Journal of Otology & RhinologyISSN: 2324-8785

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Case Report, J Otol Rhinol Vol: 4 Issue: 5

Langerhans Cell Histiocystosis of Temporal Bone: Case Reports

Mehmet Akif Aksoy*, Arma─čan Incesulu, Ercan Kaya, M Kezban Gürbüz and Hamdi Cakli
Ear, Nose and Throat Department, Eskisehir Osmangazi University, Turkey
Corresponding author : Mehmet Akif Aksoy
Ear, Nose and Throat Department, Eskisehir Osmangazi University, Turkey
E-mail: [email protected]
Received: November 19, 2014 Accepted: June 23, 2015 Published: June 26, 2015
Citation: Aksoy MA, Incesulu A, Kaya E, Gürbüz MK, Cakli H (2015) Langerhans Cell Histiocystosis of Temporal Bone: Case Reports. J Otol Rhinol 4:4. doi:10.4172/2324-8785.1000237

Abstract

Histiocytosis X or Langerhans cell histiocytosis (LCH), is a rare disease that highly varied clinical presentation ranging from single system to multisystem involvement. In otolaryngologic practice temporal bone presentation is clinically similar to otitis externa, otitis media, acute mastoiditis, chronic mastoiditis. We diagnosed and treated 3 temporal bone LCH cases. In this paper these cases are presented and their optimum treatment is discussed in detail.

Keywords: Langerhans cell histiocytosis; Temporal bone; Mastoiditis

Keywords

Langerhans cell histiocytosis; Temporal bone; Mastoiditis

Introduction

Langerhans cell histiocytosis (LCH) is a disease which is characterized by uncontrolled clonal proliferation of immature Langerhans cells and can cause multiple organ failure [1]. Etiology of this disease still remains unclear and it can presents with various types. A comprehensive literature review reveals that the disease is first described by Liechtenstein in 1953 as Histiocytosis X. In the meeting of Histiocytosis Society in 1987, the term of “Langerhans cell histiocytosis” had been used and replaced all previously used terms [2].
The disease has 3 different forms including eosinophilic granuloma, Letterer-Siwe disease and Hand-Schuller-Christian disease [3]. Although eosinophilic granuloma is characterized by regional bone involvement and has a better prognosis, Hand- Schuller-Christian disease is commonly seen under the age of 5 years and is characterized by multifocal osteolytic bone lesions, diabetes insipidus, exophthalmos and mucocutaneous skin lesions. On the other hand, Letterer-Siwe disease is associated with multiple organ involvement and is the form of the disease with a poor prognosis [1].
Because LCH can affect multipl diffuse sites, there is no classic clinical presentation. 55-80% of the patients show the symptom in the head and neck region [1]. Involvement of the head and neck may be associated with skin lesions and lymphadenopathy, and more commonly, with involvement of the cranial bones. Most commonly involved cranial bone is the temporal bone [4].
Temporal bone involvement of LCH could be mistaken with otitis externa, otitis media, acute mastoiditis, chronic mastoiditis and other similar otological diseases, therefore above differentials must be kept in mind before arriving at a diagnosis of LCH [5]. Temporal CT scan generally shows a mass that causes osteolitic lesions at temporal bone and biopsy reveals the diagnosis precisely. Treatment of LCH includes surgical excision, radiotherapy, chemotherapy, and systemic corticosteroids either alone or in different combinations.
In this article, we would like to present three patients with LCH.

