Langerhans cell histiocytosis of the temporal bones: a clinical case

DOI: https://doi.org/10.29296/25877305-2022-01-13
Download full text PDF
Issue: 
1
Year: 
2022

Professor Kh. Diab, MD; Corresponding Member of the Russian Academy of Sciences, Professor N.
Daikhes, MD; V. Saidulaev, Candidate of Medical Sciences; Professor A. Yunusov, MD; O. Pashinina, Candidate
of Medical Sciences; A. Mikhalevich, Candidate of Medical Sciences National Medical Research Center of
Otorhinolaryngology, Federal Biomedical Agency of Russia, Moscow

Langerhans cell histiocytosis (LCH) is a rare disease with an incidence of 1 to 9 cases per 1,000,000 population per year. From 75 to 90% of cases of the disease occur in childhood. The etiology of the disease is unknown. Pathogenetically, the disease is characterized by a clonal proliferation of Langerhans cells – the lymphoid dendritic cells originating from the bone marrow have an antigen-presenting function outside the dermis. The temporal bones are affected in 25-30% of cases. The disease is more common in males. The peak incidence occurs in the first 3 years of life. The disease can be asymptomatic for a long time or be masked as other common middle ear pathologies (chronic suppurative otitis media, mastoiditis, and otitis externa). The article describes a clinical case of temporal bone LCH. The feature of the clinical case in the recurrent course of the disease is that it is masked as acute mastoiditis.
Keywords: 
orphan diseases
Langerhans cell histiocytosis
temporal bone
computed tomography

It appears your Web browser is not configured to display PDF files. Download adobe Acrobat или click here to download the PDF file.

References: 
  1. Dolgov V.A., Anikin M.I., Gorbaneva G.A. i dr. Sluchaj obnaruzhenija gistiotsitoza u grudnogo rebenka, pervonachal'no diagnostiruemogo kak seroznoe vospalenie srednego uha. Rossijskaja otorinolaringologija. 2016; 1: 112–5 [Dolgov V.A., Anikin M.I., Gorbaneva G.A. et al. The case of detection of histiocytosis in nursing infant primarily diagnosed as serous inflammation of middle ear. Rossiiskaya otorinolaringologiya. 2016; 1: 112–5 (in Russ.)]. DOI: 10.18692/1810-4800-2016-1-112-115.
  2. Lazjuk I.I., Borisevich G.A., Sergeeva A.A. i dr. K voprosu diagnostiki gistiotsitoza iz kletok Langergarsa. Novosti luchevoj diagnostiki. 1998; 4: 13–5 [Lazyuk I.I., Borisevich G.A., Sergeeva A.A. i dr. K voprosu diagnostiki gistiotsitoza iz kletok Langergarsa. Novosti luchevoi diagnostiki. 1998; 4: 13–5 (in Russ.)]. URL: https://radiomed.ru/publications/obz-gistiotsitoz-k-voprosu-diagnostiki-gistiotsitoza-iz-kletok-langergarsa
  3. Blumberg J.M., Malhotra A., Wu X. et al. Langerhans Cell Histiocytosis of the Temporal Bone with Otic Capsule Involvement. Clin Neuroradiol. 2017; 27 (2): 163–8. DOI: 10.1007/s00062-015-0461-4
  4. Hussein Algahtani, Bader Shirah, Mohammed Bajunaid et al. Clinicoradiological Discrepancy in Multisystem Langerhans Cell Histiocytosis with Central Nervous System Involvement. Gulf J Oncolog. 2019; 1 (31): 72–7.
  5. Broadbent V., Egeler R.M., Nesbit M.E. et al Langerhans cell histiocytosis – clinical and epidemiological aspects. Br J Cancer. 1994; 23 (1): 11–6.
  6. Guyot-Goubin A., Donadieu J., Barkaoui M. Descriptive epidemiology of childhood Langerhans cell histiocytosis in France, 2000–2004. Pediatr Blood Cancer. 2008; 51: 71–5. DOI: 10.1002/pbc.21498
  7. Dajhes N.A., Diab H.M., Zagorskaja D.A. i dr. Gistiotsitoz kletok Langergansa visochnoj kosti. Annaly hirurgii. 2018; 23 (2): 121–6 [Daykhes N.A., Diab Kh.M., Zagorskaya D.A. et al. Langerhans cell histiocytosis of the temporal bone. Annaly Khirurgii = Russian Journal of Surgery. 2018; 23 (2): 121–6 (in Russ.)]. DOI: 10.18821/1560-9502-2018-23-2-121-126.
  8. Campos M.K., Viana M.B., de Oliveira B.M. et al. Langerhans cell histiocytosis: a 16-year experience. J Pediatr. (Rio J.). 2007; 83 (1): 79–86. DOI: 10.2223/JPED.1581
  9. Boston M., Derkay C.S. Langerhans’ cell histiocytosis of the temporal bone and skull base. Am J Otolaryngol. 2002; 23: 246–8. DOI: 10.1053/ajot.2002.123452
  10. Monsereenusorn C., Rodriguez-Galindo C. Clinical characteristics and treatment of langerhans cell histiocytosis. Hematol Oncol Clin North Am. 2015; 29 (5): 853–73. DOI: 10.1016/j.hoc.2015.06.005
  11. Stull M.A., Kransdorf M.J., Devaney K.O. Langerhans cell histiocytosis of bone. Radiographics. 1992; 12 (4): 801–23. DOI: 10.1148/radiographics.12.4.1636041
  12. Stalemark H., Laurencikas E., Karis J. Incidence of Langerhans cell histiocytosis in children: a population-based study. Pediatr Blood Cancer. 2008; 51: 76–81. DOI: 10.1002/pbc.21504
  13. Salotti J.A., Nanduri V., Pearce M.S. Incidence and clinical features of Langerhans cell histiocytosis in the UK and Ireland. Arch Dis Child. 2009; 94: 376–80. DOI: 10.1136/adc.2008.144527
  14. D’Ambrosio N., Soohoo S., Warshall C. et al. Craniofacial and intracranial manifestations of Langerhans cell histiocytosis: report of findings in 100 cases. Am J Roentgenol. 2008; 191: 589–97. DOI: 10.2214/AJR.07.3573
  15. Majumder A., Wick C.C., Collins R. et al. Pediatric Langerhans cell histiocytosis of the lateral skull base. Int J Pediatr Otorhinolaryngol. 2017; 99: 135–40. DOI: 10.1016/j.ijporl.2017.06.011
  16. Cochrane L.A., Prince M., Clarke K. Langerhans’ cell histiocytosis in the paediatric population: presentation and treatment of head and neck manifestations. J Otolaryngol. 2003; 32: 33–7. DOI: 10.2310/7070.2003.35266
  17. DiNardo L.J., Wetmore R.F. Head and neck manifestations of histiocytosis-X in children. Laryngoscope. 1989; 99: 721–4. DOI: 10.1288/00005537-198907000-00011
  18. Irving R.M., Broadbent V., Jones N.S. Langerhans’ cell histiocytosis in childhood: Management of head and neck manifestations. Laryngoscope. 1994; 104: 64–70. DOI: 10.1288/00005537-199401000-00011
  19. Vezina J.-P., Audet N., Fradet G. Cerebrospinal fluid otorrhoea: a rare presentation of Langerhans’ cell histiocytosis of the temporal bone. J Laryngol Otol. 2010; 124 (5): 545–8. DOI: 10.1017/S0022215109992295