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Korean J Hematol. 2012 Jun;47(2):150-153. English. Case Report. https://doi.org/10.5045/kjh.2012.47.2.150
Lee SY , Lee SR , Chang WJ , Kim HS , Kim BS , Kim IS .
Department of Hemato-oncology, Korea University Medical Center, Anam Hospital, Seoul, Korea.
Department of Pathology, Korea University Medical Center, Anam Hospital, Seoul, Korea. iskim@korea.ac.kr
Abstract

Interdigitating dendritic cell sarcoma (IDCS) is a very rare and aggressive neoplasm that arises from antigen presenting cells. IDCS usually involves lymph nodes; however, extra-nodal involvement has also been reported. Because a consistent standard therapy for IDCS has not been established to date, we report a case of the successful treatment of disseminated IDCS using ABVD chemotherapy (doxorubicin, bleomycin, vinblastine, and dacarbazine). A 64-year-old man was diagnosed with IDCS on the basis of immunohistochemical findings of a biopsy specimen of the inferior nasal concha. Immunohistochemical staining showed a positive reaction for CD68, leukocyte common antigen, and S-100 protein, but a negative reaction for CD34, CD1a, and CD21. Imaging studies showed cervical and axillary lymphadenopathies, subcutaneous nodules, and a soft tissue lesion in the nasal cavity. Treatment with the ABVD regimen resulted in complete remission after 8 cycles of chemotherapy.

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