Abstract：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.
WANG Ying-Chao,LI Zhuang-Zhuang,YIN Chu-Yun et al. Langerhans cell histiocytosis involving the oral and maxillofacial region: an analysis of 12 cases[J]. CJCP, 2019, 21(5): 415-420.
Berres ML, Merad M, Allen CE. Progress in understanding the pathogenesis of Langerhans cell histiocytosis:back to Histiocytosis X?[J]. Br J Haematol, 2015, 169(1):3-13.
[3]
Anastasilakis AD, Tsoli M, Kaltsas G, et al. Bone metabolism in Langerhans cell histiocytosis[J]. Endocr Connect, 2018, 7(7):R246-R253.
[4]
Papadopoulou M, Panagopoulou P, Papadopoulou A, et al. The multiple faces of Langerhans cell histiocytosis in childhood:a gentle reminder[J]. Mol Clin Oncol, 2018, 8(3):489-492.
[5]
Luz J, Zweifel D, Hüllner M, et al. Oral manifestation of Langerhans cell histiocytosis:a case report[J]. BMC Oral Health, 2018, 18(1):106.
[6]
Neves-Silva R, Fernandes DT, Fonseca FP, et al. Oral manifestations of Langerhans cell histiocytosis:a case series[J]. Spec Care Dentist, 2018, 38(6):426-433.
[7]
Abla O, Egeler RM, Weitzman S. Langerhans cell histiocytosis:current concepts and treatments[J]. Cancer Treat Rev, 2010, 36(4):354-359.
Erker C, Harker-Murray P, Talano JA. Usual and unusual manifestations of familial hemophagocytic lymphohistiocytosis and Langerhans cell histiocytosis[J]. Pediatr Clin North Am, 2017, 64(1):91-109.
[10]
Saliba I, Sidani K, El Fata F, et al. Langerhans' cell histiocytosis of the temporal bone in children[J]. Int J Pediatr Otorhinolaryngol, 2008, 72(6):775-786.
[11]
Stålemark H, Laurencikas E, Karis J, et al. Incidence of Langerhans cell histiocytosis in children:a population-based study[J]. Pediatr Blood Cancer, 2008, 51(1):76-81.
[12]
Bedran NR, Carlos R, de Andrade BAB, et al. Clinicopathological and immunohistochemical study of head and neck Langerhans cell histiocytosis from Latin America[J]. Head Neck Pathol, 2018, 12(4):431-439.
[13]
Halperson E, Weintraub M. Oral Langerhans cell histiocytosis in an infant[J]. J Dent Child (Chic), 2018, 85(2):75-78.
[14]
Merglová V, Hrusák D, Boudová L, et al. Langerhans cell histiocytosis in childhood-review, symptoms in the oral cavity, differential diagnosis and report of two cases[J]. J Craniomaxillofac Surg, 2014, 42(2):93-100.
[15]
Carrera Silva EA, Nowak W, Tessone L, et al. CD207+CD1a+ cells circulate in pediatric patients with active Langerhans cell histiocytosis[J]. Blood, 2017, 130(17):1898-1902.
[16]
Lau SK, Chu PG, Weiss LM, et al. Immunohistochemical expression of Langerin in Langerhans cell histiocytosis and non-Langerhans cell histiocytic disorders[J]. Am J Surg Pathol, 2008, 32(4):615-619.
