|Year : 2016 | Volume
| Issue : 2 | Page : 139-142
Eosinophilic granuloma of temporal bone
Sachin Gupta1, Ghulam Mohammad Mir2, Parmod Kalsotra2, Pallavi Kaul2
1 Department of ENT and HNS, ASCOMS, Sidhra, Jammu and Kashmir, India
2 Department of ENT and HNS, SMGS, GMC, Jammu and Kashmir, India
|Date of Web Publication||11-May-2016|
Ward No 6, Karan Nagar, Udhampur - 182 101, Jammu and Kashmir
Source of Support: None, Conflict of Interest: None
Eosinophilic granuloma is an uncommon granulomatous disease which can affect the temporal bone. Also known as Langerhans cell histiocytosis, the lesion is characterized by uncontrolled proliferation of Langerhans cells. Although initially silent, the disease may erode the mastoid cortex, destroy the tegmen, and extend into the cranial vault, as well as erode the semicircular canals or cochlea. These lesions almost always become infected and can be confused with chronic otomastoiditis. Equally important, temporal bone involvement may represent only one manifestation of a multifocal disease. This report describes a case of 40-year-old male with eosinophilic granuloma involving the right temporal bone extending into midbrain region causing focal compression and displacement of part of the temporal lobe.
Keywords: Eosinophilic granuloma, Histiocytosis, Temporal bone
|How to cite this article:|
Gupta S, Mir GM, Kalsotra P, Kaul P. Eosinophilic granuloma of temporal bone. Indian J Otol 2016;22:139-42
| Introduction|| |
Eosinophilic granuloma is a rare, benign lesion characterized by uncontrolled proliferation of Langerhans cells. Langerhans cells are differentiated cells of the dendritic cell system and are closely related to the monocyte-macrophage line. These antigen-presenting cells are normally found in the skin, reticuloendothelial system, heart, pleura, and lungs. When bones are involved it is usually found at flat and long bones. The most frequent sites of involvement are the skull, spine, ribs, femur, and pelvis.,, They may be identified by immunohistochemical staining or by the presence of Birbeck granules via electron microscopy.
Langerhans cell histiocytosis (LCH) comprises three different entities: Eosinophilic granuloma, Hand–Schüller–Christian disease, and Letterer–Siwe disease. In 1953, Lichtenstein grouped this disease spectrum under the name histiocytosis X. The “X” referred to the fact that the etiology was unknown. In 1987, the term “LCH” was introduced by the “Writing Group of the Histiocyte Society.” Eosinophilic granuloma is the most common form of LCH and accounts for 70% of the cases of LCH. It is a solitary lesion, usually confined to skeleton with favorable prognosis. Children and young adults are most commonly affected, although the disorder can affect any age group. Male to female ratio is 2:1. Etiology is unknown. Trauma, hereditary pattern, metabolic dysfunction, and viral infections have all been suspected. However, none of them substantiated as yet proliferation of Langerhans cells is monoclonal, so the neoplastic process is possible. An autoimmune mechanism is also proposed. The clinical picture is variable. Eosinophilic granuloma can be asymptomatic or can present with swelling' tenderness and localized pain.
Temporal bone lesions may occur either isolated or as a part of multifocal disease. Isolated temporal bone involvement is a relatively rare entity.,,, In this report, we outline the clinical presentation, diagnosis, and treatment of a patient with eosinophilic granuloma isolated to the temporal bone.
| Case Report|| |
A 40-year-old male presented with a history of hearing loss in the right ear since 1 year. It was insidious in onset but gradually progressive. He also noticed a slow growing painless mass in the right external auditory canal (EAC) since 2 months. He did not have any vertigo, tinnitus, or facial weakness. There was no history of previous ear infection or trauma. Past medical history of the patient did not reveal any significant ailment. His family history was unremarkable. There was no family history of bone disease, skin disorders, genetic abnormalities, or endocrinopathies.
On physical examination, right-sided otoscopy revealed mildly tender polyp localized to the posterior and superior walls of the EAC that prevented visualization of the right tympanic membrane. There was no mastoid tenderness. Left-sided otoscopy showed mild retraction of pars tensa. The remainder of the head and neck examination was totally normal. Medical therapy with a wide spectrum of antibiotics was introduced, but the condition was refractory to medical treatment.
Audiological examination demonstrated a moderately severe mixed hearing loss with a pure tone air conduction average of 105 dB hearing level (masked) and a 60 dB air-bone gap on his right ear. The routine laboratory tests were within the normal limits.
Computed tomography (CT) scanning of the temporal bone revealed extensive erosion involving the petrous, mastoid and squamous parts of right temporal bone, and sphenoid wing posteriorly with a well-defined hyperdense soft tissue component. The soft tissue is seen extending into subgaleal tissue and infratemporal fossa along with intracalvarial extension [Figure 1].
|Figure 1: High-resolution computed tomography scan of the temporal bone in the axial plain|
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Contrast enhanced magnetic resonance imaging (MRI) showed large lytic expansile mass lesion involving the right temporal bone as well as the right mastoid ear cells extending into midbrain region causing focal compression and superomedial displacement of the right inferolateral temporal bone [Figure 2] and [Figure 3].
|Figure 2: T1 and T2 axial magnetic resonance imaging of the temporal bone|
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Simple polypectomy was performed, and a biopsy specimen was then obtained. The diagnosis of an inflammatory polyp rich in eosinophilia was established after histopathological examination.
