Chordoma: update on disease, epidemiology, biology and medical therapies

Purpose of review Chordoma is an exceedingly rare subtype of bone sarcoma. This review aims to provide a comprehensive insight into chordoma epidemiology, and an update on the recent advances in disease, biology and medical therapies. Recent findings The incidence of chordoma is approximately 0.08/100 000 and the 5-year overall age-adjusted relative survival is 72% in the United States and 61% in Europe. Over the last years, significant steps forwards have been done in the comprehension of chordoma complexity, with insights gained into the biology and morphology of this disease. New entities have been described and potentially druggable molecular targets identified. This is becoming all the more relevant today, as new potentially active agents are under development. Summary Chordoma is a complex disease because of its rarity, biological heterogeneity and peculiar clinical behaviour. Despite the progress done, the outcome in this disease remains unsatisfactory and the identification of active systemic treatments remains an urgent, unmet medical need.
Source: Current Opinion in Oncology - Category: Cancer & Oncology Tags: SPECIAL COMMENTARY Source Type: research

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rer F Abstract BAF complexes are chromatin remodelling complexes made up of 15 subunits which overview transcription regulation. A subset of their subunits are notoriously linked to cancer, with the examples of SMARCB1, SMARCA4, ARID1A/1B and PBRM1. The complexes act as tumor suppressor genes, commonly mutated in a wide array of malignancies with an overrepresentation of sarcomas and tumors of the central nervous system. The recurrent inactivation of their genes points towards their driving role in the tumorigenesis of SMARCB1 in malignant rhabdoid tumors and SMARCA4 in small cell carcinoma of the ovary, hypercalc...
Source: Annales de Pathologie - Category: Pathology Authors: Tags: Ann Pathol Source Type: research
In conclusion, B7-H3 could be a target of bone cancers.
Source: Pathology Research and Practice - Category: Pathology Source Type: research
Summary of Background Data: Primary osseous spinal neoplasms (POSNs) include locally aggressive tumors such as osteosarcoma, chondrosarcoma, Ewing sarcoma, and chordoma. For such tumors, surgical resection is associated with improved survival for patients. Socioeconomic predictors of receiving surgery, however, have not been studied. Objective: To examine the independent effect of race on receiving surgery and survival probability in patients with POSN. Study Design: A total of 1904 patients from the SEER program at the National Cancer Institute database, all diagnosed with POSN of the spinal cord, vertebral column...
Source: Journal of Spinal Disorders and Techniques - Category: Surgery Tags: PRIMARY RESEARCH Source Type: research
Conditions:   Epithelioid Sarcoma;   Spindle Cell Sarcoma;   Sinonasal Carcinoma;   Small Cell Carcinoma of the Ovary Hypercalcemic Type;   Thoracic Sarcoma;   Poorly Differentiated Chordoma Intervention:   Drug: Tazemetostat Sponsor:   Epizyme, Inc. Temporarily not available
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
Publication date: Available online 29 January 2019Source: Journal of Bone OncologyAuthor(s): Pichaya Thanindratarn, Dylan C. Dean, Scott D. Nelson, Francis J. Hornicek, Zhenfeng DuanAbstractBone sarcomas are a collection of sporadic malignancies of mesenchymal origin. The most common subtypes include osteosarcoma, Ewing sarcoma, chondrosarcoma, and chordoma. Despite the use of aggressive treatment protocols consisting of extensive surgical resection, chemotherapy, and radiotherapy, outcomes have not significantly improved over the past few decades for osteosarcoma or Ewing sarcoma patients. In addition, chondrosarcoma and ...
Source: Journal of Bone Oncology - Category: Cancer & Oncology Source Type: research
ConclusionThe reported technique allowsen bloc resection of sacral tumours up to S1, through a posterior-only approach. It is less invasive with minimal morbidity. The functional and oncological outcomes are similar to those reported by other investigators.Graphical abstractThese slides can be retrieved from electronic supplementary material.
Source: European Spine Journal - Category: Orthopaedics Source Type: research
CONCLUSION: Surgical en bloc resection is the primary treatment for sacral chordoma. Carbon ion therapy is used when it is difficult to obtain wide surgical margins. Due to morbidity and the disabling sequelae of surgery, adrotherapy may be considered an alternative to high (above S2-S3) sacral chordoma resections. PMID: 30204925 [PubMed - as supplied by publisher]
Source: Surgical Technology International - Category: Surgery Tags: Surg Technol Int Source Type: research
Primary malignant tumors of the spine are rare and mainly include chordoma, chondrosarcoma, Ewing sarcoma or primitive neuroectodermal tumor, and osteosarcoma. The final diagnosis is based on the combination of patient age, topographic and histologic features of the tumor, and lesion pattern on computed tomography (CT) and magnetic resonance (MR) imaging. Imaging evaluation includes radiography, CT, bone scintigraphy, and MR imaging. CT is more useful than radiography for evaluating location of the lesion and analyzing bone destruction and matrix, whereas MR has unmatched ability to assess soft tissue extension. This picto...
Source: Journal of the Belgian Society of Radiology - Category: Radiology Source Type: research
Abstract Sarcomas are a common type of tumor within the pediatric population. The utilization of proton therapy as a primary attribute the ability to spare adjacent healthy tissue, therefore, proton therapy has become a preferential indication in pediatrics compared to other photon irradiation modalities. Proton therapy is also a proven and historically validated irradiation technique in the treatment of chondrosarcomas and chordomas of the skull base and spine. Additionally, proton therapy can potentially limit irradiated healthy tissue volumes in adults and limit the risk of acute and late toxicities. The evalua...
Source: Bulletin du Cancer - Category: Cancer & Oncology Authors: Tags: Bull Cancer Source Type: research
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