To perform a systematic review to investigate common otologic manifestations of Langerhans cell histiocytosis, the incidence of these findings, methods for diagnosis, as well as medical and surgical management
Data Sources
PubMed/MEDLINE, Embase, and Cochrane Library.
Review Methods
A search of PubMed/MEDLINE, Embase, and Cochrane Library for all articles published between 1963 to 2020 was performed with variations and combinations of the following search terms: Langerhans cell histiocytosis, eosinophilic granuloma, Letterer-Siwe, Hand-Schüller-Christian, otitis, otologic, ear. A review of the references of all included articles was also conducted.
Results
Sixty-two articles encompassing 631 patients met inclusion criteria. Otologic symptoms at presentation were found in 246 (39%) patients in the reported studies with 48% reporting bilaterality. The mean age was 14.8 years with a male predominance (64%). The most common otologic presenting symptom was otorrhea (46%). A majority had the multisystem variant (52%). The most common treatment modalities were chemotherapy (52%), followed by surgery (50%), systemic steroids (45%), and radiotherapy (31%). Surgery was performed in 75.8% with unisystem involvement and in 50.6% with multisystem involvement. The most effective treatments included radiotherapy (56% success rate, 17% of treated patients), systemic steroids (44% success, 20% treated), chemotherapy (41% success, 21% treated), and surgical modalities (36% success, 19% treated).
Conclusions
Otologic manifestations that occur with the multisystem variant or are at high risk for central nervous system involvement necessitate systemic treatment. For unifocal lesions, surgery is recommended. Lastly, radiotherapy should be reserved for extensive lesions involving vital structures or presenting in older patients.
BlumbergJMMalhotraAWuXVirkRKKvetonJFMichaelidesEM. Langerhans cell histiocytosis of the temporal bone with otic capsule involvement. Clin Neuroradiol. 2017;27(2):163-168.
2.
MajumderAWickCCCollinsRBoothTNIsaacsonBKutzJW. Pediatric Langerhans cell histiocytosis of the lateral skull base. Int J Pediatr Otorhinolaryngol. 2017;99:135-140.
3.
AllenAMatrovaEOzgenBRedleafMEmmadiRSaranN. Langerhans’ cell histiocytosis of the temporal bone in an adult with central diabetes insipidus. Radiol Case Rep. 2019;14(7):847-850.
4.
ModestMCGarciaJJArndtCSCarlsonML. Langerhans cell histiocytosis of the temporal bone: a review of 29 cases at a single center. Laryngoscope. 2016;126(8):1899-1904.
5.
SalibaISidaniKEl FataFArcandPQuintalMCAbelaA. Langerhans’ cell histiocytosis of the temporal bone in children. Int J Pediatr Otorhinolaryngol. 2008;72(6):775-786.
6.
BayazitYSirikciABayaramMKanlikamaMDemirABakirK. Eosinophilic granuloma of the temporal bone. Auris Nasus Larynx. 2001;28(1):99-102.
7.
Abdel-AzizMRashedMKhalifaBTalaatANassarA. Eosinophilic granuloma of the temporal bone in children. J Craniofac Surg. 2014;25(3):1076-1078.
8.
IrvingRMBroadbentVJonesNS. Langerhans’ cell histiocytosis in childhood: management of head and neck manifestations. Laryngoscope. 1994;104(1, pt 1):64-70.
9.
SuricoGMuggeoPMuggeoV, et al. Ear involvement in childhood Langerhans’ cell histiocytosis. Head Neck. 2000;22(1):42-47.
10.
ApplingDJenkinsHAPattonGA. Eosinophilic granuloma in the temporal bone and skull. Otolaryngol Head Neck Surg. 1983;91(4):358-365.
11.
McCaffreyTVMcDonaldTJ. Histiocytosis X of the ear and temporal bone: review of 22 cases. Laryngoscope. 1979;89(11):1735-1742.
12.
MumbucSKaratasEDurucuC, et al. A rare disorder mimics otitis media in children: Langerhans’ cell histiocytosis. International Journal of Pediatric Otorhinolaryngology Extra. 2006;1(1):68-71.
13.
ColemanMAMatsumotoJCarrCMEckelLJNageswara RaoAA. Bilateral temporal bone langerhans cell histiocytosis: radiologic pearls. Open Neuroimag J. 2013;7:53-57.
14.
JonesROPillsburyHC. Histiocytosis X of the head and neck. Laryngoscope. 1984;94(8):1031-1035.
15.
BaharSDalT. Langerhans cell histiocytosis of bilateral mastoid cavity. J Int Adv Otol. 2018;14(2):341-343.
16.
GungadeenAKullarPYatesP. Otic langerhans’ cell histiocytosis in an adult: a case report and review of the literature. Case Rep Otolaryngol. 2013;2013:259726.
BaserSKamanAZaimogluI, et al. A case of Langerhans cell histiocytosis mimicking child abuse. Turk J Pediatr. 2016;58(6):675-678.
19.
