Spinal hemangioblastomas are the third most common intramedullary spinal neoplasm, representing 2-6% of all intramedullary tumors 1,4,7.
This article specifically relates to spinal hemangioblastomas. For a discussion on intracranial hemangioblastomas and a general discussion of the pathology refer to the main article: hemangioblastoma.
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Epidemiology
Hemangioblastomas are mostly sporadic (two-thirds) with a peak presentation in the fourth decade. Sporadic hemangioblastomas only rarely occur in children. Males and females are affected equally 4,7.
Associations
One-third of patients with hemangioblastomas (not just spinal) have von Hippel-Lindau syndrome 6,7 and typically these patients present earlier with multiple tumors.
Clinical presentation
Clinical presentation is similar to that of other intramedullary spinal tumors, with pain, weakness and sensory changes common. Rarely, spinal hemangioblastomas may cause subarachnoid hemorrhage or hematomyelia 4.
Pathology
Hemangioblastomas are benign vascular lesions that do not undergo malignant degeneration. They are World Health Organization (WHO) grade 1 in the 5th Edition (2021) of the WHO classification of CNS tumors 9.
Histologically, they consist of large pale stromal cells packed between blood vessels.
Radiographic features
The most common location is the thoracic cord (50%), followed by the cervical cord (40%) 4,7. The majority of hemangioblastomas have an intramedullary component with two-thirds located eccentrically and having an exophytic component (most commonly along the dorsum of the cord 8). Only 25% are entirely intramedullary. A minority appears entirely extramedullary and only rarely are they extradural 2,7.
Eighty percent of hemangioblastomas are solitary. If multiple lesions are present, von Hippel-Lindau syndrome should be suspected 4.
Angiography (DSA)
A densely enhancing nidus with associated dilated arteries and prominent draining veins is characteristic of a hemangioblastoma.
CT
On non-contrast CT they may be seen as a soft tissue nodule often with a prominent hypodense cyst-like component. Contrast administration results in vivid enhancement of the solid component.
MRI
Although they usually appear as discrete nodules, there can be diffuse cord expansion. An associated tumor cyst or syrinx is common (50-100%) 2,4. Additionally, they may rarely be a source of subarachnoid hemorrhage or hematomyelia 4,7.
Reported signal characteristics of the solid components include 4,7:
-
T1
variable relative to the normal spinal cord
hypo- to isointense most common, and difficult to identify
hyperintense (25%)
-
T2
iso- to hyperintense
focal flow voids especially in larger lesions
surrounding edema and associated syrinx are usually seen
hemosiderin capping may be present
-
T1 C+ (Gd)
the tumor nodule enhances vividly
Care should be taken to image the whole neuraxis to ensure that no other lesions are present.
Treatment and prognosis
Hemangioblastomas are slow-growing. They are usually treated by surgical resection, sometimes with preceding endovascular embolization to reduce intraoperative blood loss.
Differential diagnosis
The differential diagnosis can be thought of in two groups on account of the principal features of these tumors: neoplasms of the spinal canal (enhancing component) and vascular malformations of the spinal cord (enlarged vessels)
Other enhancing masses to be considered include:
Vascular malformation to consider include:
other hypervascular cord neoplasms
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