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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 26  |  Issue : 4  |  Page : 289-292

Otological manifestations of Langerhans cell histiocytosis in a child - An enigma that persists!


1 Department of Otorhinolaryngology, University Kebangsaan Malaysia Medical Center; Department of Otorhinolaryngology, Hospital Kuala Lumpur, Kuala Lumpur, Malaysia
2 Department of Otorhinolaryngology; Center for Ear, Hearing and Speech (Pusat HEARS), University Kebangsaan Malaysia Medical Center, Kuala Lumpur, Malaysia
3 Department of Otorhinolaryngology, Hospital Kuala Lumpur, Kuala Lumpur, Malaysia
4 Department of Pathology, Hospital Kuala Lumpur, Kuala Lumpur, Malaysia

Date of Submission26-Apr-2020
Date of Decision18-Jun-2020
Date of Acceptance21-Jul-2020
Date of Web Publication23-Apr-2021

Correspondence Address:
Dr. Chow Xiao Hong
Department of Otorhinolaryngology, University Kebangsaan Malaysia Medical Center, Kuala Lumpur
Malaysia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/indianjotol.INDIANJOTOL_69_20

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  Abstract 


Langerhans cell histiocytosis (LCH) is a rare nonmalignant entity commonly seen in the pediatric population which encompasses a variety of clinical presentations. Otological involvement in LCH is uncommon which can be manifested as symptoms similar to otomastoiditis or malignancy, thus diagnosis is often delayed. Herein, we report a 1-year 7-month-old boy presented with unresolving left otorrhea complicated with left postauricular swelling. Otoscopic examination revealed granulation tissue in the left external auditory canal. Initial diagnosis of left otomastoiditis was made until high-resolution contrast-enhanced computed tomography of the temporal bone showed soft-tissue masses occupying bilateral mastoids with bony destruction. Histopathological examination with immunohistochemical staining expressed CD1a cytoplasmic immunoreactivity. The diagnosis of LCH was made. This report serves to highlight such a rare clinical entity which often masquerades as other otological diseases and hence becomes a diagnostic challenge in the pediatric population.

Keywords: Histiocytosis, Langerhans cells, mastoiditis, temporal bone


How to cite this article:
Hong CX, Abdullah A, Alias A, Yusoff SM. Otological manifestations of Langerhans cell histiocytosis in a child - An enigma that persists!. Indian J Otol 2020;26:289-92

How to cite this URL:
Hong CX, Abdullah A, Alias A, Yusoff SM. Otological manifestations of Langerhans cell histiocytosis in a child - An enigma that persists!. Indian J Otol [serial online] 2020 [cited 2021 Oct 23];26:289-92. Available from: https://www.indianjotol.org/text.asp?2020/26/4/289/314352




  Introduction Top


Langerhans cell histiocytosis (LCH), formerly known as histiocytosis X, is a rare disease described as an abnormal clonal proliferation of Langerhans cell outside the dermis in which the etiology remains unclear.[1] It is uncommon in the pediatric population and accounts for 3–5 cases per million children with slight predominance of male sex.[2] Thus, the aim of this case report serves to emphasize its rarity with common otological symptoms as the initial presentation of the disease and its diagnostic challenge.


  Case Report Top


A 1-year 7-month-old boy presented to our center with painless left postauricular swelling for 2 months, associated with minimal foul-smelling left otorrhea and intermittent fever. Before this, he was treated as simple otitis externa in primary care for a couple of months in which his symptoms did not show any improvement, and therefore, he was referred to a tertiary center with otorhinolaryngology–head-and-neck surgery specialty. Otherwise, the child was active with no constitutional symptom. Antenatal and postnatal history was unremarkable. Developmental age and immunization of the child were up to age. However, he has a strong family history of malignancy in which his elder sibling passed away at 1 year 8 months old due to acute lymphocytic leukemia.

