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CHORDOMA PowerPoint Presentation

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Description : Check out this medical presentation on Radiology and Imaging, which is titled "CHORDOMA", to know about chordoma and its diagnosis via neuroimaging (xray, CT, MRI).

Tags : Radiology, Imaging, chordoma, diagnosis, neuroimaging, xray, CT, MRI

Published on : Jul 28, 2021 | Views : 1564 | Downloads : 1

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Slide 1-

CHORDOMA Ade Wijaya, MD January 2019
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Outline: Introduction Epidemiology Predilection Pathogenesis Clinical presentation Diagnosis Management Summary
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Introduction Rare 1-4 % of bone malignancies Histologically  a low-grade neoplasm, but highly recurrent, making their clinical progression very similar to that of malignant tumours Slow growing, radioresistant tumours that are locally aggressive and invasive Healey JH, Lane JM. Chordoma: a critical review of diagnosis and treatment. Orthop Clin North Am 1989; 20: 417–26. Bergh P, Kindblom LG, Gunterberg B, Remotti F, Ryd W, Meis-Kindblom JM. Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients. Cancer 2000; 88: 2122–34. Schwab JH, Boland PJ, Agaram NP, et al. Chordoma and chondrosarcoma gene profi le: implications for immunotherapy. Cancer Immunol Immunother 2009; 58: 339–49.
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Epidemiology Incidence: 0.08 per 100.000 M > F Peak incidence: 50-60 years old Very low incidence in < 40 years old Rarely affect children and adolescents McMaster ML, Goldstein AM, Bromley CM, Ishibe N, Parry DM. Chordoma: incidence and survival patterns in the United States, 1973–1995. Cancer Causes Control 2001; 12: 1–11
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Predilection Skull Base Sacrum Spine McMaster ML, Goldstein AM, Bromley CM, Ishibe N, Parry DM. Chordoma: incidence and survival patterns in the United States, 1973–1995. Cancer Causes Control 2001; 12: 1–11
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Pathogenesis Chordomas were first characterised microscopically by Virchow in 1857 Unique, intracellular, bubble-like vacuoles that he referred to as physaliferous Ribbert first introduced the term chordoma in the 1890s Derived from undifferentiated notochordal remnants that reside within the vertebral bodies and throughout the axial skeleton Gene duplication in the transcription factor T gene (brachyury) in familial chordoma Virchow RL. Untersuchungen ueber die Entwicklung des Schaedelgrundes. Berlin: G Rimer, 1857. Horten BC, Montague SR. In vitro characteristics of a sacrococcygeal chordoma maintained in tissue and organ culture systems. Acta Neuropathol 1976; 35: 13–25. Ribbert H. Uber die Ecchondosis physaliphora phenooccipitalis. Centralbl Allg Pathol Anat 1894; 5: 457–61 Yang XR, Ng D, Alcorta DA, et al. T (brachyury) gene duplication confers major susceptibility to familial chordoma. Nat Genet 2009; 41: 1176–78
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Clinical Presentation Indolent, slow growing  clinically silent until late stage of the diseases Skull base chordoma: cranial nerve palsies, endocrinopathy, epistaxis, intracranial hemorrhage Spinal & sacral chordoma: pain and radiculopathies symptoms; late: bladder and bowel dysfunction. Cervical  airway obstruction & dysphagia 5% of chordomas show metastasis to the lungs, bone, skin, and brain at the time of initial presentation, and as high as 65% are metastatic in very advanced disease Walcott BP, Nahed BV, Mohyeldin A, Coumans JV, Kahle KT, Ferreira MJ. Chordoma: current concepts, management, and future directions. The lancet oncology. 2012 Feb 1;13(2):e69-76.
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Diagnosis Neuroimaging: xray, CT, MRI Biopsy: classical (conventional), chondroid, or dedifferentiated immunoreactivity for S-100 and epithelial markers such as epithelial membrane antigen (MUC1) and cytokeratins Chugh R, Tawbi H, Lucas DR, Biermann JS, Schuetze SM, Baker LH. Chordoma: the nonsarcoma primary bone tumor. Oncologist 2007; 12: 1344–50. Crapanzano JP, Ali SZ, Ginsberg MS, Zakowski MF. Chordoma: a cytologic study with histologic and radiologic correlation. Cancer 2001; 93: 40–51 Mitchell A, Scheithauer BW, Unni KK, Forsyth PJ, Wold LE, McGivney DJ. Chordoma and chondroid neoplasms of the spheno-occiput. An immunohistochemical study of 41 cases with prognostic and nosologic implications. Cancer 1993; 72: 2943–49. Abenoza P, Sibley RK. Chordoma: an immunohistologic study. Hum Pathol 1986; 17: 744–47
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Management Surgery Radiation therapy Medical treatment: on research Multidisciplinary approach SURVIVAL: 5 year, 10 year, and 20 year survival dropping precipitously to 67·6%, 39·9%, and 13·1%, respectively, across all races and sex Walcott BP, Nahed BV, Mohyeldin A, Coumans JV, Kahle KT, Ferreira MJ. Chordoma: current concepts, management, and future directions. The lancet oncology. 2012 Feb 1;13(2):e69-76.
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Management The gold standard treatment for chordomas of the mobile spine and sacrum is en- bloc excision with wide margins and postoperative external-beam radiation therapy. Treatment of clival chordomas is unique from other locations with an enhanced emphasis on preservation of neurological function, typified by a general paradigm of maximally safe cytoreductive surgery and advanced radiation delivery techniques Walcott BP, Nahed BV, Mohyeldin A, Coumans JV, Kahle KT, Ferreira MJ. Chordoma: current concepts, management, and future directions. The lancet oncology. 2012 Feb 1;13(2):e69-76.
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Summary Chordoma is a rare bone cancer that is aggressive, locally invasive, and has a poor prognosis Arise from transformed remnants of notochord and have a predilection for the axial skeleton, with the most common sites being the sacrum, skull base, and spine Management: multidisciplinary (surgery + radiation)
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