Sacral Chordoma: Long-term Outcome of a Large Series of Patients Surgically Treated at Two Reference Centers

Radaelli, Stefano; Stacchiotti, Silvia; Ruggieri, Pietro; Donati, Davide María; Casali, Paolo G.; Palmerini, Emanuela; Collini, Paola; Gambarotti, Marco; Porcu, Luca; Boriani, Stefano; Gronchi, Alessandro; Picci, Piero

doi:10.1097/brs.0000000000001604

Cited by 75 publications

(44 citation statements)

References 36 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Although, much studies involving sacral chordoma have shown profound results[29–32], genomic determinants for skull base chordoma progression remain elusive, urging a need for further examination. Towards this goal, we have characterized genomic and transcriptomic profiles of skull base chordomas.…”

Section: Discussionmentioning

confidence: 99%

Genomic and transcriptomic characterization of skull base chordoma

et al. 2016

View full text Add to dashboard Cite

Skull base chordoma is a primary rare malignant bone-origin tumor showing relatively slow growth pattern and locally destructive lesions, which can only be characterized by histologic components. There is no available prognostic or therapeutic biomarker to predict clinical outcome or treatment response and the molecular mechanisms underlying chordoma development still remain unexplored. Therefore, we sought out to identify novel somatic variations that are associated with chordoma progression and potentially employed as therapeutic targets. Thirteen skull base chordomas were subjected for whole-exome and/or whole-transcriptome sequencing. In process, we have identified chromosomal aberration in 1p, 7, 10, 13 and 17q, high frequency of functional germline SNP of the T gene, rs2305089 (P = 0.0038) and several recurrent alterations including MUC4, NBPF1, NPIPB15 mutations and novel gene fusion of SAMD5-SASH1 for the first time in skull base chordoma.

show abstract

Section: Discussionmentioning

confidence: 99%

Genomic and transcriptomic characterization of skull base chordoma

et al. 2016

View full text Add to dashboard Cite

show abstract

“…In particular, RFS or local control (LC) of skull-base chordomas at 5 and 10 years is 47–76% and 42–71% [3, 4], respectively, while 5- and 10-year estimated RFS for mobile spine chordomas is 58% and 32% [5]. Similarly, the 5-, 10- and 15-year local relapse (LR) incidence is reported to be 30%, 46% and 57%, respectively, in a recently published series of primary and completely resected sacral chordomas [6]. In this study, a plateau in RFS was not observed even at 15 years.…”

Section: Introductionmentioning

confidence: 99%

Best practices for the management of local-regional recurrent chordoma: a position paper by the Chordoma Global Consensus Group

Stacchiotti¹,

et al. 2017

View full text Add to dashboard Cite

Chordomas are rare, malignant bone tumors of the skull-base and axial skeleton. Until recently, there was no consensus among experts regarding appropriate clinical management of chordoma, resulting in inconsistent care and suboptimal outcomes for many patients. To address this shortcoming, the European Society of Medical Oncology (ESMO) and the Chordoma Foundation, the global chordoma patient advocacy group, convened a multi-disciplinary group of chordoma specialists to define by consensus evidence-based best practices for the optimal approach to chordoma. In January 2015, the first recommendations of this group were published, covering the management of primary and metastatic chordomas. Additional evidence and further discussion were needed to develop recommendations about the management of local-regional failures. Thus, ESMO and CF convened a second consensus group meeting in November 2015 to address the treatment of locally relapsed chordoma. This meeting involved over 60 specialists from Europe, the United States and Japan with expertise in treatment of patients with chordoma. The consensus achieved during that meeting is the subject of the present publication and complements the recommendations of the first position paper.

show abstract

“…Chordoma is a rare primary bone tumour accounting for 1-4% of all bone malignancies [1][2][3][4][5], arising in the sacrum (50-60%), skull base (35%) or vertebral bodies (15%) [5,6]. En bloc resection has been the mainstay of the treatment [5,[7][8][9][10][11], although recent evidence has underlined the increasing role of adjuvant radiotherapy [12][13][14][15][16]. Although chordoma is histologically characterized as low grade, it is highly recurrent and aggressive and associated with a poor prognosis [17].…”

Section: Introductionmentioning

confidence: 99%

Sacral chordoma: do the width of surgical margin and the use of photon/proton radiotherapy affect local disease control?

Fujiwara

Tsuda

Stevenson

et al. 2019

International Orthopaedics (SICOT)

View full text Add to dashboard Cite

Purpose Chordoma is a rare but highly aggressive primary bone sarcoma that arises commonly from the sacrum. While en bloc resection has been the mainstay of the treatment, the role of resection margin in millimetres with/without adjuvant radiotherapy (RT) has been unknown. We investigated the prognostic impact of surgical margin width, adjuvant RT, and their combined factor for sacral chordoma. Methods Forty-eight patients who underwent surgical treatment between 1996 and 2016 were studied. Of these, 11 patients (23%) received adjuvant RT; photon RT in 7 (15%) and proton RT in 4 (8%). Margins were microscopically measured in millimetres from the resection surface to the closest tumour on histologic slides. Results The five year and ten year disease-specific survival was 88% and 58%, respectively, and the local recurrence (LR) rate was 48%. The LR rate with 0-mm, < 1.5-mm, and ≥ 1.5-mm margin was 50% (group 1), 50% (group 2: RT−, 61%; group 3: RT+, 14%), and 0% (group 4), respectively. We observed a significantly lower LR rate in patients with adjuvant photon/proton RT (18%) than without it (57%; p = 0.026), and no LR was observed after post-operative proton RT. The combined factor of margin with RT clearly stratified the LR risk: patients of group 1 (positive margin) and 2 (< 1.5-mm margin, RT−) had approximately 7.5× LR risk (p = 0.049) compared with those of group 3 (< 1.5-mm margin, RT+) and 4 (≥ 1.5-mm margin). Conclusion This study identified the lowest risk of local failure in tumour resection with ≥ 1.5-mm margin or negative but < 1.5mm margin with the use of adjuvant photon/proton radiotherapy for sacral chordoma. Early results of adjuvant proton RT demonstrated excellent local control.

show abstract

Sacral Chordoma: Long-term Outcome of a Large Series of Patients Surgically Treated at Two Reference Centers

Abstract: 3.

Cited by 75 publications

References 36 publications

Genomic and transcriptomic characterization of skull base chordoma

Genomic and transcriptomic characterization of skull base chordoma

Best practices for the management of local-regional recurrent chordoma: a position paper by the Chordoma Global Consensus Group

Sacral chordoma: do the width of surgical margin and the use of photon/proton radiotherapy affect local disease control?

Contact Info

Product

Resources

About