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1例罕见的周围神经血管母细胞瘤——病例报告及文献复习

A rare case of peripheral nerve hemangioblastoma—case report and literature review.

作者信息

Rasulic Lukas, Samardzic Miroslav, Bascarevic Vladimir, Micovic Mirko, Cvrkota Irena, Zivkovic Bojana

机构信息

Clinic of Neurosurgery, Clinical Center of Serbia, Koste Todorovica 4, Belgrade, 11000, Serbia,

出版信息

Neurosurg Rev. 2015 Jan;38(1):205-9; discussion 209. doi: 10.1007/s10143-014-0587-x. Epub 2014 Oct 17.

Abstract

Hemangioblastoma is a rare benign tumor that consists of neoplastic vacuolated stromal cells and highly developed capillary blood vessels. These tumors are almost exclusively found in the central nervous system, but in extremely rare cases, it can arise in peripheral nerves. In the majority of cases, it occurs sporadically (60 to 75%), but in about 25% of cases, it is associated with von Hippel-Lindau disease. We present the first case of ulnar nerve hemangioblastoma in a 70-year-old male. The patient presented with a slow-growing palpable mass on the front side of the right upper arm. Macroscopically, the tumor was round shaped, encapsulated, reddish-orange in color, very well vascularized, and about 3 cm in diameter; one nerve fascicle was entering the tumor tissue, so it was resected with the tumor. The rest of the nerve fascicles were intact. Postoperative course was uneventful. Histopathological analysis with immunohistochemical analysis confirmed that the tumor was a peripheral nerve hemangioblastoma WHO grade I. Physical treatment was conducted, and there was no motor neurological deficit on follow-up after 3 months, only hypoesthesia of the fourth and fifth finger. These lesions are so rarely found arising from peripheral nerves that only four published cases exist in literature today. There is very little data about these tumors in world literature, so we consider our paper a valuable contribution to global knowledge on these tumors.

摘要

血管母细胞瘤是一种罕见的良性肿瘤,由肿瘤性空泡状间质细胞和高度发育的毛细血管组成。这些肿瘤几乎仅见于中枢神经系统,但在极少数情况下,也可发生于周围神经。在大多数病例中,其呈散发性(60%至75%),但约25%的病例与冯·希佩尔-林道病相关。我们报告首例70岁男性尺神经血管母细胞瘤病例。患者右侧上臂前侧出现一个生长缓慢、可触及的肿块。大体上,肿瘤呈圆形,有包膜,颜色为红橙色,血管丰富,直径约3厘米;有一个神经束进入肿瘤组织,故将其与肿瘤一并切除。其余神经束完整。术后过程顺利。组织病理学分析及免疫组化分析证实该肿瘤为WHO I级周围神经血管母细胞瘤。进行了物理治疗,3个月后随访时无运动神经功能缺损,仅第四和第五指感觉减退。这些病变极少发生于周围神经,目前文献中仅报道了4例。世界文献中关于这些肿瘤的数据非常少,因此我们认为我们的论文对全球关于这些肿瘤的认识有重要贡献。

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