HendersonAHKhanAAGoodCBaerST. A case of bilateral sensorineural hearing loss from Langerhans cell histiocytosis. BMJ Case Rep. 2015;2015:bcr2015210996.
20.
LosieJAYongMKozakFKChadhaNK. Unique case of hearing recovery after otic capsule destruction and complete sensorineural hearing loss caused by langerhans cell histiocytosis. Otol Neurotol. 2017;38(8):1129-1132.
21.
TosMJohnsenS. ENT manifestations in Letterer-Siwe disease. Acta Otolaryngol. 1963;56:84-92.
22.
SkoulakisCEDrivasEIPapadakisCEBizakiAJStavroulakiPHelidonisES. Langerhans cell histiocytosis presented as bilateral otitis media and mastoiditis. Turk J Pediatr. 2008;50(1):70-73.
23.
DavisSERiceDH. Langerhans’ cell histiocytosis: current trends and the role of the head and neck surgeon. Ear Nose Throat J. 2004;83(5):340, 342, 344.
24.
HadfieldPJBirchallMAAlbertDM. Otitis externa in Langerhans’ cell histiocytosis—the successful use of topical nitrogen mustard. Int J Pediatr Otorhinolaryngol. 1994;30(2):143-149.
25.
NanduriVRPritchardJChongWKPhelpsPDSirimannaKBaileyCM. Labyrinthine involvement in Langerhans’ cell histiocytosis. Int J Pediatr Otorhinolaryngol. 1998;46(1-2):109-115.
26.
BozdemirKTarlakBCakarH, et al. Langerhans cell histiocytosis in bilateral mastoid cavity. Case Rep Otolaryngol. 2013;2013:957926.
27.
JeunonTSousaMASantos-RodriguesNLopesR. Langerhans cell histiocytosis—a case report. Dermatol Pract Concept. 2012;2(1):25-29.
28.
DeMarinoDPDutcherPOJrParkinsCWHengererAS. Histiocytosis X: otologic presentations. Int J Pediatr Otorhinolaryngol. 1985;10(1):91-100.
29.
KarmodyCSMoloyPJ. Letterer-Siwe disease: presentation as an otologic problem. Laryngoscope. 1978;88(8, pt 1):1343-1347.
30.
RodmanOGCooperPH. Multifocal eosinophilic granuloma of skin and bone: thirteen year surveillance of a patient. Cutis. 1980;26(5):487-498.
31.
YetiserSKarahataySDeveciS. Eosinophilic granuloma of the bilateral temporal bone. Int J Pediatr Otorhinolaryngol. 2002;62(2):169-173. doi:10.1016/s0165-5876(01)00615-2
32.
AkisadaTHaradaTYoshihiroTKawaiA. A case of bilateral eosinophilic granuloma in the temporal bone. Auris Nasus Larynx. 1999;26(3):343-348. doi:10.1016/s0385-8146(99)00006-1
33.
DeRoweABernheimJOphirD. Eosinophilic granuloma presenting as chronic otitis media: pitfalls in the diagnosis of aural polyps in children. J Otolaryngol. 1995;24(4):258-260.
34.
GinsbergHNSwayneLCPeronDLMagidsonJGNewcombAW. Bilateral temporal bone involvement with eosinophilic granuloma. Comput Med Imaging Graph. 1988;12(2):107-110. doi:10.1016/0895-6111(88)90004-3
35.
UtrataJ. Xanthogranuloma of the mastoid leading to a diagnosis of Hand-Schüller-Christian disease. Int Surg. 1972;57(6):511-513.
36.
HudsonWRKenanPD. Otologic manifestations of histiocytosis X. Laryngoscope. 1969;79(4):678-693. doi:10.1288/00005537-196904000-00010
37.
OngAARosi-SchumacherMPizzutoM. Langerhans cell histiocytosis of the skull. Ear Nose Throat J. Published online September26, 2019. doi:10.1177/0145561319869605
38.
MosnierIRondini-GilliECrosaraPT, et al. Langerhans’ cell histiocytosis of the labyrinth in adults. Otol Neurotol. 2004;25(1):27-32. doi:10.1097/00129492-200401000-00006
39.
DarrAMughalZMartinT. A rare case of extensive cranial Langerhans cell histiocytosis, synchronously presenting as otitis externa and giant cell arteritis. J Surg Case Rep. 2016;2016(11):rjw094. doi:10.1093/jscr/rjw094
40.
CunninghamMJCurtinHDJaffeRStoolSE. Otologic manifestations of Langerhans’ cell histiocytosis. Arch Otolaryngol Head Neck Surg. 1989;115(7):807-813. doi:10.1001/archotol.1989.01860310045020
41.
NishalAModiJBalarH. Langerhans cell histiocytosis. Indian J Otol. 2013;19(4):194-195. doi:10.4103/0971-7749.124517
42.
NiMYangX. Langerhans’ cell histiocytosis of the temporal bone: a case report. Exp Ther Med. 2017;13(3):1051-1053. doi:10.3892/etm.2017.4072
43.
