A Case of Langerhans Cell Sarcoma of the Palatine Tonsil

Langerhans cell sarcoma (LCS) is an extremely rare disease, with only a few reported cases so far of the condition involving the palatine tonsil. We report a case of a 69-year-old man with a lesion in the right palatine tonsil, who was referred to our hospital with a history of a mass detected in the right palatine tonsil two weeks earlier and multiple lymphadenopathies in the right cervical region. Biopsy revealed an increased number of Langerhans cells with prominent nuclear dysmorphism and polymorphism. Immunohistochemistry revealed positive staining for S-100 protein and CD1a. Based on these findings, we made the diagnosis of LCS. PET-CT showed strong FDG accumulation in multiple enlarged lymph nodes, as well as FDG accumulation in the lungs, liver, bones, and skin. The patient received chemotherapy and autologous peripheral blood stem cell transplantation and remains alive. LCS is a very rare disease, and there is no established treatment. Treatment options include surgery, chemotherapy, and bone marrow transplantation, selected depending on the degree of disease progression.