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CT scan of the Brain was done for a 60-year-old man with TIA.

Plain CT (non-contrast enhanced or NECT) images showed a well-defined cyst in the right cerebellar hemisphere with thin, imperceptible capsule and a small mural nodule medially in the inferior pole that was isoattenuating to the adjacent normal cerebellar cortex (second image below). No calcification was seen.

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Contrast enhanced axial and multiplanar reformatted CT images showed intense enhancement in the eccentric mural nodule. No capsular enhancement was seen in other areas of the cyst.

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MIP images showed an arterial feeder to the mural nodule arising from the right Anterior Inferior Cerebellar artery.

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Diagnosis: Hemangioblastoma.

Hemangioblastomas are considered benign and represent 1-2% of all primary tumours of the central nervous system. Most hemangioblastomas are located in the posterior cranial fossa; in that region, hemangioblastomas comprise 8-12% of neoplasms. Hemangioblastoma is the most common primary adult intraaxial posterior fossa tumor. Cerebellar hemangioblastomas are frequently referred to as Lindau tumours because Swedish pathologist Arvid Vilhelm Lindau first described them in 1926. The second most common location of hemangioblastomas is the spinal cord, where the frequency ranges from 2-3% of primary spinal cord neoplasms to 7-11% of spinal cord tumors. This tumor’s occurrence in other locations, such as the supratentorial compartment, the optic nerve, the peripheral nerves, or the soft tissues of extremities is extremely rare. Hemangioblastomas are more common in men than in women. In most clinical series, the male-to-female ratio is approximately 2:1. Although hemangioblastomas may develop at any age, they rarely affect children; the usual age at diagnosis is between the third and fifth decades.

Historically, hemangioblastomas are linked to von Hippel-Lindau (VHL) disease. Arvid Lindau first reported the connection between retinal angiomas and hemangiomas of the cerebellum. VHL is an autosomal dominant condition involving chromosome characterized by specific benign and malignant tumours with variable expressivity. Cerebellar hemangioblastoma is the most common initial manifestation, affecting 64% of patients with VHL. In many series, cerebellar hemangioblastoma is the most important cause of mortality, affecting 47.7% of patients with VHL, followed by renal cell carcinoma. In patients with a positive family history, a single cerebellar hemangioblastoma is sufficient to make the diagnosis. If no known family history exists, at least 2 cerebellar hemangioblastomas or 1 hemangioblastoma plus 1 visceral tumor are necessary to justify the diagnosis of VHL. Various other organs can be involved. Of patients presenting with hemangioblastomas, 70% do not have a family history, and 3-25% of these patients have tumors associated with VHL. Hemangioblastoma appears to be associated more with VHL than previously reported, and it has been suggested that all patients with sporadic nonhereditary tumors should be evaluated for evidence of VHL disease. [from suggested reading 1 & 2 below]

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Suggested Reading:

1. Hemangioblastoma, Brain: Imaging - eMedicine article [Free registration required]
2. Hemangioblastoma - eMedicine Neurosurgery article [Free registration required]
3. Hemangioblastoma - CHORUS article
4. MedPix case.
5. NewYork-Presyterian Hospital’s patient information article.

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