Malignant Transformation of Thoracic Spinal Chordoma
Keywords:dedifferentiated chordoma, cord compression, thoracic spine, tumour recurrence
Chordoma of the thoracic spine is an uncommon low-grade malignant tumour of bone. It can undergo transformation into a sarcoma (dedifferentiated chordoma), characterised by an aggressive, clinical course. Sarcomatous transformation is thought to stem from exposure to therapeutic radiation although recent experience suggests it can occur de-novo, or at the site of tumour recurrence. Most dedifferentiated chordomas arise from the sacrococcygeal spine and only three elderly patients were reported to suffer from this form of chordoma of the thoracic spine. This case report is of interest as the disease affected a 15-year-old-boy. He presented with progressive paraparesis. Computed tomography revealed a bone-eroding tumour at T4, characteristic of a chordoma. Despite radical excision and adjuvant radiotherapy, it was discovered he had metastasis in bone and lymph nodes five months later. The tumour had probably dedifferentiated into a sarcoma.
McMasters 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): 1-11.
Walcott BP, Nahed BV, Mohyedin A, Coumans JV, Kahle KT, Ferreira MJ. Chordoma: current concepts, management, and future directions. Lancet Oncol. 2012; 13(2): e69-76.
Heffelfinger MJ, Dahlin DC, MacCarty CS, Beabout JW. Chordomas and cartilaginous tumors of the skull base. Cancer 1973; 32(2): 410-420.
Bisceglia M, Dâ€™Angelo VA, Guglielmi G, Dor DB, Pasquinelli G. Dedifferentiated chordoma of the thoracic spine with rhabdomyosarcomatous differentiation. Report of a case and review of the literature. Annals of Diagnostic Pathology 2007; 11(4): 262-273.
Coffin CM, Swanson PE, Wicks MR, Dehner LP. Chordoma in childhood and adolescence. A clinicopathologic analysis of 12 cases. Arch Pathol Lab Med. 1993; 117(9): 927-933.
Ridenour RV, Ahrens WA, Folpe AL, Miller DV. Clinical and histopathologic features of chordomas in children and young adults. Pediatric and Developmental Pathology 2010; 13(1): 9-12.
Rodellac MH, Feydy A, Larousserie F, Anract P, Campagna R, Babinet A, Zins M, et al. Diagnostic imaging of solitary tumors of the spine: what to do and say. Radiographics 2008; 28(4): 1019-1041.
Katonis P, Datsis G, Karantanas A, Kampouroglou A, Lianoudakis S, Licoudis S, Papoutsopoulou E, et al. Spinal osteosarcoma. Clinical Medicine Insights: Oncology 2013; 7: 199-208.
Smolders D, Wang X, Drevelengas A, Vanhoenacker F, De Schepper AM. Value of MRI in the diagnosis of non-clival, non-sacral chordoma. Skeletal Radiol. 2003; 32(6): 343-350.
Yoem KW, Lober RM, Mobley BC, Harsh H, Vogel H, Allagio R, Pearson M et al. Diffusion-weighted MRI: Distinction of skull base chordoma from chondrosarcoma. AJNR Am J Neuroradiol. 2013; 34(5): 1056-1061.
Hanna SA, Tirabosco R, Amin A, Pollock RC, Skinner JA, Cannon SR, Saifuddin A, et al. Dedifferentiated chordoma. A report of four cases arising â€˜de novoâ€™. J Bone Joint Surg (Br). 2008; 90-B(5): 652-656.
Sunderesan N, Galicich JH, Chu FCH, Huvos AG. Spinal chordomas. J Neurosurgery 1979; 50(3): 312-319.
Topsakal C, Bulut S, Erol FS, Ozercan I, Yidirim H. Chordoma of the thoracic spineâ€”case report. Neurol Med Chir (Tokyo) 2002; 42(4): 175-180.
Chambers PW, Schwinn CP. Chordoma. A clinicopathologic study of metastasis. Am J Clin Pathol 1979; 72(5): 765-766.
Clines GA, Guise TA. Hypercalcaemia of malignancy and basic research on mechanisms responsible for osteolytic and osteoblastic metastasis to bone. Endocr Relat Cancer 2005; 12(3): 549-583.
Fox JE, Batsakis JG, Owano LR. Unusual manifestations of chordoma. A report of two cases. J Bone Joint Surg Am. 1968; 50(8): 1618-1628.
Nanda A, Hirsh LF, Antoiniades K. Malignant fibrous histiocytoma in a recurrent thoracic chordoma: case report and literature review. Neurosurgery 1991; 28(4): 588-592.
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