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

  • Ilson SepúlvedaEmail author
  • Ignacio Mendoza
  • Romina Novoa
  • Geraldine Ayres
  • Nadia Inostroza
  • J Patricio Ulloa
  • Francisco Rivas-Rodriguez
case report


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.


Bone Lesion Unifocal Mandible Sphenoid Computed tomography 


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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Copyright information

© Springer-Verlag GmbH Austria, part of Springer Nature 2019

Authors and Affiliations

  • Ilson Sepúlveda
    • 1
    Email author
  • Ignacio Mendoza
    • 2
  • Romina Novoa
    • 3
  • Geraldine Ayres
    • 4
  • Nadia Inostroza
    • 5
  • J Patricio Ulloa
    • 6
  • Francisco Rivas-Rodriguez
    • 7
  1. 1.Radiology Department, Otorhinolaryngology-Head and Neck Surgery; Maxillofacial Services; General Hospital of ConcepciónFinis Terrae University School of DentistryConcepciónChile
  2. 2.Otorhinolaryngology DepartmentAir Force Clinical HospitalSantiagoChile
  3. 3.ENT-Head and Neck Surgery Service, General Hospital of ConcepcionResident University of Concepción School of MedicineConcepciónChile
  4. 4.Physician Pathologist, Pathology Department, General Hospital of ConcepciónUniversity of Concepción School of MedicineConcepciónChile
  5. 5.Pathology Department, General Hospital of ConcepciónResident University of Concepción School of MedicineConcepciónChile
  6. 6.Otorhinolaryngology-Head and Neck Surgery, General Hospital of ConcepcionUniversity of Concepción School of MedicineConcepciónChile
  7. 7.Radiology Department, Division of NeuroradiologyUniversity of Michigan Health SystemAnn ArborUSA

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