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幕上血管母细胞瘤十分罕见,自1902年由Bielschowski首次描述以来,文献中仅报道了85例,而且好发于大脑半球,偶见于侧脑室内,鞍内型者仅有2例尸检报告。13岁以下发病者仅有6例。作者报道了首例11岁儿童有症状的鞍内血管母细胞瘤。 患儿女性,双额部及右眶部疼痛伴进行性视力减退,疗程3个月。其多名一级亲属中有Von—Hip-pel—Lindau(VHL)病历史。身高及体重低于正常值约10％,右眼矫正视力20/40,左眼20/125,双颞侧偏盲,双视乳头轻度苍白,未见视网膜血管瘤,眼球
Supratentorial hemangioblastoma is very rare. Since the first description by Bielschowski in 1902, only 85 cases have been reported in the literature, and occur in the hemisphere of the brain. Occasionally in the lateral ventricle, there are only 2 autopsy reports in the secundum. Only 6 patients under 13 years of age. The authors report the first 11-year-old child with symptomatic saddle hemangioblastoma. Children with children, double-frontal and right orbital pain with progressive visual loss, treatment for 3 months. Many first-degree relatives have Von-Hip-pel-Lindau (VHL) disease history. Height and weight less than about 10% of normal, right eye corrected visual acuity 20/40, left eye 20/125, bilateral temporal hemianopsia, bimodal nipple slightly pale, no retinal hemangioma, eyeball