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Published in: Clinical Neuroradiology 2/2017

01-06-2017 | Original Article

Langerhans Cell Histiocytosis of the Temporal Bone with Otic Capsule Involvement

Authors: J. M. Blumberg, A. Malhotra, X. Wu, R. K. Virk, J. F. Kveton, E. M. Michaelides

Published in: Clinical Neuroradiology | Issue 2/2017

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Abstract

Introduction

This study is conducted to demonstrate that destructive lesions of the otic capsule by Langerhans cell histiocytosis (LCH) causing both radiographic and audiologic findings can be completely reversed with adequate treatment. Retrospective case review and analysis of clinical and imaging data were obtained as part of the diagnosis and treatment of patients with LCH of the temporal bone.

Methods

With Institutional Review Board (IRB) approval, cases of LCH involving the temporal bone were searched for within the institutional databases. Criteria for inclusion was histologic diagnosis of LCH and pretreatment computed tomography (CT) demonstrating temporal bone and/or otic capsule involvement and posttreatment follow-up CT/magnetic resonance imaging (MRI) scans obtained at least 6 months after starting treatment.

Results

We report eight cases of LCH of the temporal bone with three demonstrating otic capsule involvement radiographically and/or clinically. Review of posttreatment imaging revealed all three patients had complete restoration of the bony labyrinthine architecture and near or complete restoration of their hearing.