XieSMLiuWXiangYY, et al. A rare disorder mimics otitis media: Langerhans cell histiocytosis of the temporal bone in a child with interstitial pulmonary fibrosis. Am J Otolaryngol. 2014;35(6):816-821. doi:10.1016/j.amjoto.2014.06.018
44.
EhrhardtMJKarstJDonohouePA, et al. Recognition and treatment of concurrent active and neurodegenerative langerhans cell histiocytosis: a case report. J Pediatr Hematol Oncol. 2015;37(1):e37-e40. doi:10.1097/MPH.0000000000000085
45.
BurnsPFosterAMoranTBlayneyA. Multifocal Langerhans’ cell histiocytosis: a case report. Ir J Med Sci. 2010;179(4):611-613. doi:10.1007/s11845-008-0221-4
46.
MeyerAStarkMKarstensJHChristiansenHBrunsF. Langerhans cell histiocytosis of the cranial base: is low-dose radiotherapy effective?Case Rep Oncol Med. 2012;2012:789640. doi:10.1155/2012/789640
47.
WanifuchiNIshizukaYYabeT. A case of eosinophilic granuloma in the temporal bone. Auris Nasus Larynx. 1991;18(1):17-26. doi:10.1016/s0385-8146(12)80246-x
Yildirim-BaylanMCureogluSPaparellaMM. Langerhans’ cell histiocytosis of the temporal bone. Otol Neurotol. 2012;33(2):e15-e16. doi:10.1097/MAO.0b013e31821a80f8
50.
AlexanderRLWorthenMLPangCSMayJS. Langerhans cell histiocytosis: temporal bone invasion in an adult. Ear Nose Throat J. 2013;92(10-11):496-498.
51.
MunicchiGMarconciniSD’AmbrosioABerardiRAcquavivaA. Central precocious puberty in multisystem Langerhans cell histiocytosis: a case report. Pediatr Hematol Oncol. 2002;19(4):273-278. doi:10.1080/08880010252899451
52.
Sellari-FranceschiniSForliFPieriniSFavreCBerrettiniSMacchiaPA. Langerhans’ cells histiocytosis. Int J Pediatr Otorhinolaryngol. 1999;48(1):83-87. doi:10.1016/s0165-5876(99)00013-0
53.
ArsovicNTomanovicNBukurovB. Sudden sensorineural hearing loss as first presenting symptom of unifocal Langerhans cell histiocytosis in the temporal bone. Otol Neurotol. 2013;34(3):e24-e25. doi:10.1097/MAO.0b013e3182877b16
54.
CunninghamMJCurtinHDButkiewiczBL. Histiocytosis X of the temporal bone: CT findings. J Comput Assist Tomogr. 1988;12(1):70-74. doi:10.1097/00004728-198801000-00013
55.
BairdSMWeiBPCNguyenKBriggsR. Hearing recovery following repair of otic capsule defect secondary to semicircular canal erosion from Langerhans cell histiocytosis. ANZ J Surg. 2020;90(1-2):159-160. doi:10.1111/ans.14999
56.
PareekSSHawassNE-D. An unusual presentation of histiocytosis X. Int J Dermatol. 1985;24(2):126-128. doi:10.1111/j.1365-4362.1985.tb05398.x
57.
Lopez-RiosGBenitezJT. Histiocytosis: histopathological study of the temporal bone. Ann Otol Rhinol Laryngol. 1968;77(6):1171-1180. doi:10.1177/000348946807700613
58.
KamathMPHegdeMCBhojwaniKM, et al. Histiocytosis of the temporal bone. Indian J Otolaryngol Head Neck Surg. 2000;52(3):274-276. doi:10.1007/BF03006202
59.
BruglerG. Tumors presenting as aural polyps: a report of four cases. Pathology. 1992;24(4):315-319. doi:10.3109/00313029209068888
60.
OngHYGohLCSanthiKSha’ariyahMM. Concurrent mastoid cellulitis and Langerhans cells histiocytosis: a challenging diagnosis. Oman Med J. 2018;33(2):167-170. doi:10.5001/omj.2018.31
61.
AngeliSILuxfordWMLoWW. Magnetic resonance imaging in the evaluation of Langerhans’ cell histiocytosis of the temporal bone: case report. Otolaryngol Head Neck Surg. 1996;114(1):120-124. doi:10.1016/s0194-5998(96)70297-8
62.
YavasUSIncesuluAAcikalinM, et al. Eosinophilic granuloma of the temporal bone with extensive bilateral otic capsule involvements: incomplete reossification despite therapy. International Journal of Pediatric Otorhinolaryngology Extra. 2010;5(2):74-78.
63.
JonasNMulwafuWKhosaSAHendricksM. Case study: Langerhans’ cell histiocytosis (LCH). International Journal of Pediatric Otorhinolaryngology Extra. 2008;3(2):61-65.
64.
JacobusJCastroNSquegliaLM, et al. Adolescent cortical thickness pre- and post marijuana and alcohol initiation. Neurotoxicol Teratol. 2016;57:20-29.
65.
HauptRMinkovMAstigarragaI, et al. 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):175-184.
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