First AD

Second AD

A 34-year-old male presented with worsening numbness and increasing weakness in the right hand, progressing to involve the proximal right upper extremity. No family history of VHL. Outside brain MRI revealed a lesion in the cervicomedullary junction, further work-up with dedicated spinal MRI as shown.



There is an intramedullary mass arising in the posterior aspect of the spinal cord, associated with intraspinal cysts and the presence of a well-circumscribed peripheral, nodular enhancing soft tissue component along the dorsal subpial surface. There is some mild peritumoral spinal cord edema and some mild associated expansion/remodeling of the cervicomedullary junction.

This lesion was ultimately resected and surgical pathology revealed hemangioblastoma.




Spinal cord hemangioblastomas are low-grade, vascular neoplasms accounting for a small fraction of all spinal tumors (1.6% to 5.8%). Roughly two-thirds of cases occur sporadically and the remainder occurs in the setting of von Hippel-Lindau (VHL) disease. Once a lesion is found, the patient should undergo contrast-enhanced MRI of the entire neuroaxis (brain through whole spine) to exclude multiple hemangioblastomas, which raises greater suspicion for VHL.

Imaging features of hemangioblastoma can be variable and the lesion can be cystic, solid, hemorrhagic, or mixed in appearance. The lesions typically arise along the posterior aspect of the spinal cord along subpial surfaces and large lesions may demonstrate vascular flow-voids, manifested as serpiginous low signal intensity on T2-weighted images, reflecting enlarged feeding and draining vessels. The lesions may remodel/expand the spinal canal, as in the case shown here. T1-weighted images typically reveal a lesion iso-intense with the spinal cord, which is often difficult to distinguish. On T2-weighted images, the mass is usually hyperintense to CSF in a uniform fashion and there is often peritumoral edema.

A characteristic imaging feature is the presence of an enhancing mural nodule in a cystic lesion, as shown in this case. The solid component of hemangioblastomas typically has a well-circumscribed appearance with intense, homogenous enhancement, which can sometimes help differentiate them from astrocytomas, which tend to have more heterogeneous enhancement.
Patients may present with nonspecific sensory or motor neurologic symptoms. Most spinal lesions producing clinical symptoms have an associated syrinx. The typical age at presentation is a young adult in their 30s, but patients with family history of VHL may present earlier. The tumors grow slowly and do not undergo malignant transformation. 

Treatment options include microsurgical resection or stereotactic radioablation. Radiosurgical ablation has a greater role in patients with multiple lesions or inoperable lesions. Preoperative arterial embolization also has some role in the treatment approach.


1. Shah, LM. Diagnostic Imaging: Spine. Lippincott Williams & Wilkins Publishing; 2

  ed. 2010.

2. Baker KB et al. MR imaging of spinal hemangioblastoma. 

 2000;174:377-382.

3. Leung RS et al. Imaging features of von Hippel-Lindau disease

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