Answer for BIR CoW 14 Aug 2022
CHORDOMA
Findings
Lobulated T2 / hyperintensity lesion noted arising from basisphenoid segment extending upto the prevertebral region upto level of superior end plate of C3 and lesions with retroclival extension.
Laterally lesion seen extending upto bilateral petrous apex, displacing bilateral internal carotid artery laterally.
Anteriorly lesion seen displacing soft palate.
Evidence of patchy diffusion restriction noted within the lesions. Evidence of foci of blooming noted within the lesion.
The lesion measures 2.9 (antero-posterior) x 7.5 (transverse) x 5.6 (craniocaudal) cm.
Features suggestive of clival chordoma with nasopharyngeal and retroclival extension
Discussion
Clival chordoma represent the second commonest site of chordoma (sacrococcygeal location being the first location). Chordomas are uncommon malignant tumors that account for 1% of intracranial tumors and 4% of all primary bone tumors.
They originate from embryonic remnants of the primitive notochord (earliest fetal axial skeleton, extending from the Rathke's pouch to the coccyx). Since chordomas arise in bone, they are usually extradural and result in local bone destruction. They are locally aggressive, but uncommonly metastasize.
Treatment for intracranial chordomas is discouraging because operative mortality is high.
Radiation therapy is the most commonly employed treatment for clival and vertebral body lesions.
The prognosis for intracranial and vertebral lesions is poor, with most patients dying within 3 years of the initial diagnosis.
Findings
Lobulated T2 / hyperintensity lesion noted arising from basisphenoid segment extending upto the prevertebral region upto level of superior end plate of C3 and lesions with retroclival extension.
Laterally lesion seen extending upto bilateral petrous apex, displacing bilateral internal carotid artery laterally.
Anteriorly lesion seen displacing soft palate.
Evidence of patchy diffusion restriction noted within the lesions. Evidence of foci of blooming noted within the lesion.
The lesion measures 2.9 (antero-posterior) x 7.5 (transverse) x 5.6 (craniocaudal) cm.
Features suggestive of clival chordoma with nasopharyngeal and retroclival extension
Discussion
Clival chordoma represent the second commonest site of chordoma (sacrococcygeal location being the first location). Chordomas are uncommon malignant tumors that account for 1% of intracranial tumors and 4% of all primary bone tumors.
They originate from embryonic remnants of the primitive notochord (earliest fetal axial skeleton, extending from the Rathke's pouch to the coccyx). Since chordomas arise in bone, they are usually extradural and result in local bone destruction. They are locally aggressive, but uncommonly metastasize.
Treatment for intracranial chordomas is discouraging because operative mortality is high.
Radiation therapy is the most commonly employed treatment for clival and vertebral body lesions.
The prognosis for intracranial and vertebral lesions is poor, with most patients dying within 3 years of the initial diagnosis.
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!