Spinal Cord Hemangioblastomas in von Hippel-Lindau Disease: Management of Asymptomatic and Symptomatic Tumors

Abstract

PurposeStandard treatment of asymptomatic spinal cord hemangioblastoma in von Hippel-Lindau (VHL) disease has yet to be established. The purpose of this study was to propose guidelines for the treatment of asymptomatic spinal cord hemangioblastomas in VHL disease.Materials and MethodsVHL disease patients treated for spinal cord hemangioblastomas between 1999 and 2009 were included. All spinal cord hemangioblastomas were divided into three groups: Group 1, asymptomatic tumors at initial diagnosis followed with serial imaging studies; Group 2, asymptomatic tumors at initial diagnosis that were subsequently resected; and Group 3, symptomatic tumors at initial diagnosis, all of which were resected.ResultsWe identified 24 spinal cord hemangioblastomas in 12 patients. Groups 1, 2 and 3 comprised 13, 4 and 7 tumors, respectively. Group 1 exhibited a smaller tumor volume (257.1 mm3) and syrinx size (0.8 vertebral columns) than those of Group 2 (1304.5 mm3, 3.3 vertebral columns) and Group 3 (1787.4 mm3, 6.1 vertebral columns). No difference in tumor volume or syrinx size was observed between Groups 2 and 3. Five tumors in Group 1 were resected during follow-up because symptoms had developed or the tumor had significantly grown. Finally, among 17 asymptomatic tumors at the initial diagnosis, nine tumors were resected. Only one tumor of these nine tumors resulted in neurological deficits, while five of seven symptomatic tumors caused neurological deficits.ConclusionSelective resection of asymptomatic tumors before they cause neurological deficits might bring about better outcomes.

Spinal hemangioblastomas: analysis of surgical outcome and prognostic factors

“…Similar to the literature data, the majority of the HBs in our patients occurred in the cervical and dorsal spinal cord. A reason for this may be the predominant distribution of embryonic precursor cells in these areas of the spinal cord [11,21,22]. Overall, onset symptoms in our series recapitulate those commonly reported in other studies: sensory and motor de cits, pain, urinary dysfunction, and occasionally bulbar symptoms.…”

“…Since HBs have normally a clear border separating them from the spinal cord, monitoring is considered by some authors not useful to increase safety in surgical resection [13]. However, in the last 20 years the use of IONM during the resection of spinal HBs has become more and more common, with an increasing number of authors showing the importance of such method also in HBs removal, both for surgical strategy and for prognostic reasons [4,6,22,25,27,15,26].…”

Preoperative angiographic considerations and neurological outcome after surgical treatment of intradural spinal hemangioblastoma: a multicenter retrospective case series

“…Spinal hemangioblastomas are rare tumors and constitute only 2-6% of all primary spinal cord tumors [1][2][3]. They may appear sporadically or may be associated with a familiar neoplastic condition with autosomal dominance, the von Hippel-Lindau syndrome (VHL) [4][5][6], exhibiting other tumorous lesions such as cerebellar and retinal hemangioblastoma, abdominal pheochromocytoma, renal cell carcinoma, pancreatic cysts, and neuroendocrine tumors. [7] Hemangioblastomas present highly vascularized benign lesions and syringomyelia is often present.…”

Combined Microsurgery and Radiotherapy for Multiple Spinal Cord Hemangioblastomas with Holocord Syrinx in von Hippel-Lindau Disease: A Case Report

“…Although surgical resection constitutes the therapy of choice for spinal HABs, radiotherapy has become increasingly important for the treatment of surgically not amenable lesions in both cranial and spinal locations. 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22…”