On examination, the child appeared to not be syndromic looking and active. His vital signs were stable. General inspection revealed generalized painless seborrheic rashes on the scalp. The left pinna appeared to be pushed forward and downward. There was an erythematous diffuse firm-swelling measured about 4 cm × 3 cm at the left postauricular region [Figure 1]. Otoscopic examination of the ear revealed granulation tissue-like mass at the left external auditory canal (EAC) which obscured the tympanic membrane [Figure 2]. Right ear examination appeared to be normal. Other systemic examinations were unremarkable. The child was initially treated as left otomastoiditis until a high-resolution and contrast-enhanced computed tomography (CECT) of the petrous bone demonstrated well-defined enhancing soft-tissue masses at bilateral mastoids, with the right measured 3.4 cm × 4.0 cm × 3.6 cm and the left measured 1.6 cm × 2.1 cm × 1.7 cm, causing bony destruction of the mastoid bones, mastoid air cells, and part of the lateral wall of the temporal bone bilaterally [Figure 3]a and [Figure 3]b. The mass obliterated the left external ear canal, and the middle ear cavity was fluid filled, however, ossicles and scutum were intact bilaterally. The first biopsy taken from the mass in the left EAC was reported as inconclusive and the second reported as acute on chronic inflammation. In view of that, the child was put under general anesthesia for ultrasound-guided core biopsy of the left post auricular mass by an interventional radiologist. The histopathological and immunohistochemical examination of the left postauricular mass showed dense clusters of Langerhans cells amid lymphoid aggregates admixed with eosinophils [Figure 4]a. The Langerhans cells were highlighted by CD1a [Figure 4]b.
Figure 1: Erythematous diffuse postauricular swelling

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Figure 2: Otoendoscopic examination revealed a granulation tissue (arrow) at the left external auditory canal

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Figure 3: (a) High resolution and contrast enhanced computed tomography temporal bone - axial view bone window showed soft- tissue masses at bilateral mastoids causing bony destruction of mastoid air cells ( black arrow ) and temporal bone ( blue arrow). (b) Axial view soft tissue window showed soft tissue lesions occupying bilateral tempora bones (red arrow)

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Figure 4: (a) Histopathological examination revealed dense clusters of Langerhans cells (double arrow) amid lymphoid aggregates admixed wit eosinophils (single arrow). (b) Immunohistochemical staining expressed CD1-alpha cytoplasmic immunoreactivity

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He was then referred to pediatric oncology for further evaluation and subsequently diagnosed with multisystem LCH and commenced on chemotherapy.


  Discussion Top


In current modern medicine, LCH is divided into single system versus multisystem and unifocal versus multifocal disease in which multisystem disease is further subdivided into risk organs (liver, lung, spleen, and bone marrow) positive or negative.[3] The exact etiology of LCH remains obscure. Multiple theories of origin exist which include neoplastic with BRAF mutation, inflammatory with cytokine participation, viral and genetic.[1],[3] According to the studies by Saliba et al.,[4] 17% of LCH cases were associated with temporal bone involvement and 70% of the patients with temporal bone LCH had unilateral involvement, whereas the remaining 30% had a bilateral temporal bone involvement. Another series reported a similar result in which 22.6% LCH with temporal bone involvement and only 28.6% of its involvement are bilateral.[5] Here, we report a rare case of systemic LCH with bilateral temporal bone involvement.

The clinical presentation of LCH can be varied and depends on the site of involvement. As for temporal bone LCH, the common clinical presentations include temporal bone mass (70%), followed by otitis media or externa (60%), hearing loss, EAC polyps, otalgia, and otorrhea.[4] Temporal bone LCH may mimic other otological diseases as its clinical presentation varies. The lists of differential diagnosis are long which include otitis externa, otitis media, mastoiditis, cholesteatoma, or soft-tissue tumor like rhabdomyosarcoma or lymphoma. Thus, detailed history taking and thorough systemic clinical examination are essential as LCH can present as localized or systemic form, and hence, the diagnosis can be challenging.

The patient we report, however, presented with unresolved unilateral otorrhea which was initially misdiagnosed as unresolved otitis externa by primary care which later progressed to the development of unilateral postauricular swelling which was then misdiagnosed and treated as acute mastoiditis by ENT prior reaching the final diagnosis of LCH. Interestingly, the right ear examination of the patient showed normal finding despite extensive destructive lesions based on the high-resolution computed tomography temporal bone. Therefore, it is important for health-care providers to keep LCH in mind as a differential diagnosis, particularly in the pediatric population who presented with unresolved otorrhea and postauricular swelling as these can be the initial presentations of LCH.

Laboratory workup should always include baseline hematological and biochemistry test, erythrocyte sedimentation rate, and plain radiograph like chest radiograph as parts of the workup as systemic form of LCH can involve liver, renal, lung and brain and can be fatal.[3] Plain radiograph of LCH typically appears as punched-out lesion, however, to further diagnose LCH, CECT is often the modality of choice for demonstrating the destructive lesions in the bones.[6],[7] Notably, rhabdomyosarcoma which is likewise commonly seen in the pediatric population may mimic LCH, however, rhabdomyosarcoma tends to involve the petrous apex and middle ear whereby LCH invades usually the mastoid process and pars squama which is seen in our case.[8]

