MR findings in spinal hemangioblastoma: correlation with symptoms and with angiographic and surgical findings

Abstract

Background and purpose: To our knowledge, a detailed analysis of MR findings in spinal hemangioblastoma has not been conducted to date. Our purpose was to elucidate the MR features of this disease with special attention to tumor size, correlation with MR findings and clinical symptoms, and any differences between patients with and without von Hippel-Lindau disease (VHLD).

Methods: MR images in five patients with VHLD and seven patients without VHLD were reviewed retrospectively for spinal hemangioblastoma by two neuroradiologists. The MR findings were correlated with clinical symptoms and with angiographic and surgical findings.

Results: The MR features depended on the size of the spinal hemangioblastoma. Small (10 mm or less) hemangioblastomas were mostly isointense on T1-weighted images, hyperintense on T2-weighted images, and showed homogeneous enhancement. Larger hemangioblastomas tended to be hypointense or mixed hypo- and isointense on T1-weighted images, heterogeneous on T2-weighted images, and tended to show heterogeneous enhancement. Small hemangioblastomas were located at the surface of the spinal cord, most frequently along its posterior aspect. These were subpial in location at surgery and showed well-demarcated, intense enhancement. Symptomatic small hemangioblastomas had relatively large associated syringes, whereas asymptomatic ones did not. A hemangioblastoma larger than 24 mm was invariably accompanied by vascular flow voids. There was no difference in the MR findings between the two patient groups except for the multiplicity and higher percentage of small tumors in patients with VHLD.

Conclusion: Knowledge of these MR features helps to differentiate spinal hemangioblastoma from other diseases that show enhancing nodules.

fig 1.

Case 6: 30-year-old man without VHLD. A and B, Sagittal T1-weighted SE images (400/23/2) before (A) and after (B) intravenous administration of contrast material show a large, sausagelike, well-demarcated, intensely but heterogeneously enhancing tumor at T12–L1. Note superficial enhancement of the spinal cord (arrows, B), confirmed to be dilated perimedullary veins at surgery. Cephalic portion of the tumor resides within the spinal cord, and the caudal portion is extramedullary. A large intramedullary tumor with exophytic growth was confirmed at surgery. C, Sagittal T2-weighted FSE image (4000/96/3) shows mixed hyper- and isointense tumor. Cephalic to this tumor is syringomyelia up to C2 level (only partially shown). Note prominent vascular flow voids (arrows). D and E, Arterial (D) and venous (E) phases of digital subtraction angiograms, anteroposterior view, with right T10 intercostal artery catheterized, show intense tumor stain at T12–L1, dilated posterior spinal artery as a feeder (straight arrows, D), and dilated draining vein (curved arrows, E).

fig 2.

Case 3: 24-year-old woman with VHLD. A, Sagittal T1-weighted FSE image (700/10/6) shows a large, round, hypointense tumor in the left vertebral foramen between S1 and S2. Note vascular flow voids within the tumor. B, Sagittal T2-weighted FSE image (4100/121/5) shows mixed iso- and hyperintense tumor. C, Sagittal T2-weighted FSE image (4100/121/5), medial to slice in B, shows serpentine vascular flow voids (arrows). D, Contrast-enhanced axial T1-weighted FSE image (740/8.6/4) shows intensely but heterogeneously enhancing tumor that was confirmed to arise from left S1 nerve root at surgery. E and F, Arterial (E) and venous (F) phases of digital subtraction angiograms, right anterior oblique 30° view, with left internal iliac artery catheterized, show intense tumor stain, dilated lateral sacral arteries that supply the tumor (curved arrows, E), and early venous filling (straight arrows, F), indicating the arteriovenous shunt in the tumor. This vein corresponds with the flow voids seen in C.

fig 3.

Case 2: 42-year-old woman with VHLD without subjective symptoms. A and B, Contrast-enhanced sagittal T1-weighted FSE images (600/12/6) show nodular, intensely and homogeneously enhancing tumors at the posterior aspects of the spinal cord at levels C5, C6, T8–T9, and T9–T10. C, Sagittal T2-weighted TSE image (4500/112/3) shows a syrinx at T7–T8 and adjacent edema at T9–T10. This image is a composite of the upper and lower halves of the MR images of the spine. D, Contrast-enhanced axial T1-weighted TSE image (1000/12/2) shows the tumor surrounding a posterior nerve root at C6, as is also the case with other tumors at C5 and C7–T1 (not shown).

fig 4.

Case 3: 24-year-old woman with VHLD. A, Sagittal T1-weighted FSE image (700/10/6) shows cord enlargement and decreased signal intensity at T3–T6. B, Contrast-enhanced sagittal T1-weighted FSE image (700/10/6) shows a small, ovoid, well-demarcated, intensely and homogeneously enhancing tumor at T4–T5. C, Sagittal T2-weighted FSE image (4220/120/5) shows isointense tumor (arrow) and hyperintense pencil-shaped lesion from T3 to T6. D, Contrast-enhanced axial T1-weighted FSE image (540/9/3) shows the tumor is superficially located at the posterior aspect of the spinal cord.

fig 5.

Case 4: 44-year-old woman with VHLD who had no spinal cord symptoms. A, Contrast-enhanced sagittal T1-weighted SE image (800/14/2) shows a small, ovoid, well-demarcated, intensely enhancing intramedullary tumor at the anterior aspect of the spinal cord at T10. B, Sagittal T2-weighted TSE image (4500/112/3) shows edema (arrow). C, Contrast-enhanced axial T1-weighted SE image (660/15/1) shows the tumor is superficially located at the anterior aspect of the spinal cord.

fig 6.

Case 1: 16-year-old boy with VHLD. A, Contrast-enhanced sagittal T1-weighted TSE image (700/12/2) shows a large, well-demarcated, intensely and heterogeneously enhancing intramedullary tumor at C7–T2. Note vascular flow voids in and around the tumor (curved arrows) and superficial enhancement of the spinal cord (straight arrows), proved to be dilated perimedullary veins at surgery. Syrinx extends from medulla oblongata to T12. Also note multiple small intensely and homogeneously enhancing tumors, most of which are located superficially at the posterior aspect of the spinal cord. B, Contrast-enhanced axial T1-weighted SE image (660/15/1) at C7–T1 shows enlargement of the spinal canal and the enhancing tumor. The tumor occupies entire dural sac and the spinal cord is barely discernible. The tumor was interpreted as deeply located within the spinal cord on MR image, and was confirmed at surgery.

fig 7.

Case 7: 49-year-old woman without VHLD. A and B, Noncontrast (A) and contrast-enhanced (B) sagittal T1-weighted SE images (600/15/2) show a small, homogeneously and intensely enhancing nodular tumor at C5–C6. The tumor is isointense on noncontrast T1-weighted image. Note extensive syrinx from medulla oblongata to T3 level. C, Contrast-enhanced axial T1-weighted SE image (600/15/2) shows the tumor is well demarcated, superficially located at the anterior aspect of the spinal cord.

fig 8.

Case 5: 49-year-old man with VHLD. A, Contrast-enhanced sagittal T1-weighted TSE image (700/12/3) shows a medium-sized, ovoid, heterogeneously enhancing tumor at T9. Note several small, homogeneously enhancing nodules cephalic to this tumor. Also note flow voids within the tumor and superficial enhancement of the spinal cord. B, Contrast-enhanced axial T1-weighted SE image (660/15/1) shows the tumor at T9 occupies entire dural sac. The tumor was thought to be located deeply within the spinal cord, but turned out to be subpial in location at surgery.