Pituitary stalk hemangioblastoma: the fourth case report and review of the literature

Supratentorial hemangioblastomas (HBL) have been rarely described in the literature. Herein we report the fourth case of pituitary stalk HBL diagnosed concurrently with cerebellar HBLs in a 51-year-old woman with von Hippel-Lindau disease. Complete resection of the lesion was achieved using left frontopterional craniotomy and no recurrence was observed after 8 years of follow-up. The clinical presentation, radiological features, pathological findings, and the management of this particular case are discussed in the light of the available literature. To our knowledge, this case is the only pituitary stalk HBL with total resection, and a long follow-up fully documented.     

Sellar gangliocytoma with adrenocorticotropic and prolactin adenoma

We report a case of a 60-year-old man who presented with weight gain, headaches, dizziness, erectile dysfunction and decreased libido. He was found to have elevated adrenocorticotropic hormone (ACTH) and prolactin serum levels. The imaging studies revealed a 1.4 cm sella/suprasellar mass which was compressing the optic chiasm. Histologic slides of the lesion showed a pituitary adenoma, marked by a proliferation of biphenotypic appearing cells, associated with a gangliocytoma, and marked by a proliferation of atypical appearing neuronal cells arranged against a glial-appearing background. Pituitary adenoma-gangliocytomas are benign combination tumors that rarely occur in the sellar region. Adenomas in this setting are sometimes functional, and rare patients with mixed adenomas (adenomas secreting more than one hormone) have been reported. To our knowledge, there has been only one other report of a combined ACTH and prolactin-producing adenoma with gangliocytoma, reported in a patient who also had acromegaly. In our patient, the immunohistochemical stains demonstrated that the bulk of the adenoma cells stained with prolactin antibody, and scattered clusters of cells within the adenoma stained positively for ACTH. The adenoma did not stain with antibodies to any of the other anterior pituitary hormones. Postoperatively, the elevated prolactin and ACTH levels returned to normal levels and there was no evidence of residual tumor. Adequate sampling and immunohistochemistry are important in rendering a correct diagnosis and in identifying the hormone status of mixed adenoma-gangliocytomas.     

Pleomorphic xanthoastrocytoma arising from the suprasellar region: A report of two cases

Pleomorphic xanthoastrocytoma (PXA) is a rare astrocytic neoplasm that usually arises in children and young adults. Typically, lesions of PXA are superficially located in the cerebral hemispheres. Herein, we report two extremely rare patients with PXA arising from suprasellar regions. One of the patients is a 29-year-old man admitted to our hospital with a history of progressive headache for 1 month. The patient’s brain MRI revealed a large tumor arising from the suprasellar cistern of the third ventricle. The second patient, a 52-year-old woman, presented with progressive dizziness and visual disturbance that had developed over the course of 1 year. The MRI revealed a well-enhanced suprasellar solid mass measuring 1.4 × 1.2 × 1.4 cm. Both patients underwent surgical removal of their tumors, and both patients showed similar microscopic structures and immunohistochemical phenotypes: the tumor cells were pleomorphic with mixtures of spindle-shaped, and multinuclear giant cells. In addition, eosinophilic granular bodies and xanthomatous cells were seen on section. Immunohistochemistry was positive for GFAP, S-100, and CD34, and was negative for IDH 1, CK, and Syn. The Ki-67 proliferation index was less than 1%. Silver impregnation revealed reticulin fibers surrounding the individual tumor cells, and small cell groups. Based on these findings, the two patients were diagnosed with PXA in the suprasellar region. To date, only five such patients have been reported in the literature. PXA should be included in the differential diagnosis for tumors arising in the sellar region.     

神经内镜辅助微侵袭手术切除垂体腺瘤并鞍底重建

目的 评价应用神经内镜辅助、经单鼻孔-蝶窦入路切除垂体腺瘤(PAs)的微侵袭手术技术、鞍底重建与疗效.方法 从2001年4月至2009年3月,对第四军医大学西京医院连续收住院的426例PAs患者行神经内镜下经单鼻孔-蝶窦入路手术.本组病例根据Hardy's PAs分类标准:Ⅰ型54例(12.7%),Ⅱ型105例(24.6%),Ⅲ型181例(42.5%),Ⅳ型86例(20.2%).结果 肿瘤切除根据术术中所见及术后MRI或CT扫描进行评估,370例(86.9%)肿瘤获全切除,32例(7.5%)达次全切除,余24例(5.6%)纤维性肿瘤获部分切除.本组术后3例死亡(0.7%),24例(5.6%)发生CSF漏而须行修补术,6例(1.4%)有蝶窦黏膜炎和脑膜炎,用药物治疗得以控制.结论 质地稍软的PAs无论有无鞍上扩展、但未侵及血管者,在神经内镜下经单鼻孔-蝶窦入路、术中将内镜变换角度,可获得安全而满意的切除.     

