Langerhans cell sarcoma (LCS) is a tumor originating in the dendritic cell system. LCS was initially defined by the International Lymphoma Study Group in 2002. Before that time, multiple terms were used to describe this entity, leading to conceptual confusion. Since 2002, only a few cases have been reported in the literature. It can involve soft and hard tissues and has variable clinical manifestations depending on the site of involvement. An aggressive clinical course and overtly malignant cytologic features are the known characteristics of the disease. Patients with multiorgan involvement have a significantly poorer prognosis compared with those with single-organ disease. Early detection and accurate diagnosis are important for improving the prognosis. Consensus for optimal standard therapy has not been established in LCS. The authors report a case of a 56-year-old male patient with primary LCS of the tonsils who experienced local recurrence and lymphatic metastasis after multiple surgeries and adjuvant chemoradiotherapy during a 9-year follow-up.This case represents the longest survival and follow-up duration of LCS occurring in the head and neck region reported to date. On the basis of the summary of this case’s treatment experience and a review of the literature, for local and locoregional head and neck cases where surgery is the primary approach, efforts should be made to achieve negative margins. Concurrent selective neck dissection or sentinel lymph node biopsy should be performed to evaluate and rule out locoregional disease. If histology confirms nodal involvement, further radical neck dissection should be considered, accompanied by adjuvant radiotherapy at doses typical for other sarcomas. For patients with disseminated disease, systemic therapy and potentially hematopoietic stem cell transplantation have been associated with positive outcomes.
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