Chordoma of the Mobile Spine and Sacrum: A Retrospective Analysis of a Series of Patients Surgically Treated at Two Referral Centers

Stacchiotti, Silvia; Casali, Paolo G.; Vullo, Salvatore Lo; Mariani, Luigi; Palassini, Elena; Mercuri, Mario; Alberghini, Marco; Pilotti, Silvana; Zanella, Licciana; Gronchi, Alessandro; Picci, Piero

doi:10.1245/s10434-009-0740-x

Cited by 173 publications

(137 citation statements)

References 39 publications

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“…6 This tumor is characterized by a dual epithelial mesenchymal differentiation, with immunostaining positive for EMA, cytokeratin, and S100. 1 The immunohistochemical evaluation performed in two of our cases followed similar pattern.…”

supporting

confidence: 61%

“…5 In a study done by Silvia Stacchioti et al in Italy a total of 138 consecutive patients were identified, of which sacrum was involved in 78%, lumbar spine in 15% and cervical-dorsal spine in 7%. 1 Notochordal remnants that are extradural are most common at the sacrococcygeal region. Possibly this explains the sacrococcygeal region being the most common site of involvement.…”

Section: Discussionmentioning

confidence: 99%

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Chordoma: study of five cases

Sigdel

Baidya

2012

J Pathol Nep

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Background: Chordoma is a rare slow growing but malignant midline bone tumor arising from the notochordal remnant and recapitulates the notochord at various stages of differentiation. They involve the axial skeleton. The aim of this study was to find the frequency of this tumor and to analyze the clinical, radiological and pathological findings.Materials and Methods: Cases of chordoma diagnosed at B & B Hospital from January 1999 to November 2011 were studied for clinicopathologic features and immunohistochemical profile.Results: There were 5 cases of chordoma during the study period. Females were affected more with male to female ratio of 1:1.5. Sixty percent of the tumors involved the sacrum, while 40% were intracranial and located at the base of the skull. Median age of diagnosis was 44.8 years. Four cases (80%) were conventional chordoma and 1 case (20%) was a chondroid variant. Two cases evaluated were immunoreactive for S-100 protein, pan-cytokeratin and EMA.Conclusion: Though a low grade tumor with prolonged clinical course, chordomas can cause extensive local destruction with significant morbidity and mortality. Hence a prompt diagnosis with adequate excision is important to save the patient’s life.Journal of Pathology of Nepal (2012) Vol. 2, 260-264DOI: http://dx.doi.org/10.3126/jpn.v2i4.6874

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supporting

confidence: 61%

Section: Discussionmentioning

confidence: 99%

Chordoma: study of five cases

Sigdel

Baidya

2012

J Pathol Nep

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“…Representative 4-lm-thick sections were subjected to immunohistochemical analysis using the streptavidin-biotin peroxidase complex system (UltraVision Large Volume Detection System Anti-Polyvalent, HRP; LabVision Corp.), as previously described [19] (Supplementary Fig S1), using the primary antibody raised against Brachyury (sc-20109: Santa Cruz Biotechnology, Inc.) [16,19,[27][28][29]. Tumor samples were evaluated for extension (percentage of cytoplasm-positive cells: 0, negative; 1, \25 % positive cells; 2, 26-50 % positive cells; 3, [50 % positive cells) and intensity (0, negative; 1, weak; 2, moderate; 3, strong) of the immunoreactions.…”

Section: Immunohistochemistry Analysismentioning

confidence: 99%

The embryonic Brachyury transcription factor is a novel biomarker of GIST aggressiveness and poor survival

et al. 2015

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Background The T-box transcription factor Brachyury was recently reported to be upregulated and associated with prognosis in solid tumors. Here, we proposed to evaluate the potential use of Brachyury protein expression as a new prognostic biomarker in gastrointestinal stromal tumors (GIST). Methods Brachyury protein expression was analyzed by immunohistochemistry in a cohort of 63 bona fide GIST patients. Brachyury expression profiles were correlated with patients' clinicopathological features and prognostic impact. Additionally, an in silico analysis was performed using the Oncomine database to assess Brachyury alterations at DNA and mRNA levels in GISTs. Results We found that Brachyury was overexpressed in the majority (81.0 %) of primary GISTs. We observed Brachyury staining in the nucleus alone in 4.8 % of cases, 23.8 % depicted only cytoplasm staining, and 52.4 % of cases exhibited both nucleus and cytoplasm immunostaining. The presence of Brachyury was associated with aggressive GIST clinicopathological features. Particularly, Brachyury nuclear (with or without cytoplasm) staining was associated with the presence of metastasis, while cytoplasm sublocalization alone was correlated with poor patient survival.Conclusions Herein, we demonstrate that Brachyury is overexpressed in GISTs and is associated with worse outcome, constituting a novel prognostic biomarker and a putative target for GIST treatment.

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“…After surgical therapy of this orphan disease chordoma recurs in up to 50% of patients and metastasizes in up to 40% (1). Only a minority is cured by surgery, the disease-free survival is generally short (2)(3)(4). The median survival is 6 to 7 years after diagnosis, although the range varies from months to 25 years.…”

Section: Introductionmentioning

confidence: 99%

Preclinical Characterization of Novel Chordoma Cell Systems and Their Targeting by Pharmocological Inhibitors of the CDK4/6 Cell-Cycle Pathway

et al. 2015

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Chordomas are tumors that arise at vertebral bodies and the base of the skull. Although rare in incidence, they are deadly owing to slow growth and a lack of effective therapeutic options. In this study, we addressed the need for chordoma cell systems that can be used to identify therapeutic targets and empower testing of candidate pharmacologic drugs. Eight human chordoma cell lines that we established exhibited cytology, genomics, mRNA, and protein profiles that were characteristic of primary chordomas. Candidate responder profiles were identified through an immunohistochemical analysis of a chordoma tissue bank of 43 patients. Genomic, mRNA, and protein expression analyses confirmed that the new cell systems were highly representative of chordoma tissues. Notably, all cells exhibited a loss of CDKN2A and p16, resulting in universal activation of the CDK4/6 and Rb pathways. Therefore, we investigated the CDK4/6 pathway and responses to the CDK4/6-specific inhibitor palbociclib. In the newly validated system, palbociclib treatment efficiently inhibited tumor cell growth in vitro and a drug responder versus nonresponder molecular signature was defined on the basis of immunohistochemical expression of CDK4/6/pRb (S780). Overall, our work offers a valuable new tool for chordoma studies including the development of novel biomarkers and molecular targeting strategies. Cancer Res; 75(18); 3823-31. Ó2015 AACR.

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Chordoma of the Mobile Spine and Sacrum: A Retrospective Analysis of a Series of Patients Surgically Treated at Two Referral Centers

Cited by 173 publications

References 39 publications

Chordoma: study of five cases

Chordoma: study of five cases

The embryonic Brachyury transcription factor is a novel biomarker of GIST aggressiveness and poor survival

Preclinical Characterization of Novel Chordoma Cell Systems and Their Targeting by Pharmocological Inhibitors of the CDK4/6 Cell-Cycle Pathway

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