Cerebellar Hemangioblastoma

Case history: A 26-year old presented to the outpatient department with the chief complaints of headache and vomiting for the past six months.

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Figure 1: Axial sequences depicting a heterogeneous midline cystic mass is in the infratentorial region with its epicenter in the cerebellar parenchyma. Multiple intensely enhancing mural nodules are seen within the lesion. Some of the nodules showed a few irregular cystic/necrotic regions within.

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Figure 2: Axial image taken at a lower section shows a well defined cystic cavity with clear fluid appearing hyperintese on T2W sequence.

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Figure 3: Diffusion weighted images show that the lesion largely shows faciliated diffusion with few foci of restricred diffusion.

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Figure 4: Spectroscopy shows elevate Ch/NAA ratio in the solid enhancing component of the lesion, especially in voxels 2 and 9.

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Figure 5: Perfusion sequences show that the lesion is hyperperfused as compared to the normal parenchyma.

Findings:  The patient underwent an MRI examination which showed a midline cystic mass is in the infratentorial region with its epicenter in the cerebellar parenchyma. The lesion was associated with multiple intensely enhancing mural nodules within it and a single similar nodule in the left cerebellar hemisphere adjacent to the lesion.

These lesions showed foci of bleeding within and around the lesion with multiple associated abnormal vessels. No peritumoral edema was seen. Some of the nodules showed a few irregular cystic/necrotic regions within it. The solid enhancing lesions were hyperperfused and showed restricted diffusion with elevated choline, reduced creatine and NAA with max choline/creatine ratio.

Features were suggestive of a hemangioblastoma. Orbits were unremarkable. Spine screening was shown which showed no additional lesions. Family history was abnormal. Patient underwent surgery for the solitary cerebellar lesion.

Diagnosis: Cerebellar hemangioblastoma

Discussion: Cerebellar hemangioblastoma is a benign neoplasm of uncertain origin that comprises approximately 7 percent of posterior fossa tumors in adults. It is the second most common neoplasm in the posterior fossa in adults second only to metastases.  It is associated with the autosomal dominant tumor-suppressor disorder von Hippel-Lindau (VHL).

Approximately 44 percent to 72 percent patients with VHL have a cerebellar hemangiobalstoma while 30 percent of hemangioblastomas can be attributed to VHL. The age at presentation is the fifth and sixth decades; however in VHL patients frequently at as young adults. Multiple hemangioblastomas are characteristic of VHL and constitute a major diagnostic criterion for the same.

MR is the imaging modality of choice. Characteristic MR criteria include cystic nature of the lesion, a peripheral pial-based mural nodule of solid tissue that enhances markedly with intravenous contrast, and large vessels within and/or at the periphery of the mass. On T1W images lesions appear isointense, while on T2W sequences the lesions appear hyperintense in keeping with their cystic nature. Flow voids are frequently noted either within or in the periphery of the lesion representing large feeding/draining vessels. The closest differential for hemangioblastoma is metastases, however high cerebral blood flow is characteristic of hemangioblastoma. Also perilesional edema is unusual in the former.                                    

Treatment primarily consists of surgical resection of symptomatic tumors.

Amar Udare, MD
Radiodiagnosis, Tata Memorial Hospital
Mumbai, India

References:

• Russell DS, Rubinstein LJ. Pathology of tumors of the nervous system, 5th ed. Baltimore: Williams & Wilkins, 1989
• Leung RS, Biswas SV, Duncan M, et al. Imaging features of von Hippel-Lindau disease. RadioGraphics 2008;28:65-79
• Atlas S. Magnetic Resonance Imaging of the Brain and Spine. 4th edition. Lippincott Williams & Wilkins. 2008