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Year : 2020  |  Volume : 3  |  Issue : 2  |  Page : 27-31

Von Hippel–Lindau disease with renal cell carcinoma and multiple cerebellar and spinal hemangioblastomas without retinal manifestations: A case report

1 Department of Histopathology, Apollo Hospitals, Chennai, Tamil Nadu, India
2 Department of Neurosurgery, Apollo Hospitals, Chennai, Tamil Nadu, India
3 Department of Ophthalmology, Apollo Hospitals, Chennai, Tamil Nadu, India

Correspondence Address:
Swetha L Narla
Department of Histopathology, Apollo Cancer Centre, 320 Anna Salai, D Block, Chennai, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JHNP.JHNP_12_21

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A 35-year-old gentleman presented with headache in the occipital region, neck pain, slurring of speech, and vomiting. There was reported history of Von Hippel–Lindau disease (VHL) in his mother, who was operated for a cerebellar hemangioblastoma. On investigations, magnetic resonance imaging brain showed bilateral cerebellar hemangioblastomas and a tiny spinal hemangioblastoma. Contrast-enhanced computed tomography abdomen showed multiple small well-defined, non-enhancing hypodense lesions with fluid attenuation in the left kidney and pancreas, suggestive of simple cysts. There was history of right partial nephrectomy in the past for renal cell carcinoma. Complete excision of bilateral cerebellar hemangioblastomas was performed. Histopathological examination and immunohistochemical workup confirmed the clinical diagnosis of hemangioblastoma.

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