Chordoma of the Spinal Column

Sciubba, Daniel M.; John, H.; Rhines, Laurence D.; Gokaslan, Ziya L.

doi:10.1016/j.nec.2007.09.006

Cited by 135 publications

(108 citation statements)

References 43 publications

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“…Chordomas account for 2% to 4% of primary malignant bone tumors and represent the most common primary malignant bone tumor of the spine (1)(2)(3). Chordomas are considered to be radiation resistant and refractory to cytotoxic chemotherapy (4,5).…”

Section: Introductionmentioning

confidence: 99%

A novel target for treatment of chordoma: signal transducers and activators of transcription 3

Yang

Schwab

Schoenfeld

et al. 2009

Molecular Cancer Therapeutics

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A major obstacle in the effective treatment of chordoma is that there are no identifiable biomarkers capable of predicting prognosis. Recent research has indicated that signal transducers and activators of transcription (Stat3) may be an important prognostic marker in some cancers, but its role in chordoma tumors has not been elucidated. In this study, the expression of Stat3 was evaluated in chordoma tissue microarray that contains 70 chordoma samples. Cells in the tissue microarray showed nuclear staining for phosphorylated Stat3 in all instances. The level of phosphorylated Stat3 expression correlated with the survival and severity of the disease. Three chordoma cell lines were exposed to SD-1029, a novel inhibitor of Stat3 activation. MTT assay showed that the growth of all chordoma cell lines was inhibited by SD-1029. The expression of Stat3 signaling cascade was inhibited in all chordoma cell lines after treatment with SD-1029. The cytotoxicity of the combination of SD-1029 and chemotherapeutic drugs is significantly better than either agent alone. Phosphorylation of Stat3 in chordoma cells in vitro and cellular proliferation in three-dimensional culture were inhibited by SD-1029. In conclusion, the Stat3 pathway is constitutively activated in chordomas and the level of expression may serve as a predictor for prognosis. Blockade of the Stat3 pathway represents a potential strategy for future treatment.

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Section: Introductionmentioning

confidence: 99%

A novel target for treatment of chordoma: signal transducers and activators of transcription 3

Yang

Schwab

Schoenfeld

et al. 2009

Molecular Cancer Therapeutics

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“…Tumors are most commonly sacrococcygeal, followed by skull base and mobile spine locations. 20 Surgery has been the standard local treatment, but marginnegative resection is not always feasible. Local recurrence rates are as high as 50%-100% with subtotal resection (STR) versus 0%-53% with en bloc, margin-negative resection.…”

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confidence: 99%

High-dose proton-based radiation therapy in the management of spine chordomas: outcomes and clinicopathological prognostic factors

Rotondo

Folkert

Liebsch

et al. 2015

SPI

136

119

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OBJECT Spinal chordomas can have high local recurrence rates after surgery with or without conventional dose radiation therapy (RT). Treatment outcomes and prognostic factors after high-dose proton-based RT with or without surgery were assessed. METHODS The authors conducted a retrospective review of 126 treated patients (127 lesions) categorized according to disease status (primary vs recurrent), resection (en bloc vs intralesional), margin status, and RT timing. RESULTS Seventy-one sacrococcygeal, 40 lumbar, and 16 thoracic chordomas were analyzed. Mean RT dose was 72.4 GyRBE (relative biological effectiveness). With median follow-up of 41 months, the 5-year overall survival (OS), local control (LC), locoregional control (LRC), and distant control (DC) for the entire cohort were 81%, 62%, 60%, and 77%, respectively. LC for primary chordoma was 68% versus 49% for recurrent lesions (p = 0.058). LC if treated with a component of preoperative RT was 72% versus 54% without this treatment (p = 0.113). Among primary tumors, LC and LRC were higher with preoperative RT, 85% (p = 0.019) and 79% (0.034), respectively, versus 56% and 56% if no preoperative RT was provided. Overall LC was significantly improved with en bloc versus intralesional resection (72% vs 55%, p = 0.016), as was LRC (70% vs 53%, p = 0.035). A trend was noted toward improved LC and LRC for R0/R1 margins and the absence of intralesional procedures. CONCLUSIONS High-dose proton-based RT in the management of spinal chordomas can be effective treatment. In patients undergoing surgery, those with primary chordomas undergoing preoperative RT, en bloc resection, and postoperative RT boost have the highest rate of local tumor control; among 28 patients with primary chordomas who underwent preoperative RT and en bloc resection, no local recurrences were seen. Intralesional and incomplete resections are associated with higher local failure rates and are to be avoided.

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“…4 However, a more aggressive en bloc resection offers a survival benefit over intralesional resection. 6,13,25,33 However, some authors have noted that the complexity of cervical spine anatomy may make en bloc resections of many tumors impossible. 3 At this point, en bloc resection of cervical lesions is limited to case reports and small series.…”

Section: Discussionmentioning

confidence: 99%

Fusion following lateral mass reconstruction in the cervical spine

Clarke

Zadnik

Groves

et al. 2015

SPI

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OBJECT Recently, aggressive surgical techniques and a push toward en bloc resections of certain tumors have resulted in a need for creative spinal column reconstruction. Iatrogenic instability following these resections requires a thoughtful approach to adequately transfer load-bearing forces from the skull and upper cervical spine to the subaxial spine. METHODS The authors present a series of 7 cases in which lateral mass reconstruction with a cage or fibular strut graft was used to provide load-bearing support, including 1 case of bilateral cage placement. RESULTS The authors discuss the surgical nuances of en bloc resection of high cervical tumors and explain their technique for lateral mass cage placement. Additionally, they provide their rationale for the use of these constructs throughout the craniocervical junction and subaxial spine. CONCLUSIONS Lateral mass reconstruction provides a potential alternative or adjuvant method of restoring the load-bearing capabilities of the cervical spine.

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Chordoma of the Spinal Column

Cited by 135 publications

References 43 publications

A novel target for treatment of chordoma: signal transducers and activators of transcription 3

A novel target for treatment of chordoma: signal transducers and activators of transcription 3

High-dose proton-based radiation therapy in the management of spine chordomas: outcomes and clinicopathological prognostic factors

Fusion following lateral mass reconstruction in the cervical spine

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