OBJECTIVETo describe the clinical and pathological features, treatment and prognosis of dendritic cell sarcoma (DCS).

METHODSA group of DCS was described, including two cases of follicular dendritic cell sarcoma (FDCS), one each of interdigitating dendritic cell sarcoma (IDCS) and langerhans cell sarcoma (LCS). The related English literatures were reviewed.

RESULTSTwo patients with IDCS were a 19-year-old man and a 45-year-old woman respectively, both exhibited fever of unknown origin and painless lymphadenopathy. Pathological diagnosis of lymph node biopsy was FDCS with positive CD21 and CD35. Both patients achieved complete remission (CR) after 6 cycles of chemotherapy (CHOP: cyclophosphamide, epirubicin, vindesine, and prednisolone). However, the male patient relapsed 5 months later and another patient was still in CR at 5 months follow-up. One case of IDCS was a 42-year-old man, who manifested as paraneoplastic pemphigus. Biopsy of mediastinal lymph node demonstrated IDCS and immunohistochemistry showed positive S-100 staining. This patient died of pneumonia after two cycles of CHOP. One patient of LCS was a 54-years-old woman with fever, painless lymphadenopathy and diffused pulmonary nodules. The diagnosis of LCS was established after excisional biopsy was taken from inguinal lymph node. Positive staining of CD1a and S-100 was displayed by immunohistochemistry. Electron microscope examination confirmed the presence of Birbeck granule in tumor cells. Four cycles of chemotherapy (including ECHOP, FND) were administered, but the disease progressed.

CONCLUSIONDCS is a group of very rare sarcoma, FDCS, IDCS and LCS have different characteristic clinical features, immunophenotype and prognosis. The prognosis of most patients is poor.