All Title Author
Keywords Abstract

Otic Langerhans' Cell Histiocytosis in an Adult: A Case Report and Review of the Literature

DOI: 10.1155/2013/259726

Full-Text   Cite this paper   Add to My Lib


Objective. To present a case of otic Langerhans' cell histiocytosis in an adult. Also included the diagnosis and management of the condition and a review of the relevant literature. Case Report. We report a case of a 41-year-old man with a history of persistent unilateral ear discharge associated with an aural polyp. Radiological imaging showed bony lesions of the skull and a soft-tissue mass within the middle ear. Histological analysis of the polyp demonstrated Langerhans' cell histiocytosis. His otological symptoms were completely resolved with the systemic therapy. Conclusions. Otic Langerhans' cell histiocytosis can present in adults. Persistent ear symptoms along with evidence of soft-tissue masses within the ear and bony lesions of the skull or elsewhere should prompt the otolaryngologists to include Langerhans' cell histiocytosis in their differential diagnosis. Management should be with systemic therapy rather than local surgical treatment. 1. Introduction Langerhans’ cell histiocytosis (LCH) is a rare disease that is characterised by the idiopathic proliferation of abnormal histiocytes, either in one organ or as part of a systemic disease [1–3]. LCH is predominantly known to be a disease affecting children and is rare in adults. LCH has been reported in paediatric studies to involve the ear and temporal bones in 14% to 61% of cases [4]. However, only a few cases of ear involvement have been reported in adults. This report describes a case of LCH affecting the ear in a 41-year-old patients and it is our recommendation that nonsurgical management is preferable. 2. Case Report A 41-year-old self-employed brewery engineer was referred to the ENT department with a four-week history of persistent left-sided mucopurulent ear discharge, despite topical antibiotic therapy including gentamicin. He did not have any other otological symptoms, nor any rhinological or upper aerodigestive tract symptoms. His hearing was within normal limits. He was a smoker of 30 cigarettes per day, had a past medical history of hypertension, and had been recently diagnosed with Langerhans’ cell histiocytosis involving the skull, for which he was under the care of Haematology. His medication list included azathioprine for LCH, antihypertensives, and a statin. On examination, his left external auditory canal was very oedematous and was filled with an inflammatory polyp. Cranial nerve examination was unremarkable. He had a PET-CT as part of his LCH workup, which showed increased fludeoxyglucose (FDG) uptake in the frontal bone, left parietal bone, right occipital bone, and


[1]  J. Donadieu, F. Chalard, and E. Jeziorski, “Medical management of langerhans cell histiocytosis from diagnosis to treatment,” Expert Opinion on Pharmacotherapy, vol. 13, no. 9, pp. 1309–1322, 2012.
[2]  I. Saliba, K. Sidani, F. El Fata, P. Arcand, M. C. Quintal, and A. Abela, “Langerhans' cell histiocytosis of the temporal bone in children,” International Journal of Pediatric Otorhinolaryngology, vol. 72, no. 6, pp. 775–786, 2008.
[3]  G. Surico, P. Muggeo, V. Muggeo et al., “Ear involvement in childhood Langerhans' cell histiocytosis,” Head & Neck, vol. 22, no. 1, pp. 42–47, 2000.
[4]  B. L. Nelson, “Langerhans cell histiocytosis of the temporal bone,” Head and Neck Pathology, vol. 2, no. 2, pp. 97–98, 2008.
[5]  R. Nicollas, A. Rome, H. Bela?ch et al., “Head and neck manifestation and prognosis of Langerhans' cell histiocytosis in children,” International Journal of Pediatric Otorhinolaryngology, vol. 74, no. 6, pp. 669–673, 2010.
[6]  R. M. Irving, V. Broadbent, and N. S. Jones, “Langerhans' cell histiocytosis in childhood: management of head and neck manifestations,” Laryngoscope, vol. 104, no. 1, pp. 64–70, 1994.
[7]  T. V. McCaffrey and T. J. McDonald, “Histiocytosis X of the ear and temporal bone: review of 22 cases,” Laryngoscope, vol. 89, no. 11, pp. 1735–1742, 1979.
[8]  M. Aricò, M. Girschikofsky, T. Généreau et al., “Langerhans cell histiocytosis in adults: report from the International Registry of the Histiocyte Society,” European Journal of Cancer, vol. 39, no. 16, pp. 2341–2348, 2003.
[9]  E. G. Whitaker, D. Cerenko, and S. Muller, “Multifocal langerhans' cell histiocytosis involving bilateral temporal bones, lungs, and hypothalamus in an adult,” Skull Base Surgery, vol. 9, no. 1, pp. 51–56, 1999.
[10]  C. P. Kimmelman, E. Nielsen, and J. B. Snow, “Histiocytosis X of the temporal bone,” Otolaryngology, vol. 92, no. 5, pp. 588–590, 1984.
[11]  P. Quesada, M. L. Navarrete, and E. Perello, “Eosinophilic granuloma of the temporal bone,” European Archives of Oto-Rhino-Laryngology, vol. 247, no. 3, pp. 194–196, 1990.
[12]  M. J. Cunningham, H. D. Curtin, R. Jaffe, and S. E. Stool, “Otologic manifestations of Langerhans' cell histiocytosis,” Archives of Otolaryngology, vol. 115, no. 7, pp. 807–813, 1989.
[13]  G. Marioni, C. De Filippis, R. Stramare, M. Carli, and A. Staffieri, “Langerhans' cell histiocytosis: temporal bone involvement,” Journal of Laryngology and Otology, vol. 115, no. 10, pp. 839–841, 2001.


comments powered by Disqus