ABSTRACT
Langerhans cell sarcoma (LCS) is a rare malignant tumor of Langerhans cells and uncommonly involves head and neck regions. Unlike Langerhans cell histiocytosis (LCH), it has an aggressive clinical course with malignant cytological features. Till now, a handful of cases have been reported and the common anatomical sites involved are skin, lymph node, and bone in loco – regional cases and lymph node, lung, liver, spleen, and bone in disseminated disease. Due to its rarity, standard protocols of treatment for these patients are not yet well established. Herein, we report such a case in a 25-year-old male presenting with a bilateral submandibular swelling, which was diagnosed as LCH on Fine Needle Aspiration Cytology (FNAC) and later confirmed to be a case of LCS in histopathological examination and immunohistochemistry. The authors are aware of only a single similar case being reported in the English literature.