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[Langerhans cell histiocytosis of the temporal bone: 22 cases analysis]
Liang Chen1, Wu-qing Wang, Hui Xu
1Department of Otorhinolaryngology, EENT Hospital of Fudan University, Shanghai 200031, China.
Objective:
To analyze the clinical manifestations, diagnosis, therapy and prognosis of Langerhans cell histiocytosis (LCH) of the temporal bone.
Methods:
Twenty-two patients with LCH of the temporal bone in our hospital were retrospectively summed up from January 1994 to January 2008. Following up 1 - 15 years, their clinical manifestations, diagnosis, therapy and prognosis were studied. Survival analysis and disease free survival Log-rank test were used.
Results:
Among 22 patients, 1 case belonged to the multisystem high risk group, 3 cases to the multisystem low risk group, and 18 cases to the single system group. The clinical characters were primarily the ear presentations, CT of the temporal bones showed extensive osteolytic destructions. The misdiagnosis rate was 72.7%. Twenty-two cases were received different combined modality therapies. Following up 1 - 15 years, 21 cases were survival and 1 case dead (4.5%), while 5 cases showed residual of insipidus, dwarfism, epilepsy or unilateral serious hearing loss (22.7%). For disease free survival curve, there was a significant difference between the multisystem group and the single system group (chi(2) = 5.87, P < 0.05).
Conclusions:
As LCH of the temporal bone, the single system cases are predominant. The ear area clinical manifestations are primary. This disease is easily misdiagnosed. The therapy selection should rely on the involved systems of the disease. The prognosis of the single system group is much better than the multisystem group.