Langerhans Cell Histiocytosis Of The Head And Neck:90 Cases Analysis

Objective:To analyze and summarize the clinical manifestations,diagnosis,therapy and prognosis of Langerhans cell histiocytosis(LCH)which occurs in head and neck.The aim is to figure out the heterogeneity of its clinical manifestations,treatment efficiency,prognosis and related factors affecting the prognosis,and wish to provide a basis for future clinical workers and some evidence to update the treatment plan.Materials and Methods:Retrospective analysis was performed on 90 cases of head and neck LCH patients admitted to the first affiliated hospital of zhengzhou university from January 2010 to August 2019 to study their clinical manifestations,treatment and prognosis.The prognostic factors were analyzed by statistical methods.Results:(1)38 patients were suffered single-system Langerhans cell histiocytosis(SS-LCH)of head and neck,including 22 males and 16 females.The ratio of male to female was 1.4:1.The median age was 4 years old(4months-36 years).Patients with Central nervous system risk involvement were more likely to have disease progression and recurrence(?2=5.797,P=0.027);Gender(?2=0.816,P=0.469);age(?2=2.369,P=0.225).and number of organs involved(?2=1.293,P=0.279).(2)Totally 52 patients were diagnosed to have multiply-system Langerhans cell histiocytosis of head and neck(MS-LCH).The median follow-up time was 45.5 months(10months-123months).Results showed that the presentation age(?2=3.671,P=0.048)and poor response to initial induction chemotherapy of the first 6 weeks(?2=7.070,P=0.004),Gender was not independent factors affecting the prognosis of patients with MS-LCH.In 52 patients with head and neck MS-LCH(26.9%,14/52),cases with relapse/progress time ranged from 6 months to 34 months(median 12 months).31 cases of dangerous organ involvement group(MS-RO+)and 21 cases without risk organs involvement group(MS-RO-)(?2=4.038,P=0.027).Conclusion:The clinical manifestations of LCH are heterogeneous,and the etiology is still unclear.Patients with SS-LCH usually achieve a better prognosis,and the central nervous system is more likely to be blamed in the disease recurrence/progression.Risk organs involvement and poor response at 6 weeks of induction chemotherapy are the most important factors affecting the prognosis of MS-LCH patients,and second initial treatment may improve the survival rate of these patients.Early biopsy of suspected cases,early diagnosis,less misdiagnosis and early rational treatment may lead to a better prognosis for some mandible LCH patients.