Suprasellar Hemangioblastoma Unrelated to von Hippel-Lindau Disease Successfully Treated through Extended Transsphenoidal Approach: Diagnostic Value of Von Hippel-Lindau Disease Gene-Derived Protein.
Suprasellar Hemangioblastoma Unrelated to von Hippel-Lindau Disease Successfully Treated through Extended Transsphenoidal Approach: Diagnostic Value of Von Hippel-Lindau Disease Gene-Derived Protein.
复制标题
通过扩展经蝶入路成功治疗与冯希佩尔-林道病无关的鞍上血管母细胞瘤：冯希佩尔-林道病基因衍生蛋白的诊断价值。
DOI：
10.1055/s-0036-1582016
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发表时间：
2017
期刊：
影响因子：
--
通讯作者：
Tominaga T.
中科院分区：
文献类型：
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作者：
Wenting J;Ogawa Y;Ito J;Tominaga T.
血管母细胞瘤往往发生在幕下区域，很少发生在幕上区域。该肿瘤与 von Hippel-Lindau 病 (VHLD) 密切相关，尤其是幕上区域，只有 12 例鞍上血管母细胞瘤与 VHLD 无关。然而，所有这些病例都是基于临床筛查和家族史确认而诊断的。我们报道一例鞍上血管母细胞瘤病例，通过扩大经蝶入路成功切除，经免疫组织化学检查诊断为与VHLD无关的散发病例。一名67岁女性患有轻度尿崩症和视野缺损。头部磁共振成像显示鞍上肿瘤向上压迫视交叉。通过扩大经蝶入路，肿瘤被完全切除，没有出现并发症。术后组织学检查发现大量空泡基质细胞和丰富的毛细血管网络，诊断为血管母细胞瘤。临床筛查和家族史确认未发现特异性结果，额外的免疫组化染色显示抗VHLD基因衍生蛋白（pVHL）胞浆弥漫表达。我们强调，采用pVHL免疫组化评估是明智的，特别是对于与年龄较小和幕上病变相关的VHLD高风险的患者。
Hemangioblastoma tends to occur in the infratentorial regions and rarely in the supratentorial regions. This tumor is strongly associated with von Hippel-Lindau disease (VHLD), especially in the supratentorial regions, with only 12 cases of suprasellar hemangioblastoma unrelated to VHLD. However, all these cases were diagnosed based on clinical screening and confirmation of family history. We report a case of suprasellar hemangioblastoma that was successfully removed through an extended transsphenoidal approach and diagnosed as a sporadic case unrelated to VHLD by immunohistochemical examination.A 67-year-old woman had mild diabetes insipidus and a visual field defect. Head magnetic resonance imaging revealed a suprasellar tumor compressing the optic chiasm upward. The tumor was totally removed without complication through an extended transsphenoidal approach. Postoperative histologic examination disclosed large vacuolated stromal cells and rich capillary networks, and the diagnosis was established as hemangioblastoma. Clinical screening and confirmation of family history revealed no specific results, and additional immunohistochemical staining showed diffuse cytoplasmic expression of anti-VHLD gene-derived protein (pVHL).We emphasize that pVHL immunohistochemical evaluation would be wise to adopt, especially for patients with a high risk of VHLD related to younger age and supratentorial lesions.