Spinal hemangioblastoma

Case contributed by Dr Jeffrey Hocking

Presentation

Progressive upper limb weakness

Patient Data

Age: 50 years
Gender: Male
CT

A solitary 8.5 x 10 x 14mm (AP x LR x CC) marginally hyperattenuative well circumscribed lesion in the dorsal aspect of the spinal canal at the level of C2. Within the limits of this examination, the lesion appears to be intramedullary, causing slight anterior displacement of the spinal cord at this level. No hydrocephalus. Well circumscribed CSF density region superior to the lesion, within the spinal canal may reflect a prominent syrinx.

MRI

There is a 13 mm lesion demonstrated at the dorsal aspect of the cervical spinal cord at the T2 vertebral body level contains multiple internal flow voids with feeding vessels arising from the left VA demonstrated internally (best seen on ToF). The lesion is perimedullary with possible intramedullary extension and there is peripheral enhancement of a multiloculated large tumoral cyst extending through the central canal caudally from the lesion to the C5/C6 intervertebral disc level.
Cranial syringomyelic distension involving the obex and extending into the dorsal aspect of the medulla is likely to be secondary to distal obstruction.
There is also abnormal intramedullary spinal cord signal extending caudally from the inferior aspect of the tumoral cyst to approximately T1.

Fluoroscopy

DSA confirms the presence of a 14 mm vascular tumor receiving arterial supply from a single hypertrophied muscular branch of the left V3/V4 junction arising at the level of the dural entry. This follows a tortuous course and is directed caudally. There is no definite arteriovenous shunt. A single draining vein, directed cranially, is also tortuous traveling around the left dorsal CSF space to drain into the left IPS. The left PICA is remote to the lesion throughout its length.

Case Discussion

MICROSCOPIC
Spinal cord tumor: Sections show a circumscribed tumor composed of vascular
channels of varying sizes and thick-walled blood vessels separated by sheets
of lipidised stromal cells with focal microcystic pattern. These cells show
degenerative changes including nuclear pleomorphism and vacuolation.  There is
no mitosis or necrosis identified and there is no spinal cord tissue included.
The features are in keeping with a capillary hemangioblastoma.

Immunohistochemistry
Inhibin: Patchy positive
EMA: Focal, weak positive in single cells
CK7, Pax8: Negative

SUMMARY
Spinal cord tumor: Hemangioblastoma, WHO grade I.
 

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Case information

rID: 74110
Published: 5th Feb 2020
Last edited: 26th May 2020
Inclusion in quiz mode: Included