Hwang Ye Rin, Lee Kyung Mi, Kim Hyug-Gi, Na Kiyong
Department of Radiology, Kyung Hee University College of Medicine, Kyung Hee University Hospital, #23 Kyunghee-daero, Dongdaemun-gu, Seoul 02447, Korea.
Department of Pathology, Kyung Hee University College of Medicine, Kyung Hee University Hospital, #23 Kyunghee-daero, Dongdaemun-gu, Seoul 02447, Korea.
Diagnostics (Basel). 2022 Feb 3;12(2):400. doi: 10.3390/diagnostics12020400.
Langerhans cell histiocytosis (LCH) is a rare neoplastic disorder characterized by the clonal proliferation of CD1a +/CD 207 + dendritic cells, whose features are similar to those of epidermal Langerhans cells. LCH is more common in children than in adults. Localized osteolytic lesions in the craniofacial bones are the most common manifestations of LCH. However, LCH can also present as a multifocal and multisystem disease with poor prognosis. Locally aggressive LCH needs to be differentiated from various diseases such as osteomyelitis, malignant bone tumors, and soft tissue sarcomas. However, it is difficult to diagnose, since the imaging findings are nonspecific. We report a case of a highly aggressive LCH in the maxilla accompanied by a fluid-fluid level.
朗格汉斯细胞组织细胞增多症（LCH）是一种罕见的肿瘤性疾病，其特征为CD1a +/CD 207 +树突状细胞的克隆性增殖，这些细胞的特征与表皮朗格汉斯细胞相似。LCH在儿童中比在成人中更常见。颅面骨的局部溶骨性病变是LCH最常见的表现。然而，LCH也可表现为多灶性和多系统疾病，预后较差。局部侵袭性LCH需要与骨髓炎、恶性骨肿瘤和软组织肉瘤等各种疾病相鉴别。然而，由于影像学表现不具有特异性，因此诊断困难。我们报告一例上颌骨高度侵袭性LCH伴液-液平面的病例。
