Unisystem Langerhans cell histiocytosis in maxillofacial region in pediatrics: comprehensive and systematic review
- 16 February 2021
- journal article
- review article
- Published by Springer Science and Business Media LLC in Oral and Maxillofacial Surgery
- Vol. 25 (4), 429-444
- https://doi.org/10.1007/s10006-021-00949-9
Abstract
Background The study aimed to identify, enlist, and analyze cases of unisystem LCH in the maxillofacial pediatric population to understand the clinical presentation and encourage the consideration of this rare disease in the differential diagnosis. Langerhans cell histiocytosis (LCH) is an aggressive benign condition affecting mainly the pediatric population. It can be easily masked as periodontal disease in the maxillofacial region. Early diagnosis and a systemic evaluation are of utmost importance. Methodology We are presenting a complete review of literature in the pediatric population according to PRISMA guidelines for clinicopathologic, histopathological, immunohistochemistry, and treatment for unisystem LCH. The risk of bias assessment across studies was done using a Case series appraisal checklist by Guo et al. 53 Results Forty-nine articles (152 cases) were selected which met our inclusion and exclusion criteria to be included in our review. Most of the patients fall in 6–12 years of age with the involvement of the mandibular body region in 40.79% cases. This disease mainly presents as erythematous gingiva, pain, swelling, and mobile teeth. Management can range from minimal intervention to chemotherapy and surgery. Conclusion and practical implications Although this is a rare condition, it should be considered especially in the pediatric population with periodontitis type lesions and floating teeth and comprehensive management should be followed. Early diagnosis of the disease is very important.Keywords
This publication has 76 references indexed in Scilit:
- Intralesional infiltration of corticosteroids in the treatment of localized Langerhans cell histiocytosis of the mandible: report of two casesOral Surgery, Oral Medicine, Oral Pathology and Oral Radiology, 2013
- Eosinophilic Granuloma of Mandibular Condyle: Resection and Complete RegenerationJournal of Maxillofacial and Oral Surgery, 2013
- Skeletal involvement in Langerhans cell histiocytosisInsights into Imaging, 2013
- Does Adjunctive Chemotherapy Reduce Remission Rates Compared to Cortisone Alone in Unifocal or Multifocal Histiocytosis of Bone?Clinical Orthopaedics and Related Research, 2012
- Clinico-Pathologic Conference: Case 4Head and Neck Pathology, 2010
- Recurrent BRAF mutations in Langerhans cell histiocytosisBlood, 2010
- Intralesional corticosteroid therapy for mandibular Langerhans cell histiocytosis preserving the intralesional tooth germOral and Maxillofacial Surgery, 2008
- Descriptive epidemiology of childhood Langerhans cell histiocytosis in France, 2000–2004Pediatric Blood & Cancer, 2008
- Intralesional infiltration of corticosteroids in the treatment of localised langerhans’ cell histiocytosis of the mandible: Report of known cases and three new casesInternational Journal of Oral & Maxillofacial Surgery, 2005
- Eosinophilic granuloma of the palatal mucosa in a nine‐week‐old infantMedical and Pediatric Oncology, 1981