PDF(Pubmed)
Abstract：
Xanthogranulomas are considered rare tumors, with their sellar and non-sellar frequency ranging from 1.6 to 7% among intracranial lesions, and described as a separate entity by the World Health Organization in 2000. The diagnosis of sellar xanthogranulomas is challenging, given their uncertain origin and clinical course. In addition, the limited reporting of sellar xanthogranuloma cases and the absence of characteristic images make these entities difficult to distinguish from other cystic lesions of the sellar region, such as adamantinomatous craniopharyngiomas, Rathke\'s cleft cysts, pituitary tumors, arachnoid cysts, epidermoid cysts, and dermoid cysts. Here, we describe the clinical presentation, radiological findings, immunohistochemical/histopathological analysis, and the ultrastructural examination by transmission electron microscopy of five sellar xanthogranulomas cases reported in two care centers in Cordoba, Argentina. Two males and three females between 37 and 73 years of age (average 51.8 years) presented with persistent headaches, generalized endocrine defects, and visual problems. MRI revealed cystic formations in the sellar region, which usually projected into adjacent tissues such as the suprasellar region or cavernous sinuses, and compressed other structures such as the optic chiasm, pituitary gland, and cranial nerves. All patients underwent surgical intervention to remove the tumor tissue. The histopathological analysis of the samples showed cellular tissue with a xanthogranulomatous appearance, inflammatory cellular infiltrate (mainly lymphocytes and macrophages), fibroblasts, abundant collagen fibers, and hemorrhages. An ultrastructural analysis helped to identify cellular infiltrates and granules resulting from tumor cell activity. The data support the hypothesis that sellar xanthogranulomas could occur as an inflammatory reaction secondary to the rupture and hemorrhage of a previous cystic process, thereby generating an expansion of the tumor body toward adjacent tissues. The information obtained from these cases contributes to the current knowledge about this disease\'s origin and clinical and histological evolution. However, the scarcity of patients and the observed phenotypic heterogeneity make its diagnosis still challenging. Undoubtedly, more investigations are needed to provide additional information in order to be able to achieve a more accurate diagnosis and effective treatment of this rare disease.
摘要：
黄色肉芽肿被认为是罕见的肿瘤，在颅内病变中，鞍区和非鞍区的频率范围为1.6％至7％，并在2000年被世界卫生组织描述为一个独立的实体。鞍区黄色肉芽肿的诊断具有挑战性，鉴于其来源和临床过程不确定。此外,鞍区黄色肉芽肿病例报告有限，缺乏特征性图像，使得这些实体难以与鞍区的其他囊性病变区分开来，如金刚瘤颅咽管瘤，Rathke的裂隙囊肿,垂体瘤,蛛网膜囊肿,表皮样囊肿,和皮样囊肿.这里,我们描述了临床表现，放射学发现，免疫组织化学/组织病理学分析，通过透射电子显微镜对科尔多瓦两个护理中心报告的5例鞍状黄色肉芽肿病例进行超微结构检查，阿根廷。年龄在37至73岁（平均51.8岁）之间的两名男性和三名女性出现持续头痛，全身性内分泌缺陷，和视觉问题。MRI显示鞍区囊性形成,通常投射到邻近组织，如鞍上区或海绵窦，并压缩其他结构，如视交叉，脑垂体,和颅神经。所有患者均接受手术干预以切除肿瘤组织。样品的组织病理学分析显示细胞组织具有黄色肉芽肿样外观，炎性细胞浸润（主要是淋巴细胞和巨噬细胞），成纤维细胞,丰富的胶原纤维，和出血。超微结构分析有助于鉴定由肿瘤细胞活性引起的细胞浸润和颗粒。数据支持以下假设：鞍状黄色肉芽肿可作为先前囊性过程破裂和出血的继发炎症反应而发生。从而产生肿瘤体向邻近组织的扩张。从这些病例中获得的信息有助于目前对这种疾病的起源以及临床和组织学演变的了解。然而,患者的稀缺性和观察到的表型异质性使其诊断仍然具有挑战性.毫无疑问,需要更多的调查来提供更多信息，以便能够更准确地诊断和有效治疗这种罕见疾病。
