Abstract:
Juvenile Xanthogranuloma (XG) is a rare disorder that belongs to the heterogeneous group of histiocytic neoplasms, characterized by a clonal expansion of non-Langerhans cell histiocytes that share a dermal macrophage phenotype. Although the head and neck region is the most common reported site of involvement by the Juvenile Xanthogranuloma family, laryngeal localization is extremely rare. We report a unique case of Adult Onset Xanthogranuloma with subglottic localization, presenting as a solitary laryngeal mass without other systemic or cutaneous lesions. A review of the previously described cases of laryngeal Xanthogranuloma has been performed, highlighting 7 cases of Juvenile Xanthogranuloma and only 3 cases of Adult Onset Xanthogranuloma. Despite the extreme rarity of laryngeal localization of XG, this histiocytic neoplasm should be considered as a differential diagnosis for laryngeal masses causing airway obstruction, even in the absence of other concomitant manifestations.
摘要:
青少年黄色肉芽肿(XG)是一种罕见的疾病,属于组织细胞肿瘤的异质性组,特征在于共享真皮巨噬细胞表型的非朗格汉斯细胞组织细胞的克隆扩增。尽管头颈部是青少年黄色肉芽肿家族最常见的累及部位,喉定位极为罕见。我们报告了一个独特的病例,成人发作黄色肉芽肿,声门下定位,表现为单独的喉部肿块,没有其他全身或皮肤病变。已对先前描述的喉黄色肉芽肿病例进行了回顾,其中幼年性黄色肉芽肿7例,成人性黄色肉芽肿3例。尽管XG的喉定位极为罕见,这种组织细胞肿瘤应被视为引起气道阻塞的喉部肿块的鉴别诊断,即使没有其他伴随的表现。