Langerhan’s cell histiocytosis with oral manifestation in a 3-year-old child: a case report
DOI:
https://doi.org/10.33448/rsd-v10i5.15136Keywords:
Langerhans cell histiocytosis; Maxillofacial surgery; Oral pathology; Pediatric tumors.Abstract
Langerhans cell histiocytosis (LCH) is a disorder that may affect the bones, skin, liver, lung, and hematopoietic and neuroendocrine systems. This condition may manifest as a single lesion, multiple lesions, or as a disseminated and potentially fatal disease. We aim to report a case of a 3-year-old child with LCH in the mandible, sharing with the readers the challenging process of this diagnosis. A three-year-old male patient with persistent swelling in the right submandibular region was referred to the Department of Pediatrics of the Erasto Gaertner Hospital for an evaluation. Initial physical exam revealed a diffuse flaccid swelling occupying the entire right mandibular ramus, from the angle to the preauricular region and CT scan showed an osteolytic lesion with erosion of the internal and external cortices of the mandible and an extension to soft tissues that displaced the masseter muscle. Immunohistochemical analysis confirmed the diagnosis of Langerhans cell histiocytosis through positive tests for CD1a, CD68, S-100, and Vimentin. The treatment proposed was a combination of Vinblastine 6 mg/m3 for 6 weeks and Prednisone 40mg for 4 weeks.The differential diagnosis included pathologies such as rhabdomyosarcoma, Ewing's sarcoma, and, less likely, osteosarcoma and central giant cell granuloma.
References
Abla, O., Rollins, B., & Ladisch, S. (2019). Langerhans cell histiocytosis: progress and controversies. British Journal of Haematology. 187(5), 559-562.
Allen, C. E., Merad, M., & McClain, K. L. (2018). Langerhans-Cell Histiocytosis. New England Journal of Medicine, 379(9), 856–868.
Annibali, S., Cristalli, M., Solidani, M., Ciavarella, D., La Monaca, G., Suriano, M. & Lo Russo, L. (2009). Langerhans cell histiocytosis: oral/periodontal involvement in adult patients. Oral Diseases, 15(8), 596–601.
Claire, K., Bunney, R., Ashack, K. A., Bain, M., Braniecki, M., & Tsoukas, M. M. (2020). Langerhans Cell Histiocytosis: A greater imitator. Clin in Dermatology, 38 (2), 223-234.
Davidson, L. E., Soldani, F. A., & North, S. (2006). Blackwell Publishing Ltd Rhabdomyosarcoma of the mandible in a 6-year-old boy. International Journal of Paediatric Dentistry, 16(4), 302–306.
Donadieu, J., Chalard, F., & Jeziorski, E. (2012). Medical management of Langerhans cell histiocytosis from diagnosis to treatment. Expert Opin Pharmacother, 13(1), 1309–1322.
Guyot-Goubin, A., Donadieu, J., Barkaoui, M., Bellec, S., Thomas, C., & Clavel, J. (2008). Descriptive epidemiology of childhood Langerhans Cell Histiocytosis in France, 2000-2004. Pediatr Blood Cancer, 51(1), 71-75.
Heare, T., Hensley, M. A., & Dell' Orfano, S. (2009). Bone tumors: osteosarcoma and Ewing’s sarcoma. Current opinion in pediatrics, 21(3), 365-372.
Kontio, R., Hagstrom, J., Lindholm, P., Bohling, T., Sampo, M., Mesimaki, K., Saarilahti, K., Koivunen, P., & Makitie, A. A. (2019). Craniomaxillofacial osteosarcoma – The role of surgical margins. Journal of Cranio-Maxillo-Facial Surgery, 47(6), 922-925.
Krooks, J., Minkov, M., & Weatherall, A. G. (2018). Langerhans cell histiocytosis in children: History, classification pathobiology, clinical manifestations and prognosis. J Am Acad Dermatol, 78(6), 1035-1044.
Leung, A. K. C., Lam, J. M., & Leong, K. F. (2019). Childhood Langerhans cell histiocytosis: a disease with many faces. World Journal of Pediatrics, 15(6), 536-545.
Merglová, V., Hrusak, D., Boudová, L., Mukensnabl, P., Valentová, E., & Hosticka, L. (2014). Langerhans cell histiocytosis in childhood – Review, symptoms in the oral cavity, differential diagnosis and report of two cases. J Cranio-Maxillofac Surg, 42(2), 93-100.
Oliveira, S. V., Fernandes, L. G., Soares Jr, L. A. V., Moraes, M. F., Almeida, M. T. A., Pinto Jr, D. S., & Alves, F. A. (2019). Mandible Ewing Sarcoma in a child: Clinical, radiographic and diagnosis considerations. Oral Oncology, 98, 171-173.
Papo, M., Cohen-Aubart, F., Trefond, L., Bauvois, A., Amoura, Z., Emile, J.F., & Haroche, J. (2019). Systemic Histiocytosis (Langerhans Cell Histiocytosis, Erdheim–Chester Disease, Destombes–Rosai–Dorfman Disease): from Oncogenic Mutations to Inflammatory Disorders. Current Oncology Reports, 21(7),62.
Peters, S. M., Pastagia, J., Yoon, A. J., & Philipone, E. M. (2017). Langerhans cell histiocytosis mimicking periapical pathology in a 39-year-old man. J Endod, 43(11), 1909-1914.
Postini, A. M., Prever, A. B., Pagamo, M., Rivetti, E., Berger, M., Asaftei, S. D., Barat, V., Andreacchio, A., & Fagioli, F. (2012). Langerhans cell histiocytosis: 40 year's experience. In J Pediatric Hema‐tolol Oncol, 34(5), 353‐8.
Rodriguez-Galindo, C., & Allen, C. E. (2020). Langerhans Cell histiocytosis. Blood, 135(16), 1319-1331.
Silva, R. N. F., Tino, M. T., Costa, N. L., Batista, A, C., Mendonça, E. F., & Ribeiro-Rotta, R. F. (2018). Central Giant Cell Lesion Mimicking Osteosarcoma. Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology., 126(3): e100
Tenorio, J. R., Esteves, C. V., Heguedusch, D., Sousa, S. C. O. M., & Lemos-Júnior, C. A. (2020). Oral and cutaneous manifestations of langerhans cell histiocytosis: report of two cases. J Oral Maxillofac Surge Med Pathol, 32(1), 72-75.
Wang, Y., Le, A., El Demellawy, D., Shago, M., Odell, M., & Johnson-Obaseki, S. (2019). An aggressive central giant cell granuloma in a pediatric patient: case report and review of literature. J of Otolaryngol: Head & Neck Surg, 48(1), 32.
Weitzman, S. & Egeler, R. M. (2008). Langerhans cell histiocytosis: update for the pediatrician. Curr Opin Pediatr. 20(1),23–29.
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2021 Joana Leticia Vendruscolo; Bruna da Fonseca Wastner; Cleverson Patussi; Sergio Ossamu Ioshii; Juliana Lucena Schussel; Mara Albonei Dudeque Pianovski; Laurindo Moacir Sassi
This work is licensed under a Creative Commons Attribution 4.0 International License.
Authors who publish with this journal agree to the following terms:
1) Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
2) Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
3) Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work.