Case Report

Case 1
A 2 years old male who had admitted to the emergency service with a lump behind the left ear referred to our clinic. The patient had a history of having that lump for about 1 month which had grown within a few days. Parents have reported no history of hearing loss or external ear discharge; however, the past medical records of the patient had a bilaterally recorded otoacoustic emission test for the problem of articulating in our department 6 months ago. Physical examination revealed a reddish mass of about 1 cm in the left retroauricular region and the otological examination revealed a normal left external ear canal and intact-hyperemic tympanic membrane. Computerized tomography (CT) scan showed a soft-tissue mass in the mastoid eroding sigmoid plate and extending into middle ear. The intracranial structures were in normal. Other physical examinations and blood tests revealed no abnormality (Figure 1).
Figure 1: Axial computed tomographic scan shows a soft-tissue mass in the mastoid eroding sigmoid plate and extending into middle ear.
The patient underwent surgery with a preoperative diagnosis of a malignancy or a complication of chronic otitis media. Because a necrotic mass was found in the retroauricular region, intraoperative frozen biopsy was performed. The surgery was terminated after obtaining the biopsy result revealing histiocytic infiltration. Pathological examination was consistent with features of Langerhans cell histiocytosis and he was referred to the Haematology department. Whole-body bone scan showed no other abnormality and the patient was diagnosed as having isolated temporal bone involvement. Chemotherapy was commenced with vinblastine and prednisone which were in use at the time of writing this paper.
Case 2
A 5 years old male presented with a complaint of bilateral hearing loss and discharge for 1 year. On otologic examination, polyps were found in both external ear canals which were confirmed previously by biopsy and pathological examination. Other physical examinations and blood tests revealed no abnormality. The radiologist has interpreted the CT results of the patient that there may be a welldifferentiated tumor extending into the inner ear, granulomatous infection or a congenital cholesteatoma (Figure 2).
Figure 2:Axial computed tomographic scan reveals bilateral osteolytic defects without sclerotic margins filled with soft tissue masses involving the middle ear, mastoid, squama and petrous part of the temporal bone.
The patient underwent to the surgery and right radical mastoidectomy was performed. Intraoperative macroscopic appearance and frozen biopsy did not suggest the cholesteatoma. Thus, the biopsy specimens were obtained from temporal bone and from the polyps in left external ear canal. Pathological examination was consistent with features of Langerhans cell histiocytosis and the patient found to have no multisystemic involvement. The patient who had bilateral temporal involvement was referred to the pediatric oncology and the chemotherapy was started with vinblastine and prednisolone. The patient cured with no complications during follow-ups. Hearing aid was recommended to the patient for bilateral hearing loss.
Case 3
A 45 year old male presented with a complaint of hearing loss and discharge from left ear for six months. After carrying out otologic examination, purulent discharge was found in the left external ear canal. The tympanic membrane was intact but viewed irregular. Audiometric tests resulted mild conductive hearing loss in the left ear. Computerized tomography (CT) scan showed a soft tissue causing erosive changes the internal and external cortex and placed at back of the mastoid area and neighbourhood of sigmoid sinus. radiologically the differentials suggested were interpreted as a possible granulomatous infection (Figure 3).
Figure 3:Axial computed tomographic scan showed a soft tissue causing erosive changes in the internal and external cortex and placed at the back of mastoid area and in the neighbourhood of sigmoid sinus.
Where upon we decided to surgery. Operatively the findings were white-gray coloured granulomatous lesion and lesion was totally excised. Pathological examination was consistent with features of Langerhans cell histiocytosis and he was referred to the Hematology department. Whole-body bone scan showed no other abnormality and the patient were diagnosed as having local temporal bone involvement and follow-up treatment was recommended. After 1.5 years follow-up, control PET/BT scan showed increased FDG uptake at nasopharynx and bilateral tonsillar logy. Then tonsillectomy and nasopharynx biopsy was performed. Biopsy results reported as a chronic inflammation and the patient going to follow up (Figure 4).
Figure 4:No mastoid defect after treatment in case 3 on axial computed temporal bone tomographic scan can be seen.

