Langerhans cell sarcoma of the head and neck.

Head and neck Langerhans cell sarcoma (HNLCS) is a rare malignant tumor carrying a poor prognosis. The aim of this work was to perform a systematic review of HNLCS cases, examine outcomes, and develop an evidence-based management algorithm. We performed a systematic literature search yielding 16 studies with 17 cases of HNLCS; 33 studies with 55 Non-HNLCS were used as a comparison. Mean disease-specific survival was 20.5 months (SE ± 5.1) for HNLCS versus 26.2 months (SE±4.2) for non-HNLCS. There was no significant difference in disease-specific (p = 0.768) or disease-free survival (p = 0.880) between the two cohorts. There was a significant difference in both disease-specific (p = 0.044) and disease-free survival (p = 0.001) between local, locoregional and disseminated disease favoring more limited disease. HNLCS appears to present later, with more disseminated disease. Surgery remains the mainstay of treatment of local disease, however clear margins do not guarantee clearance.