They are almost always associated with a syrinx or significant edema.
Cases associated with edema and syrinx are more space-occupying than those only with solid part of the tumor. Consequently, the mass effect producing neurological symptoms derives from the cyst rather than the tumor itself. On the removal of hemangioblastomas in association with a syrinx, the syrinx is spontaneously opened and always stops growing and usually regresses in size. Thus, the additional opening of the syrinx or surgical removal of the syrinx is not necessary 3).
Although some investigators recommend preoperative embolization, 4) 5) in the series of Harati et al. it was usually not necessary to achieve complete resection 6). This is in concordance to several other series so that preoperative embolization is generally not recommended 7) 8) 9) 10) 11). To prevent intraoperative bleeding in selected cases, temporary artery occlusion was performed. This technique is described in detail by Clark et al. 12).
As vascular tumors, intramedullary hemangioblastomas are associated with significant intraoperative blood loss, making them particularly challenging clinical entities. The use of intraoperative indocyanine green or other fluorescent dyes has previously been described to avoid breaching the tumor capsule, but improved surgical outcomes may result from identifying and ligating the feeder arteries and arterialized draining veins.
Molina et al. presented a written and media illustration of a technique for intraoperative indocyanine green use in the en bloc resection of intramedullary hemangioblastoma 13).
Cyberknife radiosurgery has proven to be safe in the treatment of spinal HBs 14). However, as radiographic regression was achieved in only 22%, microsurgical resection remains the gold standard for spinal HBs that are clearly symptomatic or have developed radiographic progression in size, spinal cord edema, or syrinx 15) 16) 17).