Eosinophilic Granuloma of the Temporal Bone in Children

Abstract

Eosinophilic granuloma (EG) is a bony destructive disease that frequently occurs in children; it is a subtype of Langerhans cell histiocytosis. The aims of this study were to detect the presenting features of temporal bone lesions in children and to evaluate the efficacy of surgery combined with radiotherapy in treatment of the disease. A retrospective study on 12 children with EG of the temporal bone was done. Computed tomography and hearing assessment were performed for all patients. All patients were treated with cortical mastoidectomy followed by postoperative radiotherapy. Follow-up was carried out for at least 2 years. The patients' presenting symptoms were external ear canal mass in 10 patients (83.3%), postauricular swelling in 8 patients (66.7%), and persistent otorrhea in 4 patients (33.3%). Ten patients (83.3%) showed conductive hearing loss, whereas 2 patients (16.7%) showed mixed hearing loss on the affected side. Computed tomography showed osteolytic defects without sclerotic margins filled with soft tissue masses involving the mastoid bone. Histopathologic examination showed eosinophils and Langerhans cells that were immune reactive for CD1 antigen and S-100 protein. Postoperative follow-up showed complete cure of the disease in 10 children (83.3%), with recurrence detected in 2 patients (16.7%) who needed second surgical intervention. We concluded that temporal bone EG in children may present with features that mimic the features of chronic suppurative otitis media. However, computed tomography and histopathologic examination are diagnostic. Cortical mastoidectomy together with postoperative radiotherapy is an achievable treatment in most cases.

Incidence and radiological pattern of eosinophilic granuloma: a retrospective study in a Chinese tertiary hospital

“…On plain radiography and CT imaging, perforated or osteolytic bone destruction, clear border, regular or irregular morphology, and very few marginal hardening were also manifested. In the skull, the lesions developed in the diploic space are lytic, and their edges may be beveled, scalloped, or confluent, or show the “buttons like” sequestrum, or the “beveled edge” with the soft tissue mass [9, 11]. In 3 EG cases diagnosed with MRI, low T1WI signal and high T2WI signal were revealed and intracranial masses were also revealed with local dura invagination (Fig.…”

“…However, the periosteal reaction was different from that of malignant bone tumors. It was natural and continuous, and the layers were more parallel and well-proportioned, often in a range larger than the extent of bone destruction [11, 13–15]. Osteolytic destruction and soft tissue mass were also revealed in one MRI examination.…”

Facial nerve paralysis as initial symptom of langerhans cell histiocytosis

“…Involvement of the inner ear and sensorineural hearing loss are less common presenting symptoms due to compact nature of petrous apex and otic capsule. 4 …”

Langerhans Cell Histiocytosis of the Skull

“…3,4 Otologic manifestations include otorrhea resistant to medical treatment, preauricular mass, aural polyps, mastoid swelling, and/or hearing loss. 4,5…”

“…Computed tomography imaging of the skull classically reveals aggressive lytic osseous lesions with extensive destruction of bone, but without surrounding periosteal reaction or reactive sclerosis. 3,5 These radiologic findings are typically out of proportion to the clinical findings. Despite the extensive erosion present, involvement of the ossicles or inner ear is rare in eosinophilic granuloma.…”