Hostname: page-component-cd9895bd7-q99xh Total loading time: 0 Render date: 2024-12-27T22:50:37.597Z Has data issue: false hasContentIssue false
  • English
  • Français

Cerebrospinal fluid otorrhoea: a rare presentation of Langerhans' cell histiocytosis of the temporal bone

Published online by Cambridge University Press:  22 December 2009

J-P Vezina ,
N Audet  and
G Fradet
J-P Vezina*
Affiliation:
Department of Otolaryngology and Maxillofacial Surgery, Centre Hospitalier Affilie Universitaire de Quebec, Laval University, Quebec, Canada
N Audet
Affiliation:
Department of Otolaryngology and Maxillofacial Surgery, Centre Hospitalier Affilie Universitaire de Quebec, Laval University, Quebec, Canada
G Fradet
Affiliation:
Department of Otolaryngology and Maxillofacial Surgery, Centre Hospitalier Affilie Universitaire de Quebec, Laval University, Quebec, Canada
*
Address for correspondence: Jean-Philippe Vezina, Department at Laval University, Faculty of Medicine, Ferdinand-Vandry Pavilion #3222, Laval University, Québec, G1K 7P4, Canada. Fax: 418 656 5071 E-mail: jean-philippe.vezina.2@ulaval.ca
Get access Rights & Permissions [Opens in a new window]

Abstract

Objective:

To report a case of Langerhans cell histiocytosis of the temporal bone presenting with cerebrospinal fluid fistula.

Patient:

A Caucasian woman presented to a tertiary care centre in Quebec, Canada, with a new onset of cerebrospinal fluid fistula. She had a significant destructive lesion of the temporal bone, and was diagnosed with Langerhans cell histiocytosis on biopsy.

Interventions:

The patient underwent surgical resection with reconstruction of the posterior fossa and tegmen. She suffered a relapse less than one year after surgery, and was finally treated with chemotherapy.

Main outcome and results:

The patient was free of disease at three-year follow up. No recurrence of the cerebrospinal fluid leak was observed after treatment.

Conclusion:

Langerhans cell histiocytosis of the temporal bone with intra-cranial involvement is rare in adults, with only two cases previously reported. Eleven paediatric cases have been reported. To our knowledge, this patient represents the first report of cerebrospinal fluid fistula as the initial presentation of the disease.

Keywords

Temporal BoneHistiocytosisLangerhans CellCerebrospinal Fluid Otorrhea
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 124 , Issue 5 , May 2010 , pp. 545 - 548
Copyright
Copyright © JLO (1984) Limited 2009

