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酷似垂体瘤的鞍区神经母细胞瘤:病例报告及文献复习

Sellar neuroblastoma mimicking a pituitary tumour: case report and review of the literature.

作者信息

Schmalisch K, Psaras T, Beschorner R, Honegger J

机构信息

Department of Neurosurgery, University of Tuebingen, Hoppe-Seyler-Str. 3, D-72076 Tuebingen, Germany.

出版信息

Clin Neurol Neurosurg. 2009 Nov;111(9):774-8. doi: 10.1016/j.clineuro.2009.06.011. Epub 2009 Jul 28.

Abstract

Neuroblastomas of the sellar region are exceedingly rare. Only 2 cases have previously been reported. Management of these tumours depends on the tumour's primary site, the patient's age and histopathological features. We are reporting the case of a 43-year-old woman who developed progressive bitemporal hemianopsia and visual loss, accompanied by amenorrhea and hyponatremia. Laboratory findings revealed a slightly elevated prolactin level. Cranial MR-imaging displayed an intrasellar and suprasellar lesion with a maximum diameter of 2.5 cm that was suspicious for a pituitary adenoma or tuberculum sellae meningioma. The tumour was approached via a pterional trepanation. Intraoperatively, the tumour was highly vascularized and adhesive to the optic chiasm, the floor of the third ventricle, the hypothalamus and the hypophyseal stalk. Postoperatively, vision improved and prolactin dropped to normal values, but hyponatremia persisted. Histopathological examination revealed a neuroblastoma with strong positivity for synaptophysin and chromogranin, MAP-2 protein and NeuN-antigen in the immunohistochemistry. No pituitary hormone receptors were expressed. The MIB-1 labelling index was positive in 5% of the cell nucleoli. In the further course, the patient underwent radiotherapy of the neuroaxis. A brief review of the literature is presented.

摘要

鞍区神经母细胞瘤极为罕见。此前仅报道过2例。这些肿瘤的治疗取决于肿瘤的原发部位、患者年龄和组织病理学特征。我们报告一例43岁女性病例,该患者出现进行性双颞侧偏盲和视力丧失,伴有闭经和低钠血症。实验室检查结果显示催乳素水平略有升高。头颅磁共振成像显示鞍内和鞍上有一最大直径为2.5 cm的病变,怀疑为垂体腺瘤或鞍结节脑膜瘤。通过翼点开颅术处理该肿瘤。术中,肿瘤血管丰富,与视交叉、第三脑室底部、下丘脑和垂体柄粘连。术后,视力改善,催乳素降至正常水平,但低钠血症持续存在。组织病理学检查显示为神经母细胞瘤,免疫组化中突触素、嗜铬粒蛋白、微管相关蛋白2(MAP - 2)蛋白和神经元核抗原(NeuN)呈强阳性。未表达垂体激素受体。MIB - 1标记指数在5%的细胞核仁中呈阳性。在后续病程中,患者接受了神经轴放疗。本文还对相关文献进行了简要综述。

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