伴口腔颌面部受累的朗格罕细胞组织细胞增生症12例临床分析

Langerhans cell histiocytosis involving the oral and maxillofacial region: an analysis of 12 cases

目的探讨伴口腔颌面部受累的朗格罕细胞组织细胞增生症(LCH)的临床特点。方法回顾性分析2012年9月至2017年9月期间住院治疗的12例伴口腔颌面部受累LCH患儿的临床资料,分析其临床表现、病理学特征、治疗及预后。结果 12例伴口腔颌面部受累的LCH患儿中,8例(67%)伴有多系统受累,7例(58%)伴危险器官受累。受累部位以骨最常见(11例,92%),主要累及下颌骨(7例)。口腔软组织受累表现为牙龈溃疡或增生(4例)、活动牙齿(5例)、口腔黏膜病变(2例)及结节性病变(1例)。病理检查11例CDla阳性,12例CD207、CD68、S-100、LCA均阳性。治疗以手术联合化疗为主,局灶病变可单纯手术切除。痊愈或好转11例,1例放弃治疗并失访,均未见复发。结论伴口腔颌面部受累LCH患儿易合并多系统受累及危险器官受累,最常累及下颌骨,并伴有牙龈、口腔黏膜、牙齿受累。治疗以手术联合化疗为主,预后良好,不易复发。[中国当代儿科杂志,2019,21(5):415-420]

Objective To study the clinical features of Langerhans cell histiocytosis(LCH)involving the oral and maxillofacial region in children.Methods A retrospective analysis was performed for the clinical data of 12 children with LCH involving the oral and maxillofacial region who were hospitalized and treated from September 2012 to September 2017,including clinical manifestations,pathological features,treatment and prognosis.Results Of the 12 children,8(67%)had multiple system involvement and 7(58%)had the involvement of organs at risk.Bone was the most common affected site(11 children,92%),among whom 7 children had the involvement of the mandible.Oral soft tissue involvement manifested as gingival ulcer or hyperplasia in 4 children,loose teeth in 5 children,oral mucosal lesions in 2 children,and nodular lesions in 1 child.Pathological examination showed positive CDla in 11 children and positive CD207,CD68,S-100,and LCA in 12 children.Surgery combined with chemotherapy was the major treatment method,and surgical resection alone was performed for focal lesions.After treatment,11 children were cured or improved and 1 gave up treatment and was lost to follow-up.No recurrence was observed.Conclusions LCH children with oral and maxillofacial involvement often have the involvement of multiple systems and organs at risk,with the mandible as the most common affected site.These children may also have the involvement of gingiva,oral mucosa and teeth.Surgery combined with chemotherapy is the major treatment method,and the patients generally have a good prognosis without recurrence.[Chin J Contemp Pediatr,2019,21(5):415-420]