In all cases of temporal LCH, the diagnosis is made via histopathological examination of the tissue. Samples of the tissues are usually obtained through direct biopsy at the site of the lesion in the EAC, however, it often reveals only nondiagnostic granulation tissue, or it can be obtained via mastoidectomy in order to obtain a good sample for histopathological examination.[4] However, in our case, we successfully obtained a conclusive sample of biopsy from the left postauricular mass via ultrasound-guided core biopsy under general anesthesia by an interventional radiologist. This approach of obtaining the biopsy is quite different from the conventional way which commonly reveals only nondiagnostic sample and thus often delays the diagnosis. A comprehensive review by Hashmi et al.[9] shows that histopathological examination of LCH often demonstrates a collection of histiocytes with marked eosinophilic inflammatory infiltrates and S100 protein. CD1a cytoplasmic immunoreactivity is the gold standard in diagnosing LCH.[4],[10] In this case, we reached that the diagnosis of LCH with specimen stained with hematoxylin and eosin revealed dense clusters of Langerhans cells, and it was further supported by immunohistochemical staining that expressed CD1-alpha cytoplasmic immunoreactivity which is the gold standard in diagnosing LCH currently.

In modern medicine, there are several modalities of treatment available for temporal bone LCH which include surgery, chemotherapy, radiotherapy and steroids.[7],[10] The role of surgical intervention is limited in LCH. Localized bony disease can be treated with surgical curettage, whereas extensive or recurrence of the disease favors chemotherapy.[7] Scalp lesion and cervical lymph nodes with temporal bone LCH are bad indicators for recurrence.[4] Prognosis is often unfavorable in multisystem risk organ-positive population.[3]

Early referral and co-management of initial presentation of temporal LCH with pediatric oncology team is equally vital as the regimen of treatment differs between single-system LCH and multisystem LCH. It is mandatory for the greatest benefits of the patient.


  Conclusion Top


LCH is often a diagnostic challenge to a general practitioner, pediatrician, or otolaryngologist in view of the clinical heterogeneity. Hence, it is important for health-care providers to have a high index of suspicion for this rare entity, particularly in the pediatric population as an earlier diagnosis can lead to earlier treatment.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the parents have given their consent for their child's images and other clinical information to be reported in the journal. The parents understand that their child's names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Ni M, Yang X. Langerhans' cell histiocytosis of the temporal bone: A case report. Exp Ther Med 2017;13:1051-3.  Back to cited text no. 1
    
2.
Fernandes HB, Granjeiro RC, Junior JN. Langerhans Cell Histiocytosis in Otorhinolaryngology. Int Arch Otorhinolaryngol 2009;13:444-9.  Back to cited text no. 2
    
3.
Morimoto A, Oh Y, Shioda Y, Kudo K, Imamura T. Recent advances in langerhans cell histiocytosis. Pediatr Int 2014;56:451-61.  Back to cited text no. 3
    
4.
Saliba I, Sidani K, El Fata F, Arcand P, Quintal MC, Abela A. Langerhans' cell histiocytosis of the temporal bone in children. Int J Pediatr Otorhinolaryngol 2008;72:775-86.  Back to cited text no. 4
    
5.
Fernández-Latorre F, Menor-Serrano F, Alonso-Charterina S, Arenas-Jiménez J. Langerhans' cell histiocytosis of the temporal bone in pediatric patients: Imaging and follow-up. AJR Am J Roentgenol 2000;174:217-21.  Back to cited text no. 5
    
6.
Coleman MA, Matsumoto J, Carr CM, Eckel LJ, Nageswara Rao AA. Bilateral temporal bone langerhans cell histiocytosis: radiologic pearls. Open Neuroimag J 2013;7:53-7.  Back to cited text no. 6
    
7.
Boston M, Derkay CS. Langerhans' cell histiocytosis of the temporal bone and skull base. Am J Otolaryngol 2002;23:246-8.  Back to cited text no. 7
    
8.
Zheng H, Xia Z, Cao W, Feng Y, Chen S, Li YH, et al. Pediatric langerhans cell histiocytosis of the temporal bone: Clinical and imaging studies of 27 cases. World J Surg Oncol 2018;16:72.  Back to cited text no. 8
    
9.
Hashmi SS, Guha-Thakurta N, Ketonen L, Williams MD, Shah S, Debnam JM. Central nervous system and head and neck histiocytoses: A comprehensive review on the spectrum of imaging findings. Neurographics (2011) 2016;6:114-22.  Back to cited text no. 9
    
10.
Neilan RE, Kutz JW. Langerhans cell histiocytosis of the temporal bone. Otol Neurotol 2012;33:e31-2.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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