Advertisement

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
  • 4 Downloads

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.

Keywords

Bone Lesion Unifocal Mandible Sphenoid Computed tomography 

Notes

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.

References

  1. 1.
    Krooks J, Minkov M, Weatherall AG. Langerhans cell histiocytosis in children: Diagnosis, differential diagnosis, treatment, sequelae, and standardized follow-up. J Am Acad Dermatol. 2018;78(6):1047–56.  https://doi.org/10.1016/j.jaad.2017.05.060.CrossRefPubMedGoogle Scholar
  2. 2.
    Lewoczko KB, Rohman GT, LeSueur JR, Stocks RM, Thompson JW. Head and neck manifestations of langerhan’s cell histiocytosis in children: A 46-year experience. Int J Pediatr Otorhinolaryngol. 2014;78(11):1874–6.  https://doi.org/10.1016/j.ijporl.2014.08.015.CrossRefPubMedGoogle Scholar
  3. 3.
    Histiocyte Society. Langerhans cell Histiocytosis: Histiocyte society evaluation and treatment guidelines. 2009.Google Scholar
  4. 4.
    Jahandideh H, Nasoori Y, Rostami S, Safdarian M. Laryngeal Langerhans cell Histiocytosis presenting with neck mass in an adult woman. Case Rep Otolaryngol. 2016.  https://doi.org/10.1155/2016/2175856.CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Berres M, Allen C, Merad M. Pathological consequence of misguided dendritic cell differentiation in histiocytic diseases. Adv Immunol. 2013;120:127.CrossRefGoogle Scholar
  6. 6.
    Majumder A, Wick CC, Collins R, Booth TN, Isaacson B, Kutz JW. Pediatric Langerhans cell histiocytosis of the lateral skull base. Int J Pediatr Otorhinolaryngol. 2017;99:135–40.  https://doi.org/10.1016/j.ijporl.2017.06.011.CrossRefPubMedGoogle Scholar
  7. 7.
    Minkov M. An update on the treatment of pediatric-onset Langerhans cell histiocytosis through pharmacotherapy. Expert Opin Pharmacother. 2018;19(3):233–42.  https://doi.org/10.1080/14656566.2018.1429405.CrossRefPubMedGoogle Scholar
  8. 8.
    Bezdjian A, Alarfaj AA, Varma N, Daniel SJ. Isolated Langerhans cell Histiocytosis Bone lesion in pediatric patients. Otolaryngol Head Neck Surg. 2015;153(5):751–7.  https://doi.org/10.1177/0194599815598969.CrossRefPubMedGoogle Scholar
  9. 9.
    Emile J‑F, et al. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood. 2016;127(22):2672–81.  https://doi.org/10.1182/blood-2016-01-690636.CrossRefPubMedPubMedCentralGoogle Scholar
  10. 10.
    Odame I, Li P, Lau L, Doda W, Noseworthy M, Babyn P, Weitzman Sh. Pulmonary Langerhans cell histiocytosis: a variable disease in childhood. Pediatr Blood Cancer. 2006;47:889.CrossRefGoogle Scholar
  11. 11.
    Aricò M, Girschikofsky M, Généreau T, Klersy C, McClain K, Grois N, Emile JF, Lukina E, De Juli E, Danesino C. Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society. Eur J Cancer. 2003;39:2341.CrossRefGoogle Scholar
  12. 12.
    Nicollas R, Rome A, Belaïch H, Roman S, Volk M, Gentet JC, Michel G, Triglia JM. Head and neck manifestation and prognosis of Langerhans cell histiocytosis in children. Int J Pediatr Otorhinolaryngol. 2010;74(6):669–73.CrossRefGoogle Scholar
  13. 13.
    Li Z, Li Z, Zhang W, Li JR, Wang SP, Cheng Y, Wei M. Eosinophilic granuloma of the jaws: An analysis of clinical and radiographic presentation. Oral Oncol. 2006;42:574–80.CrossRefGoogle Scholar
  14. 14.
    Modest MC, Garcia JJ, Arndt CS, Carlson ML. Langerhans cell histiocytosis of the temporal bone: A review of 29 cases at a single center. Laryngoscope. 2016;126(8):1899–904.CrossRefGoogle Scholar
  15. 15.
    Minkov M, Grois N, McClain K, Nanduri V, Rodríguez-Galindo C, Simonitsch-Klupp I, Visser J, Weitzman Sh, Whitlock J, Windebank K. Langerhans cell histiocytosis. Evaluation and treatment guidelines. 2009. https://histiocytesociety.org/document.doc?id=290. Accessed 20 Aug 2016.Google Scholar
  16. 16.
    Jubran RF, Marachelian A, Dorey F, Malogolowkin M. Predictors of outcome in children with Langerhans cell histiocytosis. Pediatr Blood Cancer. 2005;45:37.CrossRefGoogle Scholar

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

Personalised recommendations