Service de neurochirurgie, centre hospitalo-universitaire Rangueil, 1, avenue du Pr-Jean-Poulhès, TSA 50032, 31059 Toulouse cedex, France.
Ann Endocrinol (Paris). 2012 Jun;73(3):216-21. doi: 10.1016/j.ando.2012.02.001. Epub 2012 Apr 11.
The primary intracranial development of olfactory neuroblastomas, outside olfactory epithelium, is rare. We report a case of primary sellar neuroblastoma without any aggressive histopathological features, managed solely surgically without adjuvant therapy, with good outcomes at 3 years. Primary sellar neuroblastomas mostly occur in women in the 4th decade with a context of a non-secreting pituitary tumour. Diagnosis is made on histopathological examination (small cells, fibrillary intercellular background, strong immunoreactivity for neurons markers, negative immunoreactivity for anterior pituitary hormones). Management is based on surgery. Adjuvant treatment is not consensual, largely depends on patient's conditions and aggressive histopathological features.
嗅神经母细胞瘤原发于嗅上皮外的颅内极为罕见。我们报告一例无任何侵袭性组织病理学特征的原发性鞍内神经母细胞瘤，仅通过手术治疗，未行辅助治疗，3 年后预后良好。原发性鞍内神经母细胞瘤主要发生于 40 岁左右的无分泌性垂体瘤女性患者。诊断基于组织病理学检查（小细胞、纤维状细胞间背景、神经元标志物强免疫反应性、前垂体激素免疫反应阴性）。治疗基于手术。辅助治疗尚无共识，主要取决于患者的情况和侵袭性组织病理学特征。
