Extramedullary intradural hemangioblastomas: approaching a strange pathology

Abstract

Background: Hemangioblastomas are highly vascularized rare tumors of the central nervous system (CNS), WHO grade I, commonly intra-axial and located in the cerebellum and retina, either sporadically or associated with von Hippel Lindau disease (VHL). Spinal topography is uncommon, and its extramedullary presentation is extremely infrequent. The experience in the literature is scarce but surgery is usually the treatment of choice.

Objective: To describe our experience in the treatment of extramedullary spinal hemangioblastomas and to review the diagnostic characteristics and therapeutic behavior.

Description of the case: Case No. 1. A 67-year-old male patient with progressive neck pain for 5 months, predominant distal weakness in the left upper limbs and hypoesthesia in the fingers of both hands. MRI with intradural-extramedullary nodular lesion at C3-C4. Case No. 2. 64-year-old male patient with lumbar back pain and right sciatica for 6 months with sensory deficit. MRI with intradural-extramedullary lesion in L1-L2 on the left, with homogeneous post-contrast enhancement. Case No. 3. A 22-year-old male patient with Von Hippel Lindau disease presented with weakness in the left lower limb. MRI with multiple cerebellar and dorsal cord hemangioblastomas, highlighting a voluminous lesion at T7-T9.

Surgery: Case No. 1. The surgery consisted of C3-C5 laminectomy and resection of the hemangioblastoma, with complete recovery. Case No. 2. Laminectomy was performed with total resection of the hemangioblastoma, with complete recovery. Case No. 3. T7-T9 laminectomy was performed with complete excision of the hemangioblastoma, with partial postoperative improvement and intervention.

Conclusion: Hemangioblastomas are rare tumors. Its spinal and extra axial presentation is even more singular. Given the high vascularization of these lesions, we consider the use of spinal angiography prior surgery for diagnostic or even therapeutic aid. Surgery is the treatment of choice, and for that purpose, the use of neuromonitoring with D wave and evoked potentials is paramount to identify neurological deficits and pursue the aim of good functional results.