Age / Sex : 49 / F

Chief complaint : buttock pain with lower extremity tingling sense


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Courtesy : 이영준,  Eugene Lee, 분당서울대학교병원, Seoul National University Bundang Hospital

Hemangioblastoma

Discussion

Findings:

 About 3.2x1.6x1.7cm sized well enhaing IDEM tumor at L1-2 level.

   - prominent tortuous vessel structures & peritumoral cyst or syrinx, cranially.

 Tumor staining through right L1 segmental artery on angiography was confirmed

Differential Diagnosis:

1. myxopapillary ependymona

2. paraganglioma

Discussion:

 Hemangioblastomas are low-grade, highly vascular tumors. Hemangioblastomas represent 1%-3% of all central nervous system tumors, most often occurring in the cerebellum. It is unusual for hemangioblastomas to be located in the spine, representing an incidence of 1%-5% of all spinal cord tumors. It is even more uncommon to see an intradural extramedullary (IDEM) location of this tumor, especially lumbar spine. Most of them are seen in intramedullary location affecting the cervical or thoracic spine. Many spinal hemangioblastomas are associated with von Hippel-Lindau (VHL) syndrome. To have an isolated IDEM hemangioblastoma of the cauda equina without VHL syndrome is rare; so far, only about 20 cases have been reported. This tumor’s radiologic features are confusing with other hypervascular tumors in the lower lumbar spine, especially not associated with VHL syndrome.

Paragangliomas and myxopapillary ependymomas show similar imaging findings as hemangioblastomas. Paragangliomas are usually isointense to the conus medullaris on T1-weighted sequences, but can be hypointense as well. On T2-weighted sequences, it is hyperintense and sometimes heterogeneous due to subacute blood clot. Serpentine flow void is a frequent feature attributed to the hypervascularity of the lesion or to the congested veins, which are compressed by the mass. Hypointense margins are usually seen on T2 or proton density (PD)-weighted images they are suggestive of an intradural well-encapsulated tumor and are attributed to the paramagnetic effect of hemosiderin or ferritin from previous hemorrhages.

Myxopapillary ependymomas tended to be hypointense to isointense compared with normal spinal cord signal intensity on T1WI and hyperintense on PD- and T2WI. Signal may also be affected by blood. Tortuous vascular structures are commonly seen in thee tumors.

Paragangliomas and myxopapillary ependymomas in the region of filum terminale or cauda equine have imaging characteristics very similar to hemangioblastomas. Similar MR imaging features of those tumors make it challenging to distinguish hemangioblastomas from paragangliomas or myxopapillary ependymomas.

References:

1. Blaty D, Malos M, Palmrose T, McGirr S. Sporadic Intradural Extramedullary Hemangioblastoma of the Cauda Equina: Case Report and Literature Review. World Neurosurg 2018;109:436-441


2. Baker KB, Moran CJ, Wippold FJ, 2nd, Smirniotopoulos JG, Rodriguez FJ, Meyers SP, et al. MR imaging of spinal hemangioblastoma. AJR Am J Roentgenol 2000;174:377-382


3. Miliaras GC, Kyritsis AP, Polyzoidis KS. Cauda equina paraganglioma: a review. J Neurooncol 2003;65:177-190


4. Wippold FJ, 2nd, Smirniotopoulos JG, Moran CJ, Suojanen JN, Vollmer DG. MR imaging of myxopapillary ependymoma: findings and value to determine extent of tumor and its relation to intraspinal structures. AJR Am J Roentgenol 1995;165:1263-1267


5. Conway JE, Chou D, Clatterbuck RE, Brem H, Long DM, Rigamonti D. Hemangioblastomas of the central nervous system in von Hippel-Lindau syndrome and sporadic disease. Neurosurgery 2001;48:55-62; discussion 62-53