鞍区巨大肿瘤术后水钠紊乱的临床分析

目的：探讨鞍区巨大肿瘤术后水钠紊乱的临床特点与治疗方法。方法：回顾性分析36例鞍区巨大肿瘤术后水钠紊乱的临床资料。结果：36例患者中28例出现尿崩症，其中9例术后1周内恢复。10例术后2～3周内恢复，5例1月内恢复，2例3月内恢复，2例术后半年仍有多饮，多尿。32例出现血钠紊乱，其中13例1周内恢复，16例术后2周内恢复，3例术后3周内恢复，结论：鞍区巨大肿瘤术后水钠紊乱情况复杂，在临床实践中水钠紊乱的各种类型常同时并存或相继发生。针对不同类型进行及时有效的治疗是关键。     

鞍区生殖细胞瘤的MR 影像

目的 分析鞍区生殖细胞瘤的MR影像特点。方法 经手术及病理证实的鞍区生殖细胞瘤38例,男12例,女26例,年龄3－32岁。结果 根据位置不同将其分为三类：I类,肿瘤位于第三脑室内,包括从第三脑室底向上长入第三脑室,共20例;Ⅱ类;仅位于第三脑室区,累及视交叉,漏斗、垂体柄、视神经和视束,共15例,Ⅲ类;仅位于鞍内,共3例,结论 MRI对Ⅰ和Ⅱ类生殖细胞瘤的诊断有重要价值,MRI对Ⅲ类不易作出诊断,必须结合临床。     

鞍区胶质瘤的CT和MRI诊断

目的分析鞍区胶质瘤的CT和MRI特点及临床发病特征。资料与方法经手术病理证实的鞍区胶质瘤8例,其中7例位于鞍上,1例位于鞍内。3例行CT检查,5例行MRI检查。结果鞍上胶质瘤体积较大,呈不规则分叶状,边界光滑锐利,基本没有瘤周水肿,瘤内偏上方多发囊变,无明显钙化及出血,少数可沿视觉通路生长,CT平扫稍低或等密度,MRI检查T1WI呈稍低、低信号,T2WI高信号,CT及MR增强扫描,肿瘤实性部分可见显著强化。此外,临床上这些患者就诊时多有眼部症状．而均无下丘脑及垂体内分泌异常症状。鞍内胶质瘤位置偏后,瘤周无水肿,瘤内可有多发微囊变,无明显钙化及出血。结论鞍上胶质瘤有比较典型的CT和MRI特点,结合患者临床发病特点,有助于提高其术前诊断率;鞍内胶质瘤易误诊,对有多发微囊变的位置偏后的鞍内肿瘤,应考虑到本病可能。     

冠状扫描在鞍区病变CT诊断中的意义

通过对９８例鞍区病变正误诊断经验分析，认为冠状ＣＴ扫描不仅在鞍区病变诊断和鉴别诊断中具有重要的意义，而且能精确地了解病变位置、大小及其发展方向，揭示病变的三维空间关系，从而为临床治疗提供方便。     

腺垂体梭形细胞嗜酸细胞瘤临床病理观察

目的探讨腺垂体梭形细胞嗜酸细胞瘤的临床病理特征、诊断及鉴别诊断要点。方法对1例鞍区腺垂体梭形细胞嗜酸细胞瘤的临床表现、影像学特征、组织形态学、免疫表型及超微结构进行分析,并复习相关文献。结果患者女性,26岁。视力下降3年,停经2年半。影像学提示鞍区占位,术前拟诊为“脑膜瘤”。光镜下梭形肿瘤细胞呈束状或不规则排列,胞质丰富,嗜酸性,局部区域明显黏液变性,间质散在或小灶性淋巴细胞浸润。肿瘤细胞vimentin、S-100和EMA（＋）,Ki-67标记指数〈1％。电镜下以瘤细胞胞质内大量肿胀的线粒体为特征,缺乏分泌颗粒。结论腺垂体梭形细胞嗜酸细胞瘤是罕见的鞍区肿瘤之一,应与鞍区的其他梭形细胞肿瘤相鉴别,熟悉其临床病理特征,依靠免疫组化和电镜可确诊。