Langerhans cell sarcoma (LCS) is extremely rare, with only 36 cases reported in English literature. In this report we represent the case of a 77-year-old woman with a 1-month history of left neck swelling and pain. A diagnosis of LCS was rendered from pathological findings of the cervical lymph node biopsy. The patient’s condition deteriorated rapidly and she died 2 days after diagnosis. A literature review in the context of the present case was performed to better enhance understanding of the early diagnosis and treatment of this unusual lesion.
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