Head and neck Langerhans cell histiocytosis: two case reports and review of the literature
Autor: | Nadia Inostroza, J. Patricio Ulloa, Romina Novoa, Francisco Rivas-Rodriguez, Ignacio Mendoza, Ilson Sepúlveda, Geraldine Ayres |
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Rok vydání: | 2019 |
Předmět: |
Chemotherapy
Pathology medicine.medical_specialty Myeloid business.industry medicine.medical_treatment Mandible Hematology medicine.disease Work-up 03 medical and health sciences Skull 0302 clinical medicine medicine.anatomical_structure Oncology Langerhans cell histiocytosis 030220 oncology & carcinogenesis Precursor cell medicine Lymph business 030215 immunology |
Zdroj: | memo - Magazine of European Medical Oncology. 12:278-283 |
ISSN: | 1865-5076 1865-5041 |
DOI: | 10.1007/s12254-019-0500-y |
Popis: | Langerhans cell histiocytosis (LCH) is an inflammatory neoplasia of myeloid precursor cells driven by mutations in the mitogen-activated protein kinase (MAPK) pathway. It can present as single or multisystem LCH. It occurs in 0.5–5.4 per million people. While it can occur at any age, it is more common in children, predominantly males, under the age of 4 years. The unifocal single system variety is the most prevalent and least aggressive presentation, making up to 70% of cases. It can affect bone (80%), lymph nodes (5–10%) or lungs (15%). The mandible and sphenoid are the least common locations; therefore, when present, it could be easily mistaken for a variety of pathologies. Depending on the clinical features, treatment can range from simple observation to chemotherapy and/or radiation. We present 2 cases of unifocal single system LCH located at the mandible and skull base, and describe the diagnostic work up and treatment. |
Databáze: | OpenAIRE |
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