Discussion

When the langerhans cells, which are normally found in the dermis, present outside and clonal proliferation LCH diagnosis is made. Although multiple theories of origin exist including neoplastic, inflammatory, viral and genetic causes; etiology of this disease still remains unclear [1]. In histopathologic examination characteristic Birbeck granules can be find. These granules are supported by immunohistochemical detection of CD1a and S-100 protein positive [2].
Langerhans cell histiocytosis is common in the male children at 1-4 years of age [1]. Accordingly, the two patients presented here are both male children. When reviewing the literature this disease incidence is 0.5 to 5 cases per million per year but the occurrence of disease in adult individuals becomes more frequent [6]. Starting from this information our Case 3 can be considered extremely rare encountered case.
Greater than two thirds of patients with LCH have nondisseminated disease at presentation like our cases [7]. Howarth et al., in their series of 314 patients published in 1999, reported cranial bone involvement in 30% of the patients. The other most commonly involved bones include the femur, vertebrae and pelvis. Frontal, parietal and temporal bones are the most common site of involvement in the cranium [8].
Temporal bone is the most commonly involved site in the skull. In the literature, temporal bone involvement has been reported to be present in 14-61% of the patients [8]. Temporal bone involvement is commonly associated with multisystem involvement. Moreover, the disease has been reported to manifest with otologic symptoms in only a few patients [2]. Therefore, the three patients presented here are extremely rare cases presenting with isolated temporal bone involvement and manifesting with otologic symptoms. In addition, case 2 had bilateral temporal bone involvement which has been reported to have an incidence of 30% in the literature [5].
Otologic presentation of LCH includes a postauricular lump in 10- 30% of the patients (5). The case 1 had presented with a postauricular lump and an intact tympanic membrane which may be explained by the preferential involvement of the middle ear [5].
The management of patients with LCH, especially in those with regional head and neck involvement, is controversial. Many clinicians address the need for invasive procedures [4]. Treatment options include surgical intervention, chemotherapy, radiotherapy and steroid administration. Our Case 1 was treated with chemotherapy and steroids; and the disease was under control at the time of writing this paper. Case 2 was also treated with a similar regimen and has cured. Case 3 had not received any adjuvant form of treatment and upon 2 years of follow up showed no evidence of recurrence.
In the literature, after complete resection of the lesion with clear margins, local recurrence rate is nearly 6% and new lesion presents in nearly 22% of patients [9]. Thus we performed nasopharynx biopsy and tonsillectomy to case 3 and no recurrence was detected from biopsy specimen.
Although temporal bone involvement may mimic many otologic diseases, as seen in our cases, clinician should consider LCH in patients if the discharge from ear does not respond to medical treatment. In suspected cases, the computerized tomographic scan is the gold standard for imaging the temporal bone lesions [10]. Temporal CT scan can reveal an osteolytic lesion or a mass. However, it should be kept in mind that LCH has no specific radiological finding [8].

References

  1. Neilan RE, Kutz JWJ (2012) Langerhans Cell Histiocytosis of the Temporal Bone. Otology & Neurotology 33: e31-e32.

  2. Kleinjung T, Woenckhaus M, Bachthaler M, Wolff JE, Wolf SR (2003) Langerhans’ cell histiocytosis with bilateral temporal bone involvement. American Journal of Otolaryngology 24: 265-270.

  3. Yavasa US, Incesulub A, Acikalinc M, Calisira C, Adapinara B (2010) Eosinophilic granuloma of the temporal bone with extensive bilateral otic capsule involvements: Incomplete reossification despite theraphy. International Journal of Pediatric Otorhinolaryngology Extra 5: 74-78.

  4. de Brito Macedo Ferreira LM, de Carvalho JDD, Almeida Pereira ST, Tavares MG (2006) Histiocytosis X of the temporal bone. Rev Bras Otorrinolaringol 72: 575.

  5. Skoulakis CE, Drivas EI, Papadakis CE, Bizaki AJ, Stavroulaki P, et al. (2008) Langerhans cell histiocytosis presented as bilateral otitis media and mastoiditis. The Turkish journal of paediatrics 50: 70-73.

  6. Arsovic N, Tomanovic N, Bukurov B (2013) Sudden sensorineural hearing loss as first presenting symptom of unifocal Langerhans cell histiocytosis in the temporal bone. Otology & Neurotology 34: e24-25.

  7. Boston M, Derkay CS (2002) Langerhans' cell histiocytosis of the temporal bone and skull base. American Journal of Otolaryngology 23: 246-248.

  8. Bozdemir K, Tarlak B, Çakar H, Doblan A, Kutluhan A, et al. (2013) Langerhans cell histiocytosis in bilateral mastoid cavity. Case reports in otolaryngology 2013: 957926.

  9. Binning MJ, Brockmeyer DL (2008) Novel multidisciplinary approach for treatment of langerhans cell histiocytosis of the skull base. Skull base 18: 53-58.

  10. Yildirim-Baylan M, Cureoglu S, Paparella MM (2012) Langerhans' cell histiocytosis of the temporal bone. Otology & Neurotology 33: e15-e16.

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