Clinical and prognostic analysis of Langerhans cell histiocytosis in oral maxillofacial head and neck: report of 36 consecutive cases

doi:10.19438/j.cjoms.2021.01.011

Abstract

Abstract: PURPOSE: This retrospective study was performed to analyze and discuss the clinical characteristics, diagnosis, treatment and prognosis of Langerhans cell histiocytosis(LCH) in oral maxillofacial head and neck region, in order to provide evidence-based evidence for clinical treatment. METHODS: The clinical data of 36 cases of LCH of adults and children treated in the Department of Oromaxillofacial Head and Neck Oncology, Shanghai Ninth People's Hospital were retrospectively analyzed. The prognosis of LCH was analyzed by SPSS 22.0 software package. RESULTS: The median age of 36 LCH patients was 31 years (8 months to 74 years). The main clinical manifestations were oral mucosal ulcer or granuloma, accompanied by pain and swelling; craniomaxillofacial bone damage was chisel like destruction; gingival necrosis, tooth displacement, tooth loss and alveolar bone destruction. 22% patients had history of diabetes insipidus. According to the scoring standard of Lavin osband, 26 cases were classified as grade Ⅰ, 9 as grade Ⅱ, 1 as grade Ⅲ and 0 as grade Ⅳ. The most common sites were jaw, gum and palate. Twenty-three patients received single treatment, 13 patients received combined treatment. The median follow-up time was 22.5 months, 1 patient was lost to follow-up, 1 patient died. The complete remission rate was 75% after initial treatment. The 5-year relapse free survival rate was 53.78%; 20 patients received systemic therapy; 17 patients had single system involvement, 19 patients had multiple systems involvement, 24 patients underwent detection of BRAF-V600E mutation, 6 patients were positive, and 1 patient took targeted drug orally and the effect was significant. CONCLUSIONS: ① The initial treatment of oral and maxillofacial head and neck Langerhans cell histiocytosis is good, the ultimate goal of the treatment is to treat the disease and prolong the time of recurrence. ② Treatment has significant impacts on the overall survival of patients. Chemotherapy can prolong the survival time of patients without recurrence. After pathological diagnosis, it is suggested that the treatment should be determined according to the involvement of organ system after PET-CT evaluation of systemic condition. ③ For patients whose first-line and second-line responses are not obvious, detecting BRAF-V600E gene mutations and choosing targeted drug therapy according to the results.

Key words: Langerhans cell histiocytosis, Clinical characteristics, BRAF-V600E, Prognosis

CLC Number:

R739.8

YANG Ran-ran, JU Hou-yu, REN Guo-xin, GUO Wei. Clinical and prognostic analysis of Langerhans cell histiocytosis in oral maxillofacial head and neck: report of 36 consecutive cases[J]. China Journal of Oral and Maxillofacial Surgery, 2021, 19(1): 52-58.

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