A 6-month-old infant with a spinal cord hemangioblastoma located in the conus medullaris is reported from the University of Washington School of Medicine, Children’s Hospital, Seattle, WA. At birth the physical examination was normal except for a lumbosacral dimple. By 1 mo the dimple had deepened and a hemangioma developed at the site. CT and CT metrizamide myelography revealed a posterior filling defect at the level of the conus medullaris. At laminectomy, a congenital dermal sinus tract, cutaneous capillary hemangioma and cord hemangioblastoma were resected. There were no neurologic deficits either postoperatively or following removal of the tumor. [1]
COMMENT. Spinal cord hemangioblastoma rarely presents in infancy and is usually manifest from the third to the fifth decades. It may be associated with a variety of cutaneous and other lesions, including von Hippel-Lindau and Cobb syndromes. Von Hippel-Lindau disease is an autosomal dominant condition characterized by hemangioblastomas of the cerebellum, medulla and spinal cord; angiomas of the retina, liver, and kidney; pheochromocytomas, adenomas, or cysts of the kidney and epididymis; and pancreatic cysts. In Cobb syndrome, cutaneomeningospinal angiomatosis, spinal cord arteriovenous malformations are associated with cutaneous vascular lesions in corresponding dermatomes. These syndromes were not found in the above case-report.