Conclusions

Though LCH of the temporal bone is a common site within the spectrum of the disease, involvement of the otic capsule remains rare. Here, we report the largest series of otic capsule involvement by LCH and investigate whether both architecture and hearing are recovered with appropriate treatment. Lastly, restoration of the bony architecture of the labyrinth suggests the mechanism of LCH is demineralization and not ablative.
Literature
1.
go back to reference Abla O, Egeler RM, Weitzman S. Langerhans cell histiocytosis: current concepts and treatments. Cancer Treat Rev. 2010;36(4):354–9.CrossRefPubMed Abla O, Egeler RM, Weitzman S. Langerhans cell histiocytosis: current concepts and treatments. Cancer Treat Rev. 2010;36(4):354–9.CrossRefPubMed
2.
go back to reference Willman CL, Busque L, Griffith BB, Favara BE, McClain KL, Duncan MH, Gilliland DG. Langerhans’-cell histiocytosis (histiocytosis X)–a clonal proliferative disease. N Engl J Med. 1994;331(3):154–60.CrossRefPubMed Willman CL, Busque L, Griffith BB, Favara BE, McClain KL, Duncan MH, Gilliland DG. Langerhans’-cell histiocytosis (histiocytosis X)–a clonal proliferative disease. N Engl J Med. 1994;331(3):154–60.CrossRefPubMed
3.
go back to reference Weitzman S, Egeler RM. Histiocytic disorders of children and adults. Cambridge: Cambridge University Press; 2005.CrossRef Weitzman S, Egeler RM. Histiocytic disorders of children and adults. Cambridge: Cambridge University Press; 2005.CrossRef
4.
go back to reference Nanduri VR, Pritchard J, Chong WK, Phelps PD, Sirimanna K, Bailey CM. Labyrinthine involvement in Langerhans’ cell histiocytosis. Int J Pediatr Otorhinolaryngol. 1998;46(1–2):109–15.CrossRefPubMed Nanduri VR, Pritchard J, Chong WK, Phelps PD, Sirimanna K, Bailey CM. Labyrinthine involvement in Langerhans’ cell histiocytosis. Int J Pediatr Otorhinolaryngol. 1998;46(1–2):109–15.CrossRefPubMed
5.
go back to reference Fernandez-Latorre F, Menor-Serrano F, Alonso-Charterina S, Arenas-Jimenez J. Langerhans’ cell histiocytosis of the temporal bone in pediatric patients: imaging and follow-up. AJR Am J Roentgenol. 2000;174(1):217–21.CrossRefPubMed Fernandez-Latorre F, Menor-Serrano F, Alonso-Charterina S, Arenas-Jimenez J. Langerhans’ cell histiocytosis of the temporal bone in pediatric patients: imaging and follow-up. AJR Am J Roentgenol. 2000;174(1):217–21.CrossRefPubMed
6.
go back to reference Suzuki H, Nishizawa S, Hohchi N, Wakasugi T, Shibata M, Ohkubo J, Tsukada J. Langerhans cell histiocytosis of the petrous bone with sudden sensorineural hearing loss. Case report. Neurol Med Chir. 2010;50(8):693–7.CrossRef Suzuki H, Nishizawa S, Hohchi N, Wakasugi T, Shibata M, Ohkubo J, Tsukada J. Langerhans cell histiocytosis of the petrous bone with sudden sensorineural hearing loss. Case report. Neurol Med Chir. 2010;50(8):693–7.CrossRef
7.
go back to reference Chisolm JJ. Otorhinologic aspects of Hand-Schuller-Christian’s disease. Laryngoscope. 1954;64(6):486–96.CrossRefPubMed Chisolm JJ. Otorhinologic aspects of Hand-Schuller-Christian’s disease. Laryngoscope. 1954;64(6):486–96.CrossRefPubMed
8.
go back to reference Cohn AM, Sataloff J, Lindsay JR, Histiocytosis X. Archives of otolaryngology (Chicago, Ill: 1960). 1970;91(1):24–9. Cohn AM, Sataloff J, Lindsay JR, Histiocytosis X. Archives of otolaryngology (Chicago, Ill: 1960). 1970;91(1):24–9.
9.
go back to reference Cunningham MJ, Curtin HD, Jaffe R, Stool SE. Otologic manifestations of Langerhans’ cell histiocytosis. Arch Otolaryngol Head Neck Surg. 1989;115(7):807–13.CrossRefPubMed Cunningham MJ, Curtin HD, Jaffe R, Stool SE. Otologic manifestations of Langerhans’ cell histiocytosis. Arch Otolaryngol Head Neck Surg. 1989;115(7):807–13.CrossRefPubMed
10.
