血管母细胞瘤常并发Von Hippel Lindau(VHL)综合症,好发于颅内。本文报告一例右桡神经血管母细胞瘤病例。 患者,女性,79岁,右肩向臂放射性疼痛4月。体查,右上臂背面一个坚实的肿块,神经系统检查未见异常。MRI示肿块2×1.8×2.2cm,分界清,与桡神经关系密切,周围多处流空信号提示血管丰富。手术见肿瘤为紫红色,有包膜,位于肱肌和肱三头肌之间软组织中,累及外侧肌间隔,周围血管丰富。冰冻切片诊断为转移癌(似肾细胞癌),切除肿瘤及贯穿于其中的桡神经。术后病情稳定,但肱桡肌远端完全性桡神经麻痹。CT发现单纯肾囊肿,无肿瘤征象。病理检查见肿瘤质硬1.5×2.5×2cm大小,切面黄色至红色,瘤内有脂肪沉积。常规光镜见大量泡沫细 Hemangioblastoma often complicated by Von Hippel Lindau (VHL) syndrome, occurs in the brain. This article reports a case of right radial neuroblastoma. Patient, female, 79 years old, radioactive pain in right shoulder arm April. Physical examination, a solid mass on the back of the right upper arm, no abnormal neurological examination. MRI showed mass 2 × 1.8 × 2.2cm, demarcation clear, and the radial nerve is closely related to multiple empty flow around the signal prompted rich blood vessels. Surgery to see the tumor was purple, enveloped, located in the soft tissue between the brachial and triceps, involving the lateral muscle space, the surrounding blood vessels rich. Frozen sections were diagnosed as metastatic carcinoma (like renal cell carcinoma), tumors were excised and the radial nerve penetrated therethrough. Postoperative stable condition, but the distal radial brachial radial muscle paralysis completely. CT found simple renal cysts, no tumor signs. Pathological examination, see the hard tumor mass 1.5 × 2.5 × 2cm size, section yellow to red, fat deposition within the tumor. Conventional light see a lot of thin foam