggressive surgery, whilst preserving key structures, and postoperative radiation in a multidisciplinary setting with an experienced team. The advancement of molecular techniques offers exciting future diagnostic and therapeutic options in the management of chordomas.

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Cited by 35 publications
(25 citation statements)
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“…Our understanding of the present consensus is that most specialists agree on three points: (1) “safe” microsurgery is indicated for all patients, although small, histopathologically verified tumours can be treated with only stereotactic radiosurgery (SRS) [ 19 , 26 , 27 , 53 ], (2) despite extensive surgical removal and adjuvant treatment, tumour recurrence/progression is common, and (3) chordomas require adjuvant treatment following initial therapy, often in close proximity to the index surgery [ 5 ].…”
Section: Introductionmentioning
confidence: 99%
“…Our understanding of the present consensus is that most specialists agree on three points: (1) “safe” microsurgery is indicated for all patients, although small, histopathologically verified tumours can be treated with only stereotactic radiosurgery (SRS) [ 19 , 26 , 27 , 53 ], (2) despite extensive surgical removal and adjuvant treatment, tumour recurrence/progression is common, and (3) chordomas require adjuvant treatment following initial therapy, often in close proximity to the index surgery [ 5 ].…”
Section: Introductionmentioning
confidence: 99%
“…The majority of chordomas involve the sacrococcygeal region but the spheno‐occipital region is implicated in 25%‐35% of cases . The differential diagnosis of these lesions includes chondrosarcoma and ecchordosis physaliphora, a benign lesion also of notochord origin . Definitive diagnosis of clival chordomas requires histological evaluation.…”
Section: Discussionmentioning
confidence: 99%
“…Although several stains are used in the diagnostic process, brachyury has become a mainstay of pathological evaluation of these lesions (Figure ). Brachyury is a transcription factor in notochord development and has a 98% sensitivity and 100% specificity for the diagnosis of chordomas when combined with cytokeratin staining . In addition to its diagnostic utility, brachyury has been targeted in emerging immunotherapy for unresectable chordomas .…”
Section: Discussionmentioning
confidence: 99%
“…Chordomas are diagnosed on contrast-enhanced MRI and bone CT, with an intermediate-low signal on T1- and a high signal on T2-weighted images, and a moderate to high contrast enhancement. Bone CT is useful for ascertaining bone erosion due to the lytic nature of the lesion 1415 .…”
Section: Introductionmentioning
confidence: 99%