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Case Reports
. 2016 Jul-Sep;11(3):311.
doi: 10.4103/1793-5482.179644.

Cerebellospinal hemangioblastoma with bilateral pheochromocytoma and hepatic cyst: A rare entity

Mrinal Bhuyan  1 , Deep Dutta  1 , Basanta Kumar Baishya  1 , Zakir Hussain  1
Affiliations
Case Reports

Cerebellospinal hemangioblastoma with bilateral pheochromocytoma and hepatic cyst: A rare entity

Mrinal Bhuyan et al. Asian J Neurosurg. 2016 Jul-Sep.

Abstract

A 45-year-old gentleman presented with headache, dizziness, and unsteadiness of gait. On imaging, he was found to have a cerebellar and a spinal intramedullary hemangioblastoma. To rule out Von Hippel-Lindau (VHL) disease, we did screening of the whole abdomen with ultrasonography followed by contrast-enhanced computed tomography of the abdomen, and surprisingly, the patient was having bilateral adrenal pheochromocytoma and a hepatic cyst. A diagnosis of VHL disease was made on this basis. The patient was operated and he made a very good recovery. We are reporting this interesting case because of its typical findings and its rarity. Disease management and review of literature were also discussed at the end.

Keywords: Cerebellospinal; Von Hippel–Lindau disease; hemangioblastoma.

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Figures

Figure 1
Figure 1
(a) T1-weighted sagittal image (b) T2-weighted axial image (c) contrast axial image of brain magnetic resonance imaging revealing a large and well-defined cystic intraaxial mass lesion predominantly in the cerebellar vermis extending to the right cerebellar hemisphere along with vascular voids, suggesting cerebellar hemangioblastoma
Figure 2
Figure 2
(a) T1-weighted sagittal image (b) T2-weighted sagittal image (c) contrast sagittal image (d) contrast axial image of magnetic resonance imaging of spine revealed an intramedullary mass lesion, involving the cervico-dorsal cord, extending from C6 to T2 vertebral body level, causing cord expansion. The lesion showed peripheral enhancement with a nodular enhancing component showing the presence of intralesional cystic areas, suggestive of hemangioblastoma
Figure 3
Figure 3
(a) Contrast-enhanced computed tomography of abdomen (b) contrast-enhanced computed tomography of abdomen showing a hepatic hemangioma (c) contrast-enhanced computed tomography of the whole abdomen revealed bilateral pheochromocytoma (d) contrast-enhanced computed tomography of the whole abdomen revealed bilateral pheochromocytoma and a hepatic hemangioma in segment VII
Figure 4
Figure 4
(a) Operative photograph showing the cystic cavity (b) the mural nodule under the microscope
Figure 5
Figure 5
(a) Postoperative picture showing complete excision of the mass (b) histopathological picture showing clusters of stromal cells separated by multiple vascular channels consistent with hemangioblastoma
See this image and copyright information in PMC

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