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Proximal nerve root spinal hemangioblastomas: presentation of three cases, MR appearance, and literature review.
Surg Neurol. 2004 Mar; 61(3):262-73; discussion 273.SN

Abstract

BACKGROUND

Hemangioblastomas (HBLs) are relatively uncommon tumors of uncertain histogenesis usually located in the cerebellum or spinal cord. Much less frequently they are identified in extramedullary locations including the filum terminale, proximal nerve roots, or even distal nerves of the peripheral nervous system (PNS). PNS cases not only present diagnostic challenges but also raise interesting questions regarding the common cell of origin for these CNS and PNS neoplasms. Few studies have detailed the neuroimaging characteristics of the rare extramedullary variants.

METHODS

Neuroimaging and intraoperative findings of three recent cases of proximal nerve root HBLs are described. The English language literature on extramedullary HBLs is reviewed and discussed, particularly in regards to magnetic resonance (MR) findings and association with von Hippel-Lindau Syndrome (VHL).

RESULTS

All 3 of our cases had prominent vessels present within the subarachnoid space on MR scans and all lesions enhanced. All were of intermediate or mildly decreased signal intensity on the T1-weighted images before contrast and were either iso- or hyperintense to spinal cord on the T2-weighted images. Two had probable cystic areas on MR, and all had cystic areas on histologic evaluation.

CONCLUSION

These neuroimaging characteristics can serve to distinguish HBLs from the more common benign nerve sheath tumors with which they are most frequently confused. Less than half of all extramedullary HBLs are diagnosed in patients with known VHL. It is important to consider HBLs in the differential diagnosis, as they are vascular and have the propensity for causing significant blood loss at surgery.

Authors+Show Affiliations

Department of Radiology, University of Colorado Health Sciences Center, Denver, Colorado, USA.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Review

Language

eng

PubMed ID

14984999

Citation

Escott, Edward J., et al. "Proximal Nerve Root Spinal Hemangioblastomas: Presentation of Three Cases, MR Appearance, and Literature Review." Surgical Neurology, vol. 61, no. 3, 2004, pp. 262-73; discussion 273.
Escott EJ, Kleinschmidt-DeMasters BK, Brega K, et al. Proximal nerve root spinal hemangioblastomas: presentation of three cases, MR appearance, and literature review. Surg Neurol. 2004;61(3):262-73; discussion 273.
Escott, E. J., Kleinschmidt-DeMasters, B. K., Brega, K., & Lillehei, K. O. (2004). Proximal nerve root spinal hemangioblastomas: presentation of three cases, MR appearance, and literature review. Surgical Neurology, 61(3), 262-73; discussion 273.
Escott EJ, et al. Proximal Nerve Root Spinal Hemangioblastomas: Presentation of Three Cases, MR Appearance, and Literature Review. Surg Neurol. 2004;61(3):262-73; discussion 273. PubMed PMID: 14984999.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Proximal nerve root spinal hemangioblastomas: presentation of three cases, MR appearance, and literature review. AU - Escott,Edward J, AU - Kleinschmidt-DeMasters,B K, AU - Brega,Kerry, AU - Lillehei,Kevin O, PY - 2002/12/23/received PY - 2003/03/06/accepted PY - 2004/2/27/pubmed PY - 2004/3/16/medline PY - 2004/2/27/entrez SP - 262-73; discussion 273 JF - Surgical neurology JO - Surg Neurol VL - 61 IS - 3 N2 - BACKGROUND: Hemangioblastomas (HBLs) are relatively uncommon tumors of uncertain histogenesis usually located in the cerebellum or spinal cord. Much less frequently they are identified in extramedullary locations including the filum terminale, proximal nerve roots, or even distal nerves of the peripheral nervous system (PNS). PNS cases not only present diagnostic challenges but also raise interesting questions regarding the common cell of origin for these CNS and PNS neoplasms. Few studies have detailed the neuroimaging characteristics of the rare extramedullary variants. METHODS: Neuroimaging and intraoperative findings of three recent cases of proximal nerve root HBLs are described. The English language literature on extramedullary HBLs is reviewed and discussed, particularly in regards to magnetic resonance (MR) findings and association with von Hippel-Lindau Syndrome (VHL). RESULTS: All 3 of our cases had prominent vessels present within the subarachnoid space on MR scans and all lesions enhanced. All were of intermediate or mildly decreased signal intensity on the T1-weighted images before contrast and were either iso- or hyperintense to spinal cord on the T2-weighted images. Two had probable cystic areas on MR, and all had cystic areas on histologic evaluation. CONCLUSION: These neuroimaging characteristics can serve to distinguish HBLs from the more common benign nerve sheath tumors with which they are most frequently confused. Less than half of all extramedullary HBLs are diagnosed in patients with known VHL. It is important to consider HBLs in the differential diagnosis, as they are vascular and have the propensity for causing significant blood loss at surgery. SN - 0090-3019 UR - https://www.unboundmedicine.com/medline/citation/14984999/Proximal_nerve_root_spinal_hemangioblastomas:_presentation_of_three_cases_MR_appearance_and_literature_review_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0090301903003999 DB - PRIME DP - Unbound Medicine ER -