go back to reference Hore I, Mitchell RB, Radcliffe G, Quiney R, Walker T. Langerhans’ cell histiocytosis—a rare cause of sudden onset unilateral sensorineural hearing loss. J Laryngol Otol. 1999;113(12):1098–100.CrossRefPubMed Hore I, Mitchell RB, Radcliffe G, Quiney R, Walker T. Langerhans’ cell histiocytosis—a rare cause of sudden onset unilateral sensorineural hearing loss. J Laryngol Otol. 1999;113(12):1098–100.CrossRefPubMed
11.
go back to reference Irving RM, Broadbent V, Jones NS. Langerhans’ cell histiocytosis in childhood: management of head and neck manifestations. Laryngoscope. 1994;104(1 Pt 1):64–70.CrossRefPubMed Irving RM, Broadbent V, Jones NS. Langerhans’ cell histiocytosis in childhood: management of head and neck manifestations. Laryngoscope. 1994;104(1 Pt 1):64–70.CrossRefPubMed
12.
go back to reference Kleinjung T, Woenckhaus M, Bachthaler M, Wolff JE, Wolf SR. Langerhans’ cell histiocytosis with bilateral temporal bone involvement. Am J Otolaryngol. 2003;24(4):265–70.CrossRefPubMed Kleinjung T, Woenckhaus M, Bachthaler M, Wolff JE, Wolf SR. Langerhans’ cell histiocytosis with bilateral temporal bone involvement. Am J Otolaryngol. 2003;24(4):265–70.CrossRefPubMed
13.
go back to reference Lopez-Rios G, Benitez JT. Histiocytosis: histopathological study of the temporal bone. Ann Otol Rhinol Laryngol. 1968;77(6):1171–80.CrossRefPubMed Lopez-Rios G, Benitez JT. Histiocytosis: histopathological study of the temporal bone. Ann Otol Rhinol Laryngol. 1968;77(6):1171–80.CrossRefPubMed
14.
15.
go back to reference McCaffrey TV, McDonald TJ. Histiocytosis X of the ear and temporal bone: review of 22 cases. Laryngoscope. 1979;89(11):1735–42.CrossRefPubMed McCaffrey TV, McDonald TJ. Histiocytosis X of the ear and temporal bone: review of 22 cases. Laryngoscope. 1979;89(11):1735–42.CrossRefPubMed
16.
go back to reference Mosnier I, Rondini-Gilli E, Crosara PT, Belmatoug N, Cyna-Gorse F, Cazals-Hatem D, Abbey-Toby A, Bozorg-Grayeli A, Sterkers O. Langerhans’ cell histiocytosis of the labyrinth in adults. Otol Neurotol. 2004;25(1):27–32.CrossRefPubMed Mosnier I, Rondini-Gilli E, Crosara PT, Belmatoug N, Cyna-Gorse F, Cazals-Hatem D, Abbey-Toby A, Bozorg-Grayeli A, Sterkers O. Langerhans’ cell histiocytosis of the labyrinth in adults. Otol Neurotol. 2004;25(1):27–32.CrossRefPubMed
17.
go back to reference Saliba I, Sidani K, Fata F E, Arcand P, Quintal MC, Abela A. Langerhans’ cell histiocytosis of the temporal bone in children. Int J Pediatr Otorhinolaryngol. 2008;72(6):775–86.CrossRefPubMed Saliba I, Sidani K, Fata F E, Arcand P, Quintal MC, Abela A. Langerhans’ cell histiocytosis of the temporal bone in children. Int J Pediatr Otorhinolaryngol. 2008;72(6):775–86.CrossRefPubMed
18.
go back to reference Goldsmith AJ, Myssiorek D, Valderrama E, Patel M. Unifocal Langerhans’ cell histiocytosis (eosinophilic granuloma) of the petrous apex. Arch Otolaryngol Head Neck Surg. 1993;119(1):113–6.CrossRefPubMed Goldsmith AJ, Myssiorek D, Valderrama E, Patel M. Unifocal Langerhans’ cell histiocytosis (eosinophilic granuloma) of the petrous apex. Arch Otolaryngol Head Neck Surg. 1993;119(1):113–6.CrossRefPubMed
19.
go back to reference Neilan RE, Kutz JW Jr. Langerhans cell histiocytosis of the temporal bone. Otol Neurotol. 2012;33(4):e31–2.CrossRefPubMed Neilan RE, Kutz JW Jr. Langerhans cell histiocytosis of the temporal bone. Otol Neurotol. 2012;33(4):e31–2.CrossRefPubMed
20.
go back to reference Saliba I, Sidani K. Prognostic indicators for sensorineural hearing loss in temporal bone histiocytosis. Int J Pediatr Otorhinolaryngol. 2009;73(12):1616–20.CrossRefPubMed Saliba I, Sidani K. Prognostic indicators for sensorineural hearing loss in temporal bone histiocytosis. Int J Pediatr Otorhinolaryngol. 2009;73(12):1616–20.CrossRefPubMed
21.
go back to reference Satter EK, High WA. Langerhans cell histiocytosis: a review of the current recommendations of the Histiocyte Society. Pediatr Dermatol. 2008;25(3):291–5.CrossRefPubMed Satter EK, High WA. Langerhans cell histiocytosis: a review of the current recommendations of the Histiocyte Society. Pediatr Dermatol. 2008;25(3):291–5.CrossRefPubMed
