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

Cerebellospinal Hemangioblastoma With Bilateral Pheochromocytoma and Hepatic Cyst: A Rare Entity

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Free PMC article
Case Reports

Cerebellospinal Hemangioblastoma With Bilateral Pheochromocytoma and Hepatic Cyst: A Rare Entity

Mrinal Bhuyan et al. Asian J Neurosurg. .
Free PMC article

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.

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

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