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Cerebellar hemangioblastoma-Is it always with Von Hippel Lindau syndrome?

A rare case with review of literature.
Introduction:
Hemangioblastoma (HB) is a vascular tumor of the CNS, accounting for only 1-2.5% of all intracranial tumorsĀ and approximately 10% of all posterior fossa tumorsĀ 3. It occurs most often in the cerebellum.1,3.
Haemangioblatomas occur both in patients with VonHippelLindau disease (VHL) as well as sporadically. About 45% of those with VHL develop HBs and 20% of those with HB have VHL3. Here we discuss a rare case of HB of brain associated with VHL in a young female.

Case Report
A 38 yr old female presented to our institute with history of headache, vomiting and gait abnormality for 2 months. She gave a history of brain tumor for which she underwent surgery three times since 2013.
Initially in May 2013 she had similar complaints and CT scan of brain showed heterogenous density (4.8 x 3.4 cm) in the left cerebellar hemisphere (LCH) causing obstruction of the 4th ventricle (FV) and dilatation of the supratentorial ventricular system (STVS) suggestive of posterior fossa space occupying lesion (SOL). On the MRI of brain the lesion was hyperintense on T2 WI, hypointense on T1 WI FLAIR with isointensity (1.4x1.2 cm) noted within plial surface (mural nodule) with perilesional edema.
She had a left sub occipital craniotomy and excision of the SOL. Cytology from the SOL (Squash) was suggestive of HB and histopathological evaluation (HPE) was suggestive of a vascular lesion.
Patient did not receive any Adjuvant treatment and the CT scan brain at 1 month follow-up showed post operative changes.
One year later patient was symptomatic and MRI brain showed a recurrent lesion (4.8x 4.2 cm) in the LCH. She underwent excision biopsy of the SOL. The HPE showed HB. Patient was treated symptomatically and kept on follow-up.
Four months later in September 2014 patient was symptomatic and on examination had bilaterally decreased visual acuity and papilledema. CT scan brain showed a 4.6x3.6 cm lesion posterior to the FV with compression of FV and dilatation of the STVS. Then patient had a right Ventriculo Peritoneal (VP) shunt placement. A Cranio Spinal Fluid analysis was also done which was within normal limits.
Patient was referred to our institute for further management of the recurrent Cerebellar HB in November 2014. When she presented to us, she was wheel chair bound accompanied by a family member. On examination she had intention tremor, horizontal nystagmus, ataxic gait and left hemiperesis.
Patient is a known type-2 diabetic and hypertensive on oral medications for 7 years. She has three children with last child born 17 years ago. Her mother had history of vision loss at the age of 65 years for unknown reason but no history of brain or kidney tumors in the family.
As patient was having HB of brain which is a rare tumor, we reviewed the literature and found the association of HB to VHL. We investigate the patient for retinal and other visceral lesions.
She underwent CT scan of abdomen and pelvis showing bilateral enlarged kidneys with bosselated contours, multiple cysts of varying sizes (largest 4 x 3.4 cm). Also multiple cysts were noted in the pancreas (largest 1.4 x 1.5 cm) suggestive of VHL.
FIGURE 1a:

Axial image of the CT scan abdomen and pelvis showing bilateral enlarged kidneys with multiple cysts.



FIGURE 1b:

Axial image of the CT scan abdomen and pelvis showing multiple cysts in the pancreas.


As VHL patients could have spinal lesions as well, patient had a CT scan and MRI of the spine along with MRI scan of the brain. There was a large hypervascular enhancing mass lesion (4.5x4.7x4.5cm) in the vermis and the LCH with significant perilesional edema extending into brainstem with mass effect over the FV s/o recurrence. Few well defined nodular lesions were noted in the right cerebellar hemisphere with minimal perilesional edema s/o small HBs.


FIGURE 2a:

Axial images of CT scan brain showing mass lesion in the vermis and the LCH with perilesional edema.



FIGURE 2b:

Coronal images of CT brain showing mass lesion in the vermis and the LCH.


Cervicomedullary junction showed a cystic mass lesion 2.4 x1.4 cm with mural nodule in the inferior aspect measuring 9x6 mm with significant compression over the cervicomedullary junction in the region of foramen magnum. Spinal cord from C2 to C5 showed cord edema. Multiple renal cortical cysts were noted in both the kidneys. Chest x-ray showed VP shunt and no active lung lesions.





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