Langerhans’ cell histiocytosis of the temporal bone in children

Summary Objective Langerhans’ cell histiocytosis (LCH) is a rare pathology that implies an abnormal proliferation of these kinds of cells associated with a granular infiltration that affects different structures of the human body, including the temporal bone. The authors present their series of LCH of the temporal bone in children at the Sainte-Justine university hospital. The twofold objective of this study is to illustrate the clinical presentation, management and prognosis of this disease, and to compare these results with previously reported series. Methods A retrospective study was conducted between 1984 and 2007 with patients diagnosed and treated for a LCH of the temporal bone at the Sainte-Justine university hospital, a paediatric tertiary care center. A chart review was performed to obtain demographic, clinical, paraclinical, and therapeutic data. They were analysed and compared to other published series. Through a MEDLINE query, we found that since 1966, 50 articles dealing with a LCH of the temporal bone have been published. Results Fifty-nine cases of LCH were diagnosed and among them, 10 children had temporal bone involvement. They were four females and six males with a mean age of 3.28 years. The two most frequent clinical manifestations were the presence of a mass in the temporal region (70%) and otitis (60%). Two of our patients had a unifocal lesion of the temporal bone implicated. Eight patients had a multisystem involvement among which, two showed evidence of organ dysfunction. In 80% of cases, the diagnosis was made by immunohistochemical findings of the S-100 protein and/or the CD1 antigen. The common radiological finding on a skull CT scan is a lytic lesion in the temporal bone. Seven patients were treated by chemotherapy, two were treated by radiotherapy as a primary treatment, and one received radiotherapy for a recurrence on the pituitary gland. Finally, one patient was treated with local steroid injections. Two patients had a recurrence. All our patients were in total remission on a mean average of 1.6 years after the diagnosis. Our results concord with other studies in which the prognosis of unifocal bone disease is excellent and children with a multifocal disease have a survival rate of 65–100%. Conclusion LCH is a rare disease. A high-index suspicion should be raised in the context of a temporal mass, chronic otitis, and otorrhea. A biopsy is recommended in the presence of a temporal bone lytic lesion. Chemotherapy is the preferred therapeutic modality.