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Head and neck Langerhans cell histiocytosis: two case reports and review of the literature

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Summary

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.

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Authors and Affiliations

  1. Radiology Department, Otorhinolaryngology-Head and Neck Surgery; Maxillofacial Services; General Hospital of Concepción, Finis Terrae University School of Dentistry, San Martin Av. 1436, Concepción, Chile

    Ilson Sepúlveda

  2. Otorhinolaryngology Department, Air Force Clinical Hospital, Santiago, Chile

    Ignacio Mendoza

  3. ENT-Head and Neck Surgery Service, General Hospital of Concepcion, Resident University of Concepción School of Medicine, Concepción, Chile

    Romina Novoa

  4. Physician Pathologist, Pathology Department, General Hospital of Concepción, University of Concepción School of Medicine, Concepción, Chile

    Geraldine Ayres

  5. Pathology Department, General Hospital of Concepción, Resident University of Concepción School of Medicine, Concepción, Chile

    Nadia Inostroza

  6. Otorhinolaryngology-Head and Neck Surgery, General Hospital of Concepcion, University of Concepción School of Medicine, Concepción, Chile

    J Patricio Ulloa

  7. Radiology Department, Division of Neuroradiology, University of Michigan Health System, Ann Arbor, MI, USA

    Francisco Rivas-Rodriguez

Authors
  1. Ilson Sepúlveda
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  2. Ignacio Mendoza
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  3. Romina Novoa
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  4. Geraldine Ayres
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  5. Nadia Inostroza
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  6. J Patricio Ulloa
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  7. Francisco Rivas-Rodriguez
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Corresponding author

Correspondence to Ilson Sepúlveda.

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Conflict of interest

I. Sepúlveda, I. Mendoza, R. Novoa, G. Ayres, N. Inostroza, J.P. Ulloa, and F. Rivas-Rodriguez declare that they have no competing interests.

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Sepúlveda, I., Mendoza, I., Novoa, R. et al. Head and neck Langerhans cell histiocytosis: two case reports and review of the literature. memo 12, 278–283 (2019). https://doi.org/10.1007/s12254-019-0500-y

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  • DOI: https://doi.org/10.1007/s12254-019-0500-y

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