Chordoma U nguyên sống

Sacrococcygeal Chordoma • Chordomas are uncommon malignant tumors of the axial skeleton that account for 1% of intracranial tumors and 4% of all primary bone tumors.  • They originate from embryonic remnants of the primitve notochord Location • • • • Sacrococcygeal: 30-50%; commonly involving the fourth and fifth sacral segments/ M:F rato 2:1, and the tumor partcularly large at presentaton Chordoma is the most common primary malignant sacral tumor spheno-occipital: 30-35% vertebral body: 15-30% Epidemiology • • • Usually seen in adults (30-70 years) • Rarely, chordomas are encountered in children with tuberous sclerosis Spheno-occipital region most commonly occur in patents 20-40 years of age sacrococcygeal chordomas are typically seen in a slightly older age group (peak around 50 years Clinical presentation • • • • • They are slow-growing tumors: palpable mass (e.g sacrococcygeal chordoma) Low back pain or tail bone pain Weakness and/or numbness in the legs Loss of bladder and bowel control Radiographic features CT • • • • centrally located well-circumscribed destructve lytc lesion expansile soft-tssue mass – usually hyper-attenuatng; inhomogenous areas may be seen due to necrosis or hemorrhage • • – soft-tssue mass is often disproportonately large relatve to the bony destructon irregular intratumoral calcificatons (thought to represent sequestra of normal bone rather than dystrophic calcificatons) moderate to marked enhancement  • • MRI • • T2: most exhibit very high signal  T1 – intermediate to low-signal intensity – small foci of hyperintensity (intratumoral hemorrhage or a mucus pool) T1 C+ (Gd) – heterogeneous enhancement with a honeycomb appearance corresponding to low T1 signal areas within the tumor • – greater enhancement has been associated with poorer prognosis SWI/GE: variable intralesional hemorrhage, suggested by the presence of blooming artefact Unenhanced abdominal-pelvic CT image shows a large presacral mass with areas of internal calcifi caton (solid arrow) and sacral erosion (dotted arrow) Sagittal reformatted CT image shows the mass arising from the sacrum, with large presacral (solid arrow) and smaller postsacral (dotted arrow) soft-tssue components A 60-year-old white man chronic lower back pain A 35 y/o woman complained of pelvic pain for months, associated with constpaton and urinary frequency (a) Coronal T1-weighted: a large, predominantly lowsignal-intensity presacral mass that contains several areas of higher signal intensity (arrows) (b) Coronal T2weighted shows markedly hyperintense signal within the mass, which has a lobular growth pattern Lowsignal-intensity areas of retculaton and lobulaton (arrows) and punctate foci of low signal intensity also are seen (c) Sagittal T2-weighted fat-saturated foci of abnormal signal intensity (arrowhead) within the marrow of several sacral segments, including S4, S5, and the coccyx These fi ndings, alongside the large anterior (solid arrow) and smaller posterior (dotted arrow) soft-tssue components of the mass, are suggestve of its osseous origin (d) Sagittal contrastenhanced T1-weighted fat-saturated mild heterogeneous enhancement within the mass Age: 25 years Gender: Female Constpaton and painful sacral mass heterogeneous signal intensites are noted, mainly T1 hypo, T2 hyperintense signal intermingled with T1 hyper and T2 hypointense foci  the lesion is expanding the right sacral foramina with anterior extension into the pelvic cavity displacing the uterus and the rectum postcontrast heterogeneous enhancement  Treatment and prognosis • • • surgical resecton: first line of treatment, radiotherapy offered for recurrent cases Metastatc spread of chordomas is observed in 7-14% of patents and includes nodal, pulmonary, bone, cerebral or abdominal visceral involvement, predominantly from massive tumors Prognosis is typically poor due to the locally aggressive nature of these tumors, with overall 10-year survival of approximately 40% • Metastatc is seen more frequently in sacral chordomas than in skull base chordomas Differential diagnosis • giant cell tumor, chondrosarcoma, myxopapillary ependymoma, and metastasis • • • • Giant cell tumor is the second most common primary sacral neoplasm after chordoma involve the upper sacrum and can be eccentric and extend across the sacroiliac joint low-to-intermediate T2 signal intensity occasionally demonstrate fluid-fluid levels Expansile, lobulated mass centered in the right sacral ala effacing the right S1 neuroforamen The mass has enhancing septatons, as well as other areas of nonenhancing T1 hyperintensity consistent with internal hemorrhage STIR sequence shows these to be multple cystc areas containing fluid-fluid levels Findings suggest an aneurysmal bone cyst • • Chondrosarcomas arise off midline, from the sacroiliac joint space cartlage, and display heterogeneous signal intensity on T2-weighted MR images because they consist of both mineralized and nonmineralized chondroid matrix Chondrosarcomas typically not contain areas of hemorrhage, a fact that helps differentate them from chordomas • Myxopapillary ependymomas, which also cause sacral destructon, may have imaging characteristcs very similar to those of chordomas; however, myxopapillary ependymomas arise in the spinal canal rather than bone, and they may demonstrate more intense gadolinium-based contrast enhancement than chordomas Conclusion Chordoma: • • • 50–60% occur in the sacrococcygeal region • • • large destructve osteolytc lesion with extraosseous extension • the most common primary tumour of the sacrum Usually arises from the third, fourth or fifth sacral vertebra in the midline or paramedian locaton CT: Internal calcificatons, centrally located MRI: hypointense or isointense on T1 weighted images and hyperintense on T2 weighted images The septatons, which divide the gelatnous components of this tumour, are hypointense on T2 weighted images Differental diagnosis: giant cell tumor, chondrosarcoma, metastases ...Sacrococcygeal Chordoma • Chordomas are uncommon malignant tumors of the axial skeleton that account for 1% of intracranial... presentaton Chordoma is the most common primary malignant sacral tumor spheno-occipital: 30-35% vertebral body: 15-30% Epidemiology • • • Usually seen in adults (30-70 years) • Rarely, chordomas... sacrococcygeal chordomas are typically seen in a slightly older age group (peak around 50 years Clinical presentation • • • • • They are slow-growing tumors: palpable mass (e.g sacrococcygeal chordoma)