AJR:174, February 2000 377 emangioblastoma is a vascular neoplasm of the central nervous system. Whereas hemangioblas- toma is the most common primary neoplasm in the adult cerebellum, it is a relatively rare tumor of the spine, representing 1.6–5.8% of all spinal tumors [1]. Hemangioblastoma may occur sporadically or as a component of von Hippel- Lindau syndrome. Pathologically, hemangio- blastoma may be difficult to differentiate from astrocytoma. Hemangioblastoma may also re- semble metastatic renal cell carcinoma, a lesion that is common in patients with von Hippel- Lindau syndrome [2]. Radiologic findings are helpful in diagnosing hemangioblastoma. Pre- operative recognition of hemangioblastoma may limit the extent of surgery and may alter patient treatment by indicating von Hippel- Lindau syndrome [3]. A number of distinct ap- pearances of spinal hemangioblastoma have been described, including hemangioblastoma with associated cyst or syrinx [4], hemangio- blastoma with diffuse spinal cord enlargement [5], exophytic hemangioblastoma with minimal spinal cord reaction [6], and extramedullary he- mangioblastoma [7]. This pictorial essay pre- sents the characteristic MR imaging findings that may assist in the preoperative recognition of spinal hemangioblastoma. Clinical Background Von Hippel-Lindau syndrome is an autoso- mal dominant disorder with near complete penetration and variable expression. The prev- alence of von Hippel-Lindau syndrome ranges from 1:40,000 to 1:50,000 [8]. Von Hippel- Lindau syndrome manifests as central nervous system hemangioblastomas, renal cysts, and renal cell carcinomas. Other lesions include retinal angiomas, pheochromocytomas, pan- creatic cysts, and epididymal cystadenomas [9]. Thirty-two percent of patients with spinal hemangioblastoma have von Hippel-Lindau syndrome; the remaining patients have sporad- ically occurring tumors [1]. Patients with spi- nal hemangioblastoma have a mean age of 30 years at onset, though patients with von Hip- pel-Lindau syndrome may present earlier [1]. Symptoms of spinal hemangioblastoma are similar to those of other spinal canal tumors and include sensory change, motor distur- bance, and pain [1]. Because the presentation of spinal hemangioblastoma is similar to that of other spinal canal masses, imaging studies can be useful in suggesting a diagnosis. MR Imaging Characteristics When compared with the spinal cord, spi- nal hemangioblastoma is usually hypointense to isointense on T1-weighted sequences and isointense to hyperintense on T2-weighted se- quences (Fig. 1). T1-weighted images after gadolinium administration show intense en- hancement. Large lesions may be visualized without contrast material, but small lesions are often isointense and thus difficult to differenti- ate from the spinal cord (Figs. 2 and 3). Therefore, gadolinium-enhanced T1-weighted images are essential for the evaluation of le- sions suggestive of hemangioblastoma. Multiple hemangioblastomas have been de- scribed only in patients with von Hippel-Lindau syndrome [1]. Small hemangioblastomas may be observed in patients with no spinal symptoms or in relatives of patients with von Hippel- Lindau syndrome. Patients with von Hippel- Lindau syndrome and spinal symptoms usually have one dominant lesion that causes symptoms, MR Imaging of Spinal Hemangioblastoma Kim B. Baker 1,2 , Christopher J. Moran 1 , Franz J. Wippold II 1,3 , James G. Smirniotopoulos 3,4 , Fabio J. Rodriguez 5 , Steven P. Meyers 6 , Todd L. Siegal 7 Received January 18, 1999; accepted after revision June 28, 1999. 1 Section of Neuroradiology, Mallinckrodt Institute of Radiology, Washington University School of Medicine, 510 S. Kingshighway Blvd., St. Louis, MO 63110. Address correspondence to C. J. Moran. 2 Present address: Department of Diagnostic Imaging, Temple University, 3401 N. Broad St., Philadelphia, PA 19104. 3 Present address: Department of Radiology and Nuclear Medicine, Uniformed Services University of the Health Sciences, 4301 Jones Bridge Rd., Bethesda, MD 20814. 4 Department of Radiologic Pathology, Armed Forces Institute of Pathology, Bldg. 54, Walter Reed Medical Center, Washington, DC 20306-6000. 5 Department of Radiology, University of Missouri at Columbia, 1 Hospital Dr., Columbia, MO 65212. 6 Department of Radiology, Strong Memorial Hospital, University of Rochester Medical Center, Box 648, Rochester, NY 14642. 7 Department of Radiology, Cooper Hospital, 1 Cooper Plaza, Camden, NJ 08103. AJR 2000;174:377–382 0361–803X/00/1742–377 © American Roentgen Ray Society H Pictorial Essay Downloaded from www.ajronline.org by 52.73.204.196 on 05/16/22 from IP address 52.73.204.196. Copyright ARRS. For personal use only; all rights reserved