Hemangioblastoma¶

Definition¶

Hemangioblastoma is a benign, highly vascular tumor that can occur as a sporadic lesion or in association with von Hippel-Lindau (VHL) disease. It is the third most common intramedullary spinal cord tumor after ependymoma and astrocytoma. In VHL disease, hemangioblastomas are often multiple and may occur throughout the craniospinal axis.

Imaging Findings¶

MRI¶

Location — Intramedullary (most common) or less commonly on the surface of the cord (exophytic) or on nerve roots. Most common in the cervical and thoracic cord.
T1-weighted — Well-defined, isointense to slightly hypointense nodule
T2-weighted — Hyperintense nodule, often with prominent flow voids around the tumor from enlarged feeding and draining vessels
Enhancement — Intense, homogeneous enhancement of the solid nodule (the "mural nodule")
Cyst with mural nodule — A classic pattern: a large T2-bright syrinx or cyst with a small, intensely enhancing nodule at one end. The cyst wall does not enhance (reactive, non-neoplastic). Only the nodule is tumor.
Flow voids — Prominent serpentine flow voids on T2 images represent enlarged vessels on the cord surface (pial arteriovenous shunting)

Spinal Angiography¶

Intensely vascular tumor with enlarged feeding arteries and early draining veins
May be used for preoperative embolization of large tumors

Clinical Pearl

The combination of an intensely enhancing intramedullary nodule with prominent flow voids and an associated syrinx/cyst is characteristic of hemangioblastoma. If the patient has multiple hemangioblastomas (spinal and/or cerebellar), always evaluate for VHL disease — which includes renal cell carcinoma, pheochromocytoma, and retinal angiomas. VHL accounts for approximately 30% of spinal hemangioblastomas.

von Hippel-Lindau Disease¶

Autosomal dominant disorder (VHL gene, chromosome 3p25)
Multiple hemangioblastomas (cerebellum, spinal cord, retina)
Renal cell carcinoma, pheochromocytoma, pancreatic cysts/tumors
Screening with serial MRI is essential for VHL patients

Management¶

Surgical excision of the enhancing nodule (the cyst resolves after nodule removal)
Preoperative embolization for large, highly vascular tumors
Stereotactic radiosurgery for surgically inaccessible or residual lesions
VHL patients require lifelong surveillance

Key Points¶

Third most common intramedullary tumor
Intensely enhancing nodule with flow voids and associated cyst/syrinx is characteristic
Only the nodule is tumor — the cyst wall is reactive
~30% are associated with VHL disease
Multiple hemangioblastomas mandate screening for VHL
Surgical excision of the nodule is curative

References¶

Shih RY, Koeller KK. Intramedullary Masses of the Spinal Cord: Radiologic-Pathologic Correlation. Radiographics. 2020;40(4):1125-1145.
Chu BC, Terae S, Hida K, Furukawa M, Abe S, Miyasaka K. MR findings in spinal hemangioblastoma: correlation with symptoms and with angiographic and surgical findings. AJNR Am J Neuroradiol. 2001;22(1):206-217.
Zhang Z, Low SW, Sun I, Lim SL, Tan CL, Goh CP. Spinal Hemangioblastoma: The Role of Imaging Characteristics in Preoperative Diagnosis and Surgical Planning. Cureus. 2025;17(4):e82740.
Khan MM, Hall WA, Belkhair S. Hemangioblastoma. In: StatPearls. Treasure Island (FL): StatPearls Publishing; 2025.
Hoff DJ, Tampieri D, Just N. Imaging of spinal cord hemangioblastomas. Can Assoc Radiol J. 1993;44(5):377-383.
Spinal haemangioblastoma. Radiopaedia.org. Available from: https://radiopaedia.org/articles/spinal-haemangioblastoma-1