Spinal and cerebellar vermis hemangioblastomas - vHL

Case contributed by RMH Neuropathology

Patient Data

Age: 18
Gender: Male

At the dorsal surface of the cord at C2/3 level there is an ovoid enhancing nodule measuring 2 x 4 x 4.5 mm. This is associated with T2 hyperintensity of the cervical cord extending from C1/2 to C5, and mild prominence of the central canal of the spinal cord posterior to the C6 vertebral body.

No further sites of abnormal contrast enhancement. Remainder of the cord (thoracic and lumbar spine - not shown) has normal signal.

Caliber of the spinal canal is capacious throughout. No high-grade foraminal stenosis.

Mild reversal of the normal cervical lordosis. Alignment is otherwise normal. Normal vertebral body heights and marrow signal.

There are stable size and configuration of the enhancing nodule in the medial aspect of the left cerebellar hemisphere within the left side of the cerebellar vermis adjacent to but not at the roof of the fourth ventricle.

The lesion is FLAIR hyperintense, T1 hypointense and measures 6 x 6 x 6 mm, unchanged compared with the previous exam. There is no other area of focal enhancement in the brain. No other area of signal change in the parenchyma.

Histology (spinal lesion)

Pathology

The patient underwent to a surgery resection of the spinal lesion. 

MACROSCOPIC DESCRIPTION:

One ovoid piece of dark brown tissue 6mm. 

MICROSCOPIC DESCRIPTION:

Sections show a moderately cellular, highly vascular tumor comprising small nests of cells with vacuolated cytoplasm, nuclei with smooth contours and even chromatin and absent nucleoli.  Scattered cells with large mildly hyperchromatic nuclei are present. There are abundant thin-walled small caliber vessels. Mitoses and necrosis are not identified. The vacuolated cells stain with inhibin and are AE1/3 and CD10 negative.

DIAGNOSIS:

Spinal tumor: Capillary hemangioblastoma (WHO I).

Case Discussion

The MRI findings of enhancing nodules in the dorsal aspect of the cord at C2/3 level and in the cerebellar vermis are consistent with hemangioblastomas in a patient with von Hippel Lindau syndrome

 

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