The patient underwent radical mastoidectomy. The mass was completely removed. The eroded ossicles were removed and middle ear sealed with temporalis fascia graft. The mastoid cavity was obliterated with gelfoam. Meatoplasty was done. The wound was closed in layers The patient tolerated the procedures well, and no complications occurred in the postoperative period.
Histopathological examination of the mass revealed rather large histiocytes with increased plasma cells and eosinophils and fibrous tissue. Based on these findings, the case was diagnosed as eosinophilic granuloma [Figure 4].
|Figure 4: Postoperative histologic section showing granulomatous inflammation with histiocytes, plasma cells, and eosinophilic cell infiltrate|
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After surgery, pure tone air conduction average improved to 20 dB hearing level. Complete body survey to consider metastasis with whole body bone scintigraphy was normal, but follow-up CT evaluation of the temporal bone 1 month postoperatively revealed a suspicious area of soft tissue density in the squamous portion of the temporal bone. The patient underwent low-dose radiation therapy (10 Gy).
| Discussion|| |
Eosinophilic granuloma, which is a subtype of LCH, can manifest as either single or multiple bony diseases.,, Unifocal eosinophilic granuloma of bone usually occurs before age 10 years, but may also be seen in adults.,, Males are slightly more affected than females. The causative factor of this disease is still unknown. More recently, the evidence seems to favor the concept of a deranged immune response leading to hyperplasia of Langerhans cells.
Eosinophilic granuloma of the temporal bone may present as an aural polyp or granulation tissue in the EAC. The granulation tissue in the EAC is found along the posterior bony canal wall. The initial symptoms of eosinophilic granuloma include otalgia, otorrhea, hearing impairment, and postauricular swelling. Hearing loss, if present, is usually conductive type. Sensorineural deafness may also be seen if destruction of the bony labyrinth occurs., The disease usually tends to spare the tympanic membrane and the ossicular chain. Perforation of the tympanic membrane is more likely to result from secondary infection of the ear. Vertigo due to the destruction of the otic capsule, and facial paralysis due to a compromised blood supply are seen in fewer than 5% of the patients. Destruction of bone may allow tumor invasion into the middle or posterior cranial fossas. Appling et al. stated that “granulations appearing in a fistula in the posterior wall of the EAC in the presence of an intact tympanic membrane are strongly suggestive of eosinophilic granuloma or Hand–Schüller–Christian disease”.
Differential diagnosis of eosinophilic granuloma includes suppurative otitis/mastoiditis, cholesteatoma, external otitis, primary bone tumors, and metastatic tumors. Laboratory and radiologic evaluation must serve to rule out infectious or metabolic processes, neoplastic diseases, and cholesteatoma. If eosinophilic granuloma is suspected, the possibility of systemic involvement or multiple bony lesions should be considered. For this reason, the investigation should include hematologic evaluation, erythrocyte sedimentation rate, liver function tests, urinalysis, chest X-ray, bone scintigraphy, and metastatic series. CT also better defines the soft tissue margins of the granulomatous mass in relationship to the central nervous system and extratemporal sites., The lesion tends to have sharp borders with a punched out appearance. Involvement of both the inner and outer tables results in a double-contour or beveled edge appearance. At times a button sequestrum may be present within the osteolytic lesion, representing residual bone. MRI helps in the better demarcation of the soft tissues and relationship of the tumor to the dura with the possibility of infiltration.
The histopathological diagnosis of Eosinoplilic granuloma (EG) is established on the basis of morphologic, immunochemical, and ultrastructural features. Grossly, the tumor mass is fragile and faintly yellow to reddish-brown and may contain areas of necrosis or hemorrhage. Microscopically, numerous large round histiocytes with rounded, often indented, nuclei are surrounded by variable numbers of eosinophils with occasional neutrophils, lymphocytes, and plasma cells. Multinucleated giant cells are usually present. Immunochemical staining usually provides a positive diagnosis with CD1 antigen, S-100 protein, ATPase, alfa-D-mannosidase, and peanut lectin. Cytoplasmic organelles, termed Birbeck granules, are highly specific in Langerhans' cell but can only be seen by electron microscopy.
Treatment depends on the extent of disease at the time of diagnosis. Single lesions can be successfully treated with either surgery or radiotherapy alone., Although controversial, some authors report good results with local injections of steroids, avoiding radiation in children., Spontaneous remission is also reported. For multisystemic disease, besides surgery and local radiotherapy, chemotherapy may be required. In our case, we performed radical mastoid surgery. Low-dose radiation therapy (10–20 Gy) was added due to close proximity of tumor to the dura. The prognosis is self-limiting and excellent in the absence of nonosseous involvement or multifocal process., Local recurrence rate is 6%, and new lesions appear in about 22% of patients, so long-term follow-up is recommended for early detection of recurrence or progression of the disease to new bony lesions or systemic involvement. Adverse prognostic factors are young age and multiorgan involvement and organ dysfunction. Bilateral simultaneous involvement occurs quite rarely.
| Conclusion|| |
Eosinophilic granuloma has a favorable prognosis, and its treatment should be individualized based on the localization of the lesion and age of the patient. In the management of solitary eosinophilic granuloma, it is important to rule out multifocal or disseminated forms of LCH. Early detection and multidisciplinary approach are imperative to plan the best management strategy for the best possible outcome of the disease.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]