Kleinschmidt-DeMasters B K, Lillehei Kevin O, Hankinson Todd C
Department of Pathology, University of Colorado Anschutz Medical Campus, Aurora, CO.
Department of Neurology, University of Colorado Anschutz Medical Campus, Aurora, CO.
Brain Pathol. 2017 May;27(3):377-395. doi: 10.1111/bpa.12498.
Xanthomatous lesions of the sellar region have traditionally been divided into two separate categories, xanthomatous hypophysitis (XH) and xanthogranuloma (XG) of the sellar region. The seminal article on XH, a condition typified by foamy histiocytes and lymphoplasmacytic infiltrates in the pituitary gland/sellar region, but usually little or no hemosiderin pigment, detailed three patients. However, most reports since that time have been single cases, making understanding of the entity difficult. In contrast, the seminal report on XG, characterized by sellar region cholesterol clefts, lymphoplasmacytic infiltrates, marked hemosiderin deposits, fibrosis, multinucleated giant cells around cholesterol clefts, eosinophilic granular necrotic debris, and accumulation of macrophages, included 37 patients, allowing more insights into etiology. Few examples could be linked to adamantinomatous craniopharyngioma, and although ciliated epithelium similar to that of Rathke cleft cyst (RCC) was identified up to 35% of the 37 cases, it could not be proven that XG was related to hemorrhage into RCC. Case reports since that time, however, occasionally linked XG to RCC when an etiology could be identified at all, and a few recognized that a spectrum exists in xanthomatous lesions of the sella. They review literature, adding 23 cases from our own experience, to confirm that overlap occurs between XH and XG, and that the majority-but not all-can be linked to RCC leakage/rupture/hemorrhage. It was suggested that progressive accumulation of hemosiderin pigment in the lesion, possibly caused by the multiple episodes of bleeding, could account for the transition of at least some cases of XH to XG.
鞍区黄色瘤样病变传统上分为两个不同类别,即鞍区黄色瘤性垂体炎(XH)和鞍区黄色肉芽肿(XG)。关于XH的开创性文章详细介绍了3例患者,该病的典型特征是垂体/鞍区出现泡沫状组织细胞和淋巴细胞浆细胞浸润,但通常很少或没有含铁血黄素沉着。然而,自那时以来的大多数报告都是单个病例,这使得对该疾病的理解变得困难。相比之下,关于XG的开创性报告纳入了37例患者,其特征为鞍区胆固醇裂隙、淋巴细胞浆细胞浸润、明显的含铁血黄素沉积、纤维化、胆固醇裂隙周围的多核巨细胞、嗜酸性颗粒状坏死碎片以及巨噬细胞聚集,这使得对其病因有了更多了解。很少有病例与造釉细胞瘤性颅咽管瘤相关,尽管在37例病例中有高达35%的病例发现了类似于拉克氏囊肿(RCC)的纤毛上皮,但无法证实XG与RCC出血有关。然而,自那时以来的病例报告在能够确定病因时偶尔会将XG与RCC联系起来,少数报告认识到鞍区黄色瘤样病变存在一个谱系。他们回顾了文献,并补充了我们自己经验中的23例病例,以证实XH和XG之间存在重叠,且大多数(但不是全部)可与RCC渗漏/破裂/出血相关。有人提出,病变中含铁血黄素沉着的逐渐积累可能是由多次出血引起的,这可能解释了至少部分XH病例向XG的转变。