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ABSTRACT: 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.
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.
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%.
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.
International Journal of Pediatric Otorhinolaryngology 07/2008; 72(6):775-86. DOI:10.1016/j.ijporl.2008.02.001 · 1.32 Impact Factor
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ABSTRACT: Rhabdomyosarcoma is the most frequent soft tissue sarcoma in the pediatric age group. The authors present their series of rhabdomyosarcoma of the temporal bone in children at Saint Justine Hospital. The twofold objective of this study is to illustrate the clinical presentation, management, and prognosis of this malignant striated muscle tumor, and to compare these results with previously reported series.
A retrospective study was conducted of patients diagnosed and treated for rhabdomyosarcoma of the head and neck at Saint Justine Hospital, a tertiary pediatric center, between 1970 and 2005. Only cases of temporal bone rhabdomyosarcoma were included in the study. A thorough review of medical and surgical charts was performed to obtain demographic, clinical, paraclinical, and therapeutic data, which were subsequently analyzed and compared to published results. A MEDLINE search yielded 34 studies dealing with temporal bone rhabdomyosarcoma since the year 1966.
Thirty-nine patients with rhabdomyosarcoma of the head and neck region were identified, among which only six children had temporal bone rhabdomyosarcoma. The mean age at the time of diagnosis was 4.15 years. Chronic otitis media was the most common clinical presentation. Five children had the embryonal subtype and one had the botryoid subtype on histology. All patients except two received combined chemotherapy and radiotherapy as treatment. Five-year survival rate was 66%. Our results match those reported in the literature 41-81%.
Rhabdomyosarcoma of the temporal bone is an aggressive tumor that clinically simulates chronic otitis media. A high index of suspicion should be raised in the context of otitis media that is unresponsive to ordinary medical treatment. A biopsy is hence recommended in the presence of polyps in the external auditory canal that are resistant to medical treatment. Early diagnosis and the adoption of multimodal therapy offer the best outcome.
International Journal of Pediatric Otorhinolaryngology 06/2007; 71(5):807-14. DOI:10.1016/j.ijporl.2007.02.002 · 1.32 Impact Factor
The Journal of otolaryngology 07/2000; 29(3):179-82. · 0.50 Impact Factor