Spinal extradural hemangioblastoma: A systematic review of characteristics and outcomes

Abstract

Extradural spinal nerve root hemangioblastoma is a rare entity with very few cases reported in the literature. A comprehensive picture of the treatments and outcomes of the same is thus not available. A systematic search was done according to PRISMA guidelines. Search criteria included terms: spinal extradural hemangioblastoma, extradural hemangioblastoma, and spinal root hemangioblastoma. The parameters considered were treatment, motor, and sensory outcome, association with von-Hippel-Lindau (VHL) syndrome. Twenty-two studies (19 full text articles) were available for the review. A total of 39 cases of extradural spinal nerve root hemangioblastoma have been reported. These cases had a median age of 44 years with male predominance (2:1) and up to 48% occur in the thoracic level, similar to our case. Thirty-six percent of patients were associated with VHL syndrome. Surgical resection was the primary modality of treatment with embolization used in selected cases (20%). They had mean follow-up of 23 (±11) months. The prognosis was better than the intradural counterpart with no motor deficit and sensory deficit in only 9%. Preoperative identification of the extradural nature of this pathology and complete excision at the first surgery offers excellent outcomes compared to intradural lesion. Targeted embolization may be used in cases anticipated with high blood loss.

Keywords: Hemangioblastoma; nerve root hemangioblastoma; spinal hemangioblastoma; systematic review.

Figure 1

PRISMA chart of methodology of the systematic review

Figure 2

(a) Gadolinium-enhanced axial magnetic resonance image showing a brilliantly enhancing lesion et the T8 level that is extradural (black line forming the inner margin is the dura mater) with severe compression of the cord and preserved rib surface. (b) Gadolinium-enhanced axial magnetic resonance image taken on postoperative day 1 showing complete resection of the lesion with resolution of the compression on the spinal cord and preserved rib and pleural surface

Figure 3

(a) T2-sagittal magnetic resonance image of the cervical spine with a nodule at the C4 level with syrinx extending up to the medullary region level. (b) Gadolinium-enhanced sagittal Magnetic resonance image showing a brilliantly enhancing lesion et the T8 level with scalloping of the T8 vertebral body. (c) Gadolinium-enhanced sagittal Magnetic resonance image taken on postoperative day 1 showing complete excision of the lesion with no residual enhancement

Figure 4

(a) Picture of en bloc excision of the lesion with coagulated surface visible. (b and c): Histopathology sequence showing the hemangioblastoma composed of many vascular channels (b, H and E, ×100) separated by vacuolated stromal cells (c, H and E, ×200)