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Abstract：
Sellar xanthogranulomas are extremely rare intracranial lesions, particularly in pediatric patients, and their diagnostic and therapeutic challenges prompt thorough investigation. We describe a case of a two-year-old toddler diagnosed with sellar xanthogranuloma, highlighting the challenges encountered in its diagnosis and management. The child presented with symptoms, including headache, ptosis of the left eye, and neurological deficits. Brain computed tomography (CT) and magnetic resonance imaging (MRI) revealed a hypodense sellar lesion. The patient underwent a left pterional craniotomy for resection of the mass. Histopathological examination suggested the diagnosis of sellar xanthogranuloma, characterized by foamy macrophages, giant cells, lymphocytic infiltrates, fibrous proliferation, necrotic detritus, and hemosiderin deposits. Further diagnostic precision was achieved through immunohistochemical staining, including CD1a and langerin, which successfully ruled out the possibility of Langerhans cell histiocytosis (LCH), reinforcing the diagnosis of sellar xanthogranuloma. The successful surgical resection of the lesion led to a favorable outcome, evidenced by the significant alleviation of symptoms as well as the restoration of normal neurological function. Post-operative assessments demonstrated a marked improvement in the patient\'s quality of life, and there were no observed complications or recurrence of the lesion during the follow-up period. In summary, our case report not only highlights the rarity and diagnostic challenges of sellar xanthogranulomas but also emphasizes the importance of collaborative medical expertise in achieving accurate diagnosis and successful therapeutic outcomes in pediatric patients. The successful management of this case offers valuable insights into the clinical presentation, diagnostic complexities, and treatment strategy of sellar xanthogranulomas, further enriching our understanding of this uncommon intracranial pathology.
摘要：
鞍区黄色肉芽肿是极其罕见的颅内病变，特别是在儿科患者中，他们的诊断和治疗挑战促使彻底的调查。我们描述了一个被诊断患有鞍状黄色肉芽肿的两岁幼儿的病例，强调其诊断和管理中遇到的挑战。孩子出现了症状，包括头痛,左眼的眼睑下垂，和神经缺陷。脑计算机断层扫描（CT）和磁共振成像（MRI）显示鞍区低密度病变。患者接受了左侧翼点开颅手术切除肿块。组织病理学检查提示诊断为鞍状黄色肉芽肿，以泡沫巨噬细胞为特征，巨细胞,淋巴细胞浸润，纤维增生，坏死的碎屑，和含铁血黄素的沉积物.通过免疫组织化学染色进一步达到诊断精度，包括CD1a和langerin，成功排除了朗格汉斯细胞组织细胞增生症（LCH）的可能性，加强鞍区黄色肉芽肿的诊断。手术切除病灶的成功导致了良好的结果，症状的显着缓解以及正常神经功能的恢复都证明了这一点。术后评估显示患者的生活质量有显著改善，随访期间未观察到并发症或病灶复发。总之,我们的病例报告不仅突出了鞍状黄色肉芽肿的罕见性和诊断挑战,而且还强调了协作医疗专业知识在儿科患者中实现准确诊断和成功治疗结果的重要性.该病例的成功治疗为临床表现提供了宝贵的见解，诊断复杂性，鞍区黄色肉芽肿的治疗策略，进一步丰富了我们对这种罕见的颅内病理的理解。
