Journal of Otology & RhinologyISSN: 2324-8785

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Case Report, J Otol Rhinol Vol: 7 Issue: 2

Langerhans Cell Histiocytosis in Temporal Bone: Case Report

Ahmed Abdelrahman Abdelziz1*, Nasr Mohamed M Osman2 and Mariana F Gayyed3

1Department of Otorhinolaryngology, El Minia University Hospital, Egypt

2Department of Radiology, El Minia University Hospital, Minia, Egypt

3Department of Pathology, EL Minia Faculty of Medicine, EL Minia, Egypt

*Corresponding Author : Ahmed Abdelrahman Abdelaziz
Lecturer of Otorhinolaryngology, El Minia University Hospital, El Minia, Egypt
Tel:
00201006203684
E-mail: [email protected]

Received: Janaury 15, 2018 Accepted: February 02, 2018 Published: February 10, 2018

Citation: Abdelziz AA, Osman NMM, Gayyed MF (2018) Langerhans Cell Histiocytosis in Temporal Bone: Case Report. J Otol Rhinol 7:2. doi: 10.4172/2324-8785.1000339

Abstract

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by uncontrolled proliferation of Langerhans cells. We report a case of a 5-years old girl with left post auricular swelling, otalagia and otorrhea of 2 month duration, High resolution computed tomography of temporal bone show destructive expanding tissue density mass. Apparent diffusion coefficient is 1.08×10?³ mm²/s in diffusion weight magnetic resonance imaging .Post auricular incision revealed a pinkish, firm granulomatous mass occupied middle ear and mastoid Histopathological examination revealed a mixture of Langerhans histocytes and eosinophils. CD1 and S100 are positive in mmunohistochemistry.

Keywords: Histiocytosis; Temporal bone; Diffusion weight MRI; CT temporal bone

Introduction

Langerhans Cell Histiocytosis (LCH) is a rare disorder characterized by uncontrolled proliferation of Langerhans cells [1]. LCH commonly affects children between 1 and 4 years of age, with an incidence of 5-6 cases per a million children [2].

Case Presentation

A 5-year old girl was admitted to Minia University Hospital in January 2016 with left post auricular swelling, otalagia and otorrhea of two month duration. She sought medical advice before admission and was treated with antibiotics with no improvement. On physical examination, there was tender post auricular swelling with external auditory canal swelling (polyp); there was no nystagmus or facial nerve palsy. Audiometry showed severe to profound left SNHL hearing loss. Lab investigation revealed mild normocytic normochromic anemia, mild leukocytosis, elevated ESR and elevated CRP. High resolution computed tomography of temporal bone showed destructive expanding tissue density mass, completely filling the left middle ear cavity and mastoid antrum with erosion of ossicles, mastoid cortex, lateral semicircular canal and posterior external auditory canal (EAC) extended through the left EAC and laterally through the mastoid bony defect (Figure 1).

Figure 1: CT petrous bone axial cut show: soft tissue mass with destruction of mastoid cortex and middle ear.

Diffusion weight MRI revealed hypo intense in T1, slightly hyper intense in T2, constructive interference in steady state (CISS): hyper intense, weight diffusion (DW): mild restriction (slightly hyper intense) and apparent diffusion coefficient (ADC) is 1.08×10̄³ mm²/s.

Post auricular incision revealed a pinkish, firm granulomatous mass occupied middle ear and mastoid (auto mastoidectomy) (Figure 2). The mass was separated from surrounding in ease (Figure 3). The researcher identified facial nerve with difficulty and dissected it carefully from the mass (nerve is intact after dissection as shown in Figure 4.

Figure 2: After post auricular incision: A pinkish granulomatous mass occupied middle ear and mastoid (auto mastoidectomy).

Figure 3: Mass after excision about 4×3 cm.

Figure 4: Facial nerve (dotted line) after dissection of mass from it.

Histopathological examination revealed a mixture of Langerhans histocytes and eosinophil’s. CD1 and S100 are positive in immunohistochemistry (Figure 5).

Figure 5: Immunohistochemistry show CD1 and S100 are positive.

Multifocal (systemic) involvement of the patient through laboratory was excluded. Radiological investigations e.g. bone marrow aspiration, C.T. chest, abdominal sonar and skeletal surveys were used. Immediate postoperative left facial palsy occurred. Follow-up electrophysiological tests for the patient showed neuropraxia. The patient received physiotherapy for three months until complete recovery of the facial nerve palsy. Audiogram after 12 weeks showed no improvement.

Discussion

Temporal bone is involved in a range of 14 to 61% in different literature [3]. Coleman et al. [4]; note that the bony labyrinth is not affected in LCH; sensorineural hearing does not occur. This is not true as involvement of the petrous bone is less frequent, but might occur. Saliba and Sidani wrote a literature review through midline for SNHL in temporal bone LCH related articles that were published in between 1954-2008. The researchers found 6 cases bilateral, 13 cases unilateral and 15 cases with SNHL [5]. In the case in question, there is involvement of inner ear with SNHL. High resolution tomography is a gold standard for imaging temporal bone with no specific radiological finding for LCH; only bony erosion with a homogenous soft tissue [3,4] and this agree with CT in the case investigated in the current paper. Main differential diagnosis for LCD is cholesteatoma. In our case ADC of Diffusion weight MRI is 1.08×10-3 mm2/s and ADC value in cholesteatoma ranges from 1.368-0.743×10-3 mm2/s, so Diffusion weight MRI cannot differentiate LCH from cholesteatoma [6].

Surgery, chemotherapy, radiotherapy and intralesional steroid injection are used in different combination or single modality [7]. Chemotherapy is the treatment of choice in multifocal lesion. Solitary lesions of temporal bone are usually treated with surgery alone or surgical curettage of the lesions followed by radiotherapy, or local injection of steroids [8,9]. Radical temporal bone surgery has a high risk of hearing loss, labyrinthine fistula and facial nerve injury [2]. However, petrous bone lesions, which are not accessible to local steroid injection and/or not resectable, receive radiotherapy or chemotherapy [10]. In the case in question, the mass of LCH erodes mastoid bone and posterior wall of EAC conjoins mastoid cavity and middle ear as one cavity (automastoidectomy) with loss of surgical landmarks. Facial nerve was recognized and dissected with difficulty. In spite of intact facial nerve after dissection of the mass from it, patient complains of facial nerve palsy for three month until complete recovery. This may be due surgical manipulation with facial nerve during dissection of mass from it and also radiotherapy has great morbidity. So, chemotherapy is strongly recommended in treatment due to its lesser morbidity and less side effects [5].

Conclusion

LCH is rare but must be included in differential diagnosis of mass destructive lesions of temporal bone especially in children. Diffusion weight imaging MRI cannot differentiate LCH from cholesteatoma. Surgery can be done for large destructive LCH; facial nerve palsy remains a hazard.

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