Chordoma U nguyên sống

involving the fourth and fifth sacral segments/ M:F rato 2:1, and the tumor partcularly large at presentaton.. Radiographic featuresCT • centrally located • well-circumscribed • destru

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. 

involving the fourth and fifth sacral

segments/ M:F rato 2:1, and the tumor

partcularly large at presentaton.

malignant sacral tumor

• Usually seen in adults (30-70 years)

• 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

• Rarely, chordomas are encountered in children with tuberous sclerosis

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

hemorrhage

• irregular intratumoral calcificatons (thought to represent sequestra of normal bone rather than dystrophic calcificatons)

• moderate to marked enhancement 

• MRI

• T1

• T2: most exhibit very high signal   

• T1 C+ (Gd)

signal areas within the tumor

• 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

chronic lower back pain

A 35 y/o woman complained of pelvic pain for 2 months, associated with

constpaton and urinary frequency.

(a) Coronal T1-weighted: a large, predominantly signal-intensity presacral mass that contains several areas of higher signal intensity (arrows) (b) Coronal T2- weighted shows markedly hyperintense signal within the mass, which has a lobular growth pattern Low- signal-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 contrast- enhanced T1-weighted fat-saturated mild heterogeneous enhancement within the mass

low-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

can be eccentric and extend across the sacroiliac joint

intensity

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.

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 do

Chordoma:

• 50–60% occur in the sacrococcygeal region

• the most common primary tumour of the sacrum

• Usually arises from the third, fourth or fifth sacral vertebra in the midline or paramedian locaton

• large destructve osteolytc lesion with extraosseous extension

• 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