Abstract
A woman presented with buttock and low back pain, voiding difficulty, a feeling of rectal fullness, and unintentional weight loss. A destructive tumor centered in the sacrum was identified on magnetic resonance imaging and resected, and pathologic examination diagnosed a chordoma. Which statement is most accurate?a.Chordomas are only seen in the setting of tuberous sclerosis complexb.This tumor is highly responsive to hemotherapeutic regimensc.Neurofibromatosis type 2 is characteristically associated with chordomad.Chordomas are usually sporadic Answer: d. Chordomas are usually sporadic Chordoma is a slow-growing malignant bone tumor that arises from remnant embryonic notochord cells and commonly involves the axial skeleton (sacrococcygeal, spinal, or spheno-occipital regions). Surgical resection with clear margins is the treatment of choice, with or without adjuvant radiation therapy, because these tumors are not very responsive to most chemotherapeutic regimens. Most chordomas are sporadic. Duplication of the T (brachyury) gene is suggested to confer susceptibility to familial chordoma.1Yang X.R. Ng D. Alcorta D.A. et al.T (brachyury) gene duplication confers major susceptibility to familial chordoma.Nat Genet. 2009; 41: 1176-1178Crossref PubMed Scopus (217) Google Scholar An association of chordoma with tuberous sclerosis complex has also been suggested.
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