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Dandy-Walker Cyst with Occipital Meningocele
Joseph Junewick, MD FACR
over 7 years ago
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Hemangioblastoma

Case Detail

Anatomy: Brain-Spine
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Neoplasia Malignant
Created: over 7 years ago
Updated: over 7 years ago
Tags: PEDS
Modality/Study Types: MR
Activities:
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History

Teenage male with vonHippel Lindau disease.


Case Images


Diagnosis

Hemangioblastoma

Clinical Notes

Previous thoracic hemangioblastoma.

Findings

MR – Sagittal T1, T2 and postgadolinium T1 and axial T2 and postgadolinium T1 images of the cervical spine demonstrate two enhancing nodules (C3 and C5-C6) with intramedullary edema and cyst.

Discussion

von Hippel-Lindau is a multisystem genetic disease (autosomal dominant with high penetrance and variable expressivity) resulting in inactivation of tumor suppressor gene on chromosome 3 (p25.5). Manifestations include CNS hemangioblastomas, renal cysts, retinal hemangiomas, renal carcinoma, pheochromocytoma, epididymal cystadenomas, and pancreatic cysts.

About 1/3 of patients with spinal hemangioblastomas (sHbl) have von Hippel-Lindau disease; multiple hemangioblastomas are virtually pathognomotic of von Hippel-Lindau disease. The mean age of diagnosis of sHbl is 30 years. Symptoms of sHbl include sensory changes, motor deficits and pain.

Hemangioblastomas are highly vascular tumors and may be associated with subarachnoid or intramedullary hemorrhage. Most sHbl occur in the thoracic or cervical spinal cord but may originate anywhere within the spinal canal or vertebral body. Of the intramedullary sHbl, 66% originate near the surface, 25% are completely intramedullary and 8% are exophytic. Intramedullary sHbl are associated with syrinx in 55% and cord swelling in 23%.

On MRI, sHbl are hypointense or isointense on T1, hyperintense on T2, densely enhancing and often associated with flow voids.

Reference

Baker, KB et al. MR Imaging of Spinal Hemangioblastoma. AJR 2000; 174: 377-382.

Chu BC, et al. MR Findings in Spinal Hemangioblastoma: Correlation with Symptoms and Surgical Findings. AJNR 2001; 22:206-217.

Leung RS, et al. Imaging Features of von Hipple-Lindau Disease. Radiographics 2008; 28:65-79.



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