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36- year- old male with bilateral arm weakness.
Dx= Cervical hemangioblastoma.
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  • On sagittal T1W1 ( top row), there is widening of the cord from C2 to T2 by an irregular, “cystic” septated intramedullary lesion that is hypointense to cord and slightly hyperintense to CSF, that extends from the cervicomedullary cord to at least T3. On T2W1 (bottom row), this intramedullary process becomes hyper intense to cord, isointense to CSF.
  • Post- gadolinium axial and sagittal T1-weighted images show three enhancing nodules at the posterior margin of the cord. The largest is at C6-7 and the two smaller are seen on the right at C7-T1 and left signal void foci on all imaging sequences, particularly within the dorsal aspect of the canal from C3 to C6.

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  • Hemangioblastomas account for 2% of all primary cord tumors. Most occur in the dorsal portion of the cord, with 60% intramedullary and 40% intradular/ extramedullary. They are frequently solitary 80%, with the most common location thoracic 50%, with 40% occurring in the cervical region. These tumors manifest in the fourth decade and 1/3 of patients have von Hippel-Lindau disease. They often present as intramedullary cysts with one or more vascular nodules and dilated, tortuous veins usually on the dorsal surface of the cord seen on angiography. About 40% of all hemangioblastomas and 60% of intramedullary hemangioblastomas are associated with a syrinx.
  • MR findings typically show an enlarged, sometimes irregular widening of the cord that is predominately hypointense on T1W1 and become hyperintense on T2W1. This is due to the cystic component, which is also indistinguishable from the surrounding edema on T2W1. The tumor nidus may sometimes be seen as isointense (to cord), nodular on T1- weighted sequence within the intramedullary or intradular cystic cavity, but becoming hyperintense on T2W1 (usually indistinguishable from cystic cavity or surrounding edema). An associated syrnix maybe quite extensive, extending over multiple levels above and below the tumor. Reports have shown that the tumor nidus rapidly and intensely enhances on T1W1 following the administration of gadopentetate dimeglumine, helping to demarcate from the surrounding edema, thus providing valuable preoperative localization.
    Additionally, focal areas of signal void on both T1 and T2W1 may be seen, depicting the flow in dilated feeding/ draining vessels. Initial experience suggests that a densely enhancing solid tumor nodule within a large “syrinx” cavity and associated “feeding” vessels is highly suggestive, if not diagnostic, of a hemangioblastoma.
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