Winner of CoW 04 September 2016
NEUROFIBROMATOSIS TYPE 1
Findings
This is a Whole Body MRI Coronal STIR (short T1 inversion recovery)Sequence. It shows: Multiple STIR hyperintense subcuatneous nodules. intradural lesion with extraforaminal extension in the lower cervical spine. Long segment STIR hyperintense lesions in both pelvis and thighs involving the sacral plexus and sciatic nerves and extending upto the knee level –consistent with Plexiform Neurofibromatosis.
Discussion
Neurofibromas can occur anywhere in the body in people with NF1, and at least 40% of affected adults have neurofibromas internally, although most are not apparent on physical examination Cutaneous neurofibromas develop from the terminal branches of peripheral nerves distal to the perineurial sheath.5Subcutanteous neurofibromas, in contrast, develop within a peripheral nerve and are circumscribed by the perineurium. internal plexiform neurofibromas, such as those of major spinal nerves or nerve roots, also develop intraneurally and carry a relatively high risk of malignant transformation. Hence regular followup of these individuals are essential.
Findings
This is a Whole Body MRI Coronal STIR (short T1 inversion recovery)Sequence. It shows: Multiple STIR hyperintense subcuatneous nodules. intradural lesion with extraforaminal extension in the lower cervical spine. Long segment STIR hyperintense lesions in both pelvis and thighs involving the sacral plexus and sciatic nerves and extending upto the knee level –consistent with Plexiform Neurofibromatosis.
Discussion
Neurofibromas can occur anywhere in the body in people with NF1, and at least 40% of affected adults have neurofibromas internally, although most are not apparent on physical examination Cutaneous neurofibromas develop from the terminal branches of peripheral nerves distal to the perineurial sheath.5Subcutanteous neurofibromas, in contrast, develop within a peripheral nerve and are circumscribed by the perineurium. internal plexiform neurofibromas, such as those of major spinal nerves or nerve roots, also develop intraneurally and carry a relatively high risk of malignant transformation. Hence regular followup of these individuals are essential.
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!