Langerhans cell histiocytosis: Case series and literature review

• Autores: Cardeña-Mamani, Rakel, Arauco-Nava, Pedro, Villena-Pairazaman, Fermín Felipe, Mejía-Pelaez, Guillermo, Flores-Alvarez, Willys
• Formato: artículo
• Fecha de Publicación: 2021
• Institución: Cuerpo Médico Hospital Nacional Almanzor Aguinaga Asenjo
• Repositorio: Revista del Cuerpo Médico Hospital Nacional Almanzor Aguinaga Asenjo
• Lenguaje: español
• OAI Identifier: oai:cmhnaaa_ojs_cmhnaaa.cmhnaaa.org.pe:article/1067
• Enlace del recurso: https://cmhnaaa.org.pe/ojs/index.php/rcmhnaaa/article/view/1067
• Nivel de acceso: acceso abierto
• Materia: histiocitosis; células de Langerhans; cráneo; Informes de Casos; Niño; Neoplasias; histiocytosis; Langerhans cells; skull; Case Reports; Child; Neoplasms

Background: Langerhans cells histiocytosis is reported in approximately three out of every million children. It is of unknown origin, considered a clonal neoplasm, with a BRAF, RAS mutation, in the myeloid differentiation; depending on its presentation, both prognosis and survival are variable. Cases report: Three cases of patients aged 10, 11 and 9 years, diagnosed with Langerhans cell histiocytosis at an early age and followed-up are presented. Two of them had cranial tumors, and the remaining one with lesions in the femur and humerus, which progressed from unifocal unisystemic disease to multifocal, multisystemic disease. Conclusion: Langerhans cells histiocytosis should be considered in the differential diagnoses of rapidly progressing cranial tumors and bone tumors in childhood, due to its high frequency of bone involvement. The follow-up was performed by oncology, and management with the classification of multisystemic involvement.