Zhang Michael, Mahavadi Anil K, Deftos Michael L, Ali Arshad, Singh Harminder
Department of Neurosurgery, Stanford Medical Center, Stanford, California, United States.
Department of Neurological Surgery, University of Miami Miller School of Medicine, Miami, United States.
J Neurol Surg B Skull Base. 2021 May 12;83(Suppl 3):e655-e656. doi: 10.1055/s-0041-1727128. eCollection 2022 Aug.
Sellar xanthogranulomas (XGAs) are a rare pathological subtype of hypophysitis reflecting a degenerative process of Rathke's cleft cyst with predilection in young adults. While the histological features have been described, there is limited discussion on the technical expectations in surgical management. We present the clinical, radiographic, and surgical features of the third literature-reported XGA in the pediatric population. The patient was a 17-year-old boy who first identified by ophthalmologically confirmed peripheral vision loss. Subsequent endocrine workup identified delayed-onset puberty and hypopituitarism. Magnetic resonance imaging (MRI) showed a nonenhancing 2.6-cm T1 and T2 sellar-based hyperintense mass with suprasellar extension and mass effect on the optic chiasm. A small T1 hypointense encased nodule was also seen ( Fig. 1 ). Consent for resection was obtained. Intraoperatively the tumor was firm and adherent, requiring piecemeal removal. Radiofrequency ablation enabled ergonomic debulking and minimize thermal injury ( Fig. 2 ). 1 We used initial settings of 25 W, equivalent to 55 W. A cystic component with motor oil-like fluid was encountered and decompressed. The tumor was notably very adherent to the optic nerve and infiltrated the stalk, requiring its truncation. Closure was achieved by fat graft dead space plugging, fascia lata underlay, Medpor gasket seal, and nasoseptal flap. Pathology confirmed dense fibrous tissue with features of chronic inflammation, cholesterol clefts, hemosiderin pigment, multinucleate giant cells, and foamy macrophages. Additional cyst wall sampling identified squamous and ciliated epithelial lining, collectively consistent with Rathke's cleft cyst and xanthogranulomatous reaction. These lesions can undergo surgical cure with resection, most commonly by transsphenoidal approach. The link to the video can be found at: https://youtu.be/S2n5iQ3aFgc .
鞍区黄色肉芽肿(XGAs)是一种罕见的垂体炎病理亚型,反映了拉克氏裂囊肿的退行性过程,好发于年轻人。虽然已经描述了其组织学特征,但关于手术治疗的技术期望的讨论有限。我们报告了儿科人群中第三例文献报道的XGA的临床、影像学和手术特征。
患者为一名17岁男孩,最初因眼科确诊的周边视力丧失而被发现。随后的内分泌检查发现青春期延迟和垂体功能减退。磁共振成像(MRI)显示一个2.6厘米的鞍区T1和T2加权像上的高信号肿块,无强化,向上延伸至鞍上,对视交叉有占位效应。还可见一个小的T1加权像低信号包绕结节(图1)。获得了切除的同意。
术中肿瘤质地硬且粘连,需要分块切除。射频消融实现了符合人体工程学的减容并将热损伤降至最低(图2)。我们使用的初始设置为25瓦,相当于55瓦。遇到一个含有机油样液体的囊性成分并进行了减压。肿瘤明显与视神经紧密粘连并浸润了垂体柄,需要将其截断。通过脂肪移植填充死腔、阔筋膜下层铺垫、Medpor垫片密封和鼻中隔瓣实现了缝合。
病理证实为致密纤维组织,具有慢性炎症、胆固醇裂隙、含铁血黄素色素、多核巨细胞和泡沫状巨噬细胞的特征。额外的囊肿壁取样发现鳞状和纤毛上皮内衬,总体符合拉克氏裂囊肿和黄色肉芽肿反应。这些病变通过切除手术通常可以治愈,最常见的是经蝶窦入路。视频链接可在:https://youtu.be/S2n5iQ3aFgc 找到。