Langerhans cell sarcoma of the nasopharynx: a rare case.

Langerhans cell sarcoma, a tumour with markedly malignant cytological features that originates from Langerhans cells, is a very rare disease. We report the first case of 39-year-old male with Langerhans cell sarcoma arising in the nasopharynx. We chose the 2-chlorodeoxyadenosine (2-CDA) regimen as first-line chemotherapy, and clinical improvement of Langerhans cell sarcoma was obtained. After the fourth cycle of 2-CDA therapy, however, disease progression was observed, and we administered ESHAP regimen (etoposide, carboplatin, cytarabine, methylprednisolone) as a second-line therapy. After we administered two cycles of ESHAP, however, the patient developed aggressive progression and he died. The importance of immunohistochemical findings is obvious in Langerhans cell sarcoma diagnosis. Considering that Langerhans cell sarcoma behaves in a very malignant fashion, a more aggressive treatment approach is necessary for patients with Langerhans cell sarcoma.