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1Arico, M, Girschikofsky, M, Genereau, T, Klersy, C, McClain, K, Grois, N et al. Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society. Eur J Cancer 2003;39:2341–8CrossRefGoogle ScholarPubMed
2McCaffrey, TV, McDonald, TJ. Histiocytosis X of the ear and temporal bone: review of 22 cases. Laryngoscope 1979;89:1735–42CrossRefGoogle ScholarPubMed
3Cunningham, MJ, Curtin, HD, Jaffe, R, Stool, SE. Otologic manifestations of Langerhans' cell histiocytosis. Arch Otolaryngol Head Neck Surg 1989;115:807–13CrossRefGoogle ScholarPubMed
4Surico, G, Muggeo, P, Muggeo, V, Conti, V, Novielli, C, Romano, A et al. Ear involvement in childhood Langerhans' cell histiocytosis. Head Neck 2000;22:42–73.0.CO;2-5>CrossRefGoogle ScholarPubMed
5Rawlings, CE 3rd, Wilkins, RH. Solitary eosinophilic granuloma of the skull. Neurosurgery 1984;15:155–61CrossRefGoogle ScholarPubMed
6Lichtenstein, L, Jaffe, HL. Eosinophilic granuloma of bone – with report of a case. Am J Pathol 1940;16:595–604Google ScholarPubMed
7Lichtenstein, L. Histiocytosis X; integration of eosinophilic granuloma of bone, Letterer-Siwe disease, and Schuller-Christian disease as related manifestations of a single nosologic entity. AMA Arch Pathol 1953;56:84–102Google ScholarPubMed
8Nezelof, C, Basset, F, Rousseau, MF. Histiocytosis X histogenetic arguments for a Langerhans cell origin. Biomedicine 1973;18:365–71Google ScholarPubMed
9Chu, A, Favara, BE, Ladisch, S, Nezelof, C, Prichard, J. Report and recommendations of the workshop on the childhood histiocytoses: concepts and controversies. Med Pediatr Oncol 1986;14:116–17CrossRefGoogle Scholar
10Raney, RB Jr, D'Angio, GJ. Langerhans' cell histiocytosis (histiocytosis X): experience at the Children's Hospital of Philadelphia, 1970–1984. Med Pediatr Oncol 1989;17:20–8CrossRefGoogle ScholarPubMed
11Devaney, KO, Putzi, MJ, Ferlito, A, Rinaldo, A. Head and neck Langerhans cell histiocytosis. Ann Otol Rhinol Laryngol 1997;106:526–32CrossRefGoogle ScholarPubMed
12Cinberg, JZ. Eosinophilic granuloma in the head and neck: a five year review with report of an instructive case. Laryngoscope 1978;88:1281–9CrossRefGoogle ScholarPubMed
13Arseni, C, Danaila, L, Constantinescu, A. Cranial eosinophilic granuloma. Neurochirurgia (Stuttg) 1977;20:189–99Google ScholarPubMed
14Brisman, JL, Feldstein, NA, Tarbell, NJ, Cohen, D, Cargan, AL, Haddad, J Jr et al. Eosinophilic granuloma of the clivus: case report, follow-up of two previously reported cases, and review of the literature on cranial base eosinophilic granuloma. Neurosurgery 1997;41:273–8, 278–9CrossRefGoogle ScholarPubMed
15Groopman, JE, Golde, DW. The histiocytic disorders: a pathophysiologic analysis. Ann Intern Med 1981;94:95–107CrossRefGoogle ScholarPubMed
16Favara, BE. Langerhans' cell histiocytosis pathobiology and pathogenesis. Semin Oncol 1991;18:3–7Google ScholarPubMed
17Laman, JD, Leenen, PJ, Annels, NE, Hogendoorn, PC, Egeler, RM. Langerhans-cell histiocytosis ‘insight into DC biology’. Trends Immunol 2003;24:190–6CrossRefGoogle ScholarPubMed
18Egeler, RM, D'Angio, GJ. Langerhans cell histiocytosis. J Pediatr 1995;127:1–11CrossRefGoogle ScholarPubMed
19Broadbent, V, Gadner, H, Komp, DM, Ladisch, S. Histiocytosis syndromes in children: II. Approach to the clinical and laboratory evaluation of children with Langerhans cell histiocytosis. Clinical Writing Group of the Histiocyte Society. Med Pediatr Oncol 1989;17:492–5CrossRefGoogle Scholar
20Peuchot, M, Eftekhari, F, Van Tassel, P, Cangir, A. Computed tomography in histiocytosis X of the temporal bone. J Comput Tomogr 1988;12:9–12CrossRefGoogle ScholarPubMed
21Ochsner, SF. Eosinophilic granuloma of bone – experience with 20 cases. Am J Roentgenol Radium Ther Nucl Med 1966;97:719–26CrossRefGoogle ScholarPubMed
22Sweet, RM, Kornblut, AD, Hyams, VJ. Eosinophilic granuloma in the temporal bone. Laryngoscope 1979;89:1545–52CrossRefGoogle ScholarPubMed
23Chu, T, D'Angio, GJ, Favara, BE, Ladisch, S, Nesbit, M, Pritchard, J. Histiocytosis syndromes in children. Lancet 1987;2:41–2CrossRefGoogle ScholarPubMed
24Gadner, H, Grois, N, Arico, M, Broadbent, V, Ceci, A, Jakobson, A et al. A randomized trial of treatment for multisystem Langerhans' cell histiocytosis. J Pediatr 2001;138:728–34CrossRefGoogle ScholarPubMed
25Lahey, E. Histiocytosis X – an analysis of prognostic factors. J Pediatr 1975;87:184–9CrossRefGoogle ScholarPubMed
26Minkov, M, Grois, N, Heitger, A, Potschger, U, Westermeier, T, Gadner, H. Response to initial treatment of multisystem Langerhans cell histiocytosis: an important prognostic indicator. Med Pediatr Oncol 2002;39:581–5CrossRefGoogle ScholarPubMed
27Arcand, P, Caouette, H, Dufour, JJ. Eosinophilic granuloma of the temporal bone: simultaneous bilateral involvement. J Otolaryngol 1985;14:375–8Google ScholarPubMed
28Cataltepe, O, Colak, A, Saglam, S, Onol, B. Unusual location of histiocytosis X. Presenting as a petrous apex syndrome. J Clin Neuroophthalmol 1991;11:31–4Google ScholarPubMed
29Goldsmith, AJ, Myssiorek, D, Valderrama, E, Patel, M. Unifocal Langerhans' cell histiocytosis (eosinophilic granuloma) of the petrous apex. Arch Otolaryngol Head Neck Surg 1993;119:113–16CrossRefGoogle ScholarPubMed
30Hadjigeorgi, C, Parpounas, C, Zarmakoupis, P, Lafoyianni, S. Eosinophilic granuloma of the temporal bone: radiological approach in the pediatric patient. Pediatr Radiol 1990;20:546–9CrossRefGoogle ScholarPubMed
31Leiberman, A, Tal, A, Ziv, JB. Intracranial hypertension due to eosinophilic granuloma of the temporal bone. J Pediatr 1979;95:275–6CrossRefGoogle ScholarPubMed
32Miller, C, Lloyd, TV, Johnson, JC, Hunt, WE. Eosinophilic granuloma of the base of the skull. Case report. J Neurosurg 1978;49:464–6CrossRefGoogle ScholarPubMed
33Rosenfeld, JV, Wallace, D, Klug, GL, Danks, A. Transnasal stereotactic biopsy of a clivus tumor. Technical note. J Neurosurg 1992;76:878–9CrossRefGoogle ScholarPubMed
34Sampson, JH, Rossitch, E Jr, Young, JN, Lane, KL, Friedman, AH. Solitary eosinophilic granuloma invading the clivus of an adult: case report. Neurosurgery 1992;31:755–7, 757–8Google ScholarPubMed
35Krishna, H, Behari, S, Pal, L, Chhabra, AK, Banerji, D, Chhabra, DK et al. Solitary Langerhans cell histiocytosis of the clivus and sphenoid sinus with parasellar and petrous extensions: case report and a review of literature. Surg Neurol 2004;62:447–54CrossRefGoogle Scholar
36Mihova, L, Yaneva, M, Sopadjieva, M, Andreev, V, Nejtcheva, I. Primary solitary eosinophilic granuloma of the brain and postoperative radiotherapy: a clinical case. J BUON 2007;12:125–8Google ScholarPubMed