脊髄血管芽腫の手術 : 散発例とVHL合併例とで差があるのか?

概要

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Spinal hemangioblastomas may occur sporadically or in association with von Hippel-Lindau(VHL) syndrome. The purpose of this study is to elucidate the clinical differences between sporadic and VHL groups retrospectively. Eighteen patients presented with spinal hemangioblastomas. Eleven patients harbored sporadic ones and 7 had VHL syndrome. Although patients in VHL group tend to be presented with clinical symptoms at a younger age than do patients in sporadic group, no differences were found in initial symptoms, duration of symptoms and the location of the tumor. MRI findings depended on the size of tumors, however, there was no difference between two patient groups, except for the multiplicity and higher percentage of small tumors in VHL group. Surgical outcomes are favorable in both groups. Surgical outcomes for spinal hemangioblastomas are generally favorable. However, management of lesions is more difficult for patients with VHL syndrome. Therefore, in patients with spinal hemangioblastoma, clinical and neuroradiological screenings are important to differentiate between sporadic hemangioblastoma or in association with VHL syndrome.
日本脊髄外科学会の論文