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Langerhans' cell histiocytosis of the temporal bone in children
I Saliba1, K Sidani, F El Fata
1ENT Otology and Neurotology - CHU Sainte-Justine, 3175, Côte Sainte-Catherine, Service ORL, Montréal, QC H3T 1C5, Canada. issam.saliba@umontreal.ca
Insights
Langerhans cell histiocytosis (LCH) of the temporal bone is rare in children. Early diagnosis via biopsy and chemotherapy offer excellent prognosis, especially for unifocal bone disease.
Area of Science:
- Pediatric Pathology
- Oncology
- Otolaryngology
Background:
- Langerhans cell histiocytosis (LCH) is a rare disorder characterized by abnormal proliferation of Langerhans cells.
- Temporal bone involvement in LCH presents unique diagnostic and management challenges in pediatric patients.
Purpose of the Study:
- To present a series of pediatric temporal bone LCH cases.
- To illustrate the clinical presentation, management, and prognosis of temporal bone LCH.
- To compare findings with existing literature.
Main Methods:
- Retrospective chart review of pediatric patients diagnosed with temporal bone LCH (1984-2007).
- Data collected included demographics, clinical presentation, diagnostic methods, treatment, and outcomes.
- Comparative analysis with published series via MEDLINE search.
Main Results:
- Ten pediatric cases of temporal bone LCH identified, with common symptoms including temporal masses and otitis.
- Diagnosis often confirmed by S-100 protein and CD1 antigen immunohistochemistry; CT scans revealed lytic lesions.
- Chemotherapy was the primary treatment; prognosis was excellent for unifocal disease, with a 65-100% survival rate for multifocal disease.
Conclusions:
- High suspicion for LCH is warranted with temporal masses, chronic otitis, or otorrhea in children.
- Biopsy is crucial for diagnosing temporal bone lytic lesions.
- Chemotherapy is the recommended treatment modality for LCH of the temporal bone.
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.