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Neuroradiology Case of
the Week
Case 127
Osbert N. Adjei, MD, Ravinder Sidhu, MD,
and Per-Lennart Westesson, MD, PhD, DDS
Clinical
Presentation: A
56-year-old female with a left cerebellar mass.
Radiological
Findings: A left cerebellar mass with an intramural nodule and a cystic component is seen. The mural nodule is isointense to gray matter on both T1 and T2-weighted images (Figs. 1A&B), and shows intense enhancement on post contrast images (Figs.1C&D). There was significant increased blood volume on perfusion map images.
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Figure 1A |
Figure 1B |
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Figure 1C |
Figure 1D |
Diagnosis:
Cerebellar hemangioblastoma
Discussion: Hemangioblastoma is a pathologically benign lesion of the central nervous system. This rare tumor is most commonly seen in the cerebellum, however hemangioblastomas involving the spinal cord have been reported. Other locations, including the supratentorial compartment, optic nerve and peripheral nerves, are extremely rare. The disease is relatively rare in children, and is seen more commonly in adult males between the third and fifth decades
Most cases of hemangioblastomas are sporadic, however an association between Von Hippel Landau disease is seen in about 25% of cases. Hence in patients with hemangioblastomas, abdominal imaging in addition to a complete neural axis imaging is recommended by some to evaluate for additional lesions that may be associated with VHL syndrome.
The clinical presentation depends on the location. Intracranial lesions typically present with a long history of minor neurological symptoms, followed by an acute exacerbation that may necessitate emergent surgical intervention.
Diagnosis by imaging modality has improved with the introduction of MRI. MRI is superior to CT in the diagnosis of posterior fossa hemangioblastoma. This is due to the presence of beam hardening artifacts in the posterior fossa on CT.
The imaging findings mirror the gross features of the tumor. Hemangioblastomas are highly vascular tumors with two principal components i.e. capillaries and stromal cells. The tumor typically consists of an intramural solid nodule with a cystic component (about 60% of all tumors), Purely solid tumors are also seen, however purely cystic tumors are very rare.
On T1-weighted images the solid component presents as an isointense intramural nodule. On T2-weighted images, the lesion is isointense. There is usually uniform enhancement of the solid component on post-contrast images.
Surgery is the preferred treatment modality for the tumor. Surgery may be preceded by Gamma Knife Stereotatic Surgery or endovascular embolization to shrink the tumor or reduce its vascularity.
References:
- Sano T. Horiguchi H. Von Hippel-Lindau disease. Microsc Res Tech. 2003 Feb 1;60(2):159-64. [Medline]
- Georg AE, Lunsford LD, Kondziolka D, Flickinger JC, Maitz A. Hemangioblastoma of the posterior fossa. The role of multimodality treatment. Arq Neuropsiquiatr. 1997 Jun;55(2):278-86. [Medline]
- Sharma RR, Cast IP. O’Brien C. Supratentorial hemangioblastoma not associated with Von Hippel-Lindau complex or polycythemia: case report and review of the literature. Br J Neurosurg. 1995;9(1):81-4. [Medline]
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