Clinical Analysis Of 34 Cases Of Langerhans Cell Histiocytosis Of The Temporal Bone

ObjectiveTo investigate the clinical characteristics of Langerhans cell histiocytosis(LCH)of the temporal bone.MethodsThe clinical data of 34 patients with LCH who confirmed by pathology admitted to the First Affiliated Hospital of Zhengzhou University from November 2011 to August 2019 were analyzed retrospectively,including gender,age of onset,ototemporal symptoms and other clinical manifestations,blood routine and liver function indicators,imaging results,otology results,treatment plan and prognosis.According to the Lavin-osband score,the patients in this study were divided into groups according to the age of onset and the number of organs involved,because no evidence of organ damage was found.According to the age of onset,the patients were divided into two groups,the patients who younger than 2-year-old are group B,the patients who older than 2-year-old are group A.The clinical data of the two groups were statistically analyzed to compare whether there was statistical significance between the two groups in ototemporal symptoms and efficacy prognosis.According to the clinical classification,the patients were divided into single-system involvement group and multi-system involvement group,and the differences between the two groups of ototemporal symptoms and efficacy prognosis were analyzed.Results1.The ratio of male to female was 1.4:1 and the median age was 22.5 months in 34 patients.Among the 34 cases,there were 8 patients with bilateral involvement,accounting for 23.53%of the patients in this group.About 67.65%of the patients were single system involvement and 32.35%were multi system involvement.2.Clinical manifestations:?Ototemporal symptoms:The most common ototemporal symptoms were otorrhea and ototemporal masses,followed by neoformation of the external auditory canal,otodynia and hearing loss,without facial paralysis.The most common symptom of ototemporal region was otorrhea,followed by ototemporal mass in patients under 2 years old,while the most common symptom of ototemporal region was ototemporal mass in patients over 2 years old,followed by otorrhea.There was no statistically significant difference between the two groups of different age(p>0.05).The most common ototemporal symptoms were otorrhea and ototemporal mass both in the single system involvement and multi system involvement groups,there was no statistically significant difference between the two groups(p>0.05).? The most common symptoms of without ototemporal region in patients with multiple system involved LCH are rash and Cervical lymph nodes swelling.3.In this research,the most frequently damaged part was mastoid(34/34),the second common damaged part was osseous external auditory canal(32/34),the middle ear is often involved(30/34)and the inner ear was less involved(2/34),the auditory ossicles and facial nerves were seldom involved.4.In this study,11 patients(32.35%)with multiple system involvement and 7 patients(20.59%)with single system involvement and multiple focus underwent biopsy combined chemotherapy,16 patients(47.06%)with single system involvement and single focus underwent temporal tumor resection combined chemotherapy,and the chemotherapy generally adopts VP scheme,the patients in high risk were treated with vinorelbine+prednisone+etoposide+methotrexate.5.In this research,29 patients(29/34,85.29%)had better response,4 patients(4/34,11.76%)had moderate response and 1 patient(1/34,2.94%)had worsening response.There was no significant difference in the therapeutic effect between patients under 2 years old and patients over 2 years old(p>0.005).There was significant difference in the curative effect between patients with multiple system involvement and patients with single system involvement(p<0.05).Conclusions1.LCH of the temporal bone occurs mostly in children.Single system involvement is common,and multiple system involvement involves skin and neck lymph nodes except temporal bone.2.In LCH of the temporal bone,the mastoid and the posterior wall of the osseous external auditory canal were most often involved,and the ossicles,facial nerve and inner ear were less involved.The most common ototemporal symptoms are ototemporal mass and otorrhea.The severity of symptoms was not related to age and clinical classification.3.The patients with multiple system involvement and single system involvement with multiple focus underwent systemic chemotherapy after pathology,the patients with single system involvement with single focus underwent temporal tumor resection combined chemotherapy.The chemotherapy generally adopts VP scheme,the overall prognosis of patients is better,and the prognosis of patients with multiple system involvement is worse than that of patients with single system involvement.Age ?2 years is not a risk factor for prognosis.