Yağci Begül, Kandemir Nurgün, Yazici Nalan, Yalçin Bilgehan, Varan Ali, Akyüz Canan, Büyükpamukçu Münevver
Department of Pediatric Oncology, Hacettepe University Institute of Oncology, Ankara, Turkey.
Eur J Pediatr. 2007 Sep;166(9):901-4. doi: 10.1007/s00431-007-0487-y. Epub 2007 Apr 19.
Langerhans cell histiocytosis (LCH) is a disorder with unclear etiology and pathogenesis characterized by abnormal clonal proliferation and accumulation of antigen presenting Langerhans' cells at various tissues and organs. Almost all organs or systems may be involved, and the prognosis depends on the involved sites and the presence of organ dysfunction. Thyroid tissue is a rarely affected site in children, and without histopathological evaluation it may be difficult to distinguish from other thyroid disorders because of the similar physical examination, laboratory and imaging findings. Here we report on two patients with histopathologically proven thyroid involvement of LCH. Additionally, the differential diagnosis of diffusely enlarged or multinodular thyroid glands in children is discussed, and a review of the literature of thyroid involvement in LCH is given. In the differential diagnosis of enlarged thyroid glands, especially in the presence of other endocrinological manifestations, LCH must be taken into consideration.
朗格汉斯细胞组织细胞增多症(LCH)是一种病因和发病机制不明的疾病,其特征是抗原呈递朗格汉斯细胞在各种组织和器官中异常克隆增殖和积聚。几乎所有器官或系统都可能受累,预后取决于受累部位及器官功能障碍的存在情况。甲状腺组织是儿童中很少受累的部位,由于体格检查、实验室检查及影像学表现相似,在没有组织病理学评估的情况下,很难与其他甲状腺疾病相鉴别。在此,我们报告两例经组织病理学证实甲状腺受累的LCH患者。此外,还讨论了儿童弥漫性甲状腺肿大或多结节性甲状腺肿的鉴别诊断,并对LCH甲状腺受累的文献进行了综述。在甲状腺肿大的鉴别诊断中,尤其是存在其他内分泌表现时,必须考虑到LCH。