Ayasa Laith A, Rahhal Sara, Najjar Ala'a K, Aldarawish Asad, Bakri Izzeddin A
Faculty of Medicine, Al-Quds University, Jerusalem, PSE.
School of Medicine, The University of Jordan, Amman, JOR.
Cureus. 2023 Nov 25;15(11):e49405. doi: 10.7759/cureus.49405. eCollection 2023 Nov.
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.
鞍区黄色肉芽肿是极其罕见的颅内病变，尤其在儿科患者中，其诊断和治疗挑战促使进行全面调查。我们描述了一例诊断为鞍区黄色肉芽肿的两岁幼儿病例，突出了其诊断和管理中遇到的挑战。该患儿出现了包括头痛、左眼上睑下垂和神经功能缺损等症状。脑部计算机断层扫描（CT）和磁共振成像（MRI）显示鞍区有低密度病变。患者接受了左翼点开颅手术以切除肿块。组织病理学检查提示鞍区黄色肉芽肿的诊断，其特征为泡沫状巨噬细胞、巨细胞、淋巴细胞浸润、纤维增生、坏死碎屑和含铁血黄素沉积。通过免疫组织化学染色，包括CD1a和朗格蛋白，进一步提高了诊断准确性，成功排除了朗格汉斯细胞组织细胞增多症（LCH）的可能性，强化了鞍区黄色肉芽肿的诊断。病变的成功手术切除带来了良好的结果，症状明显缓解以及神经功能恢复正常即为证明。术后评估显示患者生活质量显著改善，随访期间未观察到并发症或病变复发。总之，我们的病例报告不仅突出了鞍区黄色肉芽肿的罕见性和诊断挑战，还强调了多学科医学专业知识在儿科患者实现准确诊断和成功治疗结果方面的重要性。该病例的成功管理为鞍区黄色肉芽肿的临床表现、诊断复杂性和治疗策略提供了宝贵见解，进一步丰富了我们对这种罕见颅内病变的认识。