23.
go back to reference Haupt R, Minkov M, Astigarraga I, Schafer E, Nanduri V, Jubran R, Egeler RM, Janka G, Micic D, Rodriguez-Galindo C, Van Gool S, Visser J, Weitzman S, Donadieu J; Euro Histio Network. Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years. Pediatr Blood Cancer. 2013;60(2):175–84.CrossRefPubMed Haupt R, Minkov M, Astigarraga I, Schafer E, Nanduri V, Jubran R, Egeler RM, Janka G, Micic D, Rodriguez-Galindo C, Van Gool S, Visser J, Weitzman S, Donadieu J; Euro Histio Network. Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years. Pediatr Blood Cancer. 2013;60(2):175–84.CrossRefPubMed
24.
go back to reference Nesbit ME, Kieffer S, D’Angio GJ. Reconstitution of vertebral height in histiocytosis X: a long-term follow-up. J Bone Joint Surg Am. 1969;51(7):1360–8.CrossRefPubMed Nesbit ME, Kieffer S, D’Angio GJ. Reconstitution of vertebral height in histiocytosis X: a long-term follow-up. J Bone Joint Surg Am. 1969;51(7):1360–8.CrossRefPubMed
25.
go back to reference Raab P, Hohmann F, Kuhl J, Krauspe R. Vertebral remodeling in eosinophilic granuloma of the spine. A long-term follow-up. Spine. 1998;23(12):1351–4.CrossRefPubMed Raab P, Hohmann F, Kuhl J, Krauspe R. Vertebral remodeling in eosinophilic granuloma of the spine. A long-term follow-up. Spine. 1998;23(12):1351–4.CrossRefPubMed
26.
go back to reference Brown RE. More on pamidronate in Langerhans’-cell histiocytosis. N Engl J Med. 2001;345(20):1503.CrossRefPubMed Brown RE. More on pamidronate in Langerhans’-cell histiocytosis. N Engl J Med. 2001;345(20):1503.CrossRefPubMed
27.
go back to reference Sivendran S, Harvey H, Lipton A, Drabick J. Treatment of Langerhans cell histiocytosis bone lesions with zoledronic acid: a case series. Int J Hematol. 2011;93(6):782–6.CrossRefPubMed Sivendran S, Harvey H, Lipton A, Drabick J. Treatment of Langerhans cell histiocytosis bone lesions with zoledronic acid: a case series. Int J Hematol. 2011;93(6):782–6.CrossRefPubMed
28.
go back to reference Abildgaard N, Rungby J, Glerup H, Brixen K, Kassem M, Brincker H, Heickendorff L, Eriksen EF, Nielsen JL. Long-term oral pamidronate treatment inhibits osteoclastic bone resorption and bone turnover without affecting osteoblastic function in multiple myeloma. Eur J Haematol. 1998;61(2):128–34.CrossRefPubMed Abildgaard N, Rungby J, Glerup H, Brixen K, Kassem M, Brincker H, Heickendorff L, Eriksen EF, Nielsen JL. Long-term oral pamidronate treatment inhibits osteoclastic bone resorption and bone turnover without affecting osteoblastic function in multiple myeloma. Eur J Haematol. 1998;61(2):128–34.CrossRefPubMed
29.
go back to reference D’Souza MJ, Oettinger CW, Shah A, Tipping PG, Huang XR, Milton GV. Macrophage depletion by albumin microencapsulated clodronate: attenuation of cytokine release in macrophage-dependent glomerulonephritis. Drug Dev Ind Pharm. 1999;25(5):591–6.CrossRefPubMed D’Souza MJ, Oettinger CW, Shah A, Tipping PG, Huang XR, Milton GV. Macrophage depletion by albumin microencapsulated clodronate: attenuation of cytokine release in macrophage-dependent glomerulonephritis. Drug Dev Ind Pharm. 1999;25(5):591–6.CrossRefPubMed
30.
go back to reference Arzoo K, Sadeghi S, Pullarkat V. Pamidronate for bone pain from osteolytic lesions in Langerhans’-cell histiocytosis. N Engl J Med. 2001;345(3):225.CrossRefPubMed Arzoo K, Sadeghi S, Pullarkat V. Pamidronate for bone pain from osteolytic lesions in Langerhans’-cell histiocytosis. N Engl J Med. 2001;345(3):225.CrossRefPubMed
Metadata
Title
Langerhans Cell Histiocytosis of the Temporal Bone with Otic Capsule Involvement
Authors
J. M. Blumberg
A. Malhotra
X. Wu
R. K. Virk
J. F. Kveton
E. M. Michaelides
Publication date
01-06-2017
Publisher
Springer Berlin Heidelberg
Published in
Clinical Neuroradiology / Issue 2/2017
Print ISSN: 1869-1439
Electronic ISSN: 1869-1447
DOI
https://doi.org/10.1007/s00062-015-0461-4

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