Lee Suk-Young, Lee Se Ryeon, Chang Won Jin, Kim Hye Sook, Kim Byung Soo, Kim In Sun
Department of Hemato-oncology, Korea University Medical Center, Anam Hospital, Seoul, Korea.
Korean J Hematol. 2012 Jun;47(2):150-3. doi: 10.5045/kjh.2012.47.2.150. Epub 2012 Jun 26.
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.
交指状树突状细胞肉瘤（IDCS）是一种非常罕见且侵袭性强的肿瘤，起源于抗原呈递细胞。IDCS通常累及淋巴结；然而，也有结外受累的报道。由于迄今为止尚未建立针对IDCS的一致标准治疗方案，我们报告一例使用ABVD化疗（阿霉素、博来霉素、长春碱和达卡巴嗪）成功治疗播散性IDCS的病例。一名64岁男性根据下鼻甲活检标本的免疫组化结果被诊断为IDCS。免疫组化染色显示CD68、白细胞共同抗原和S-100蛋白呈阳性反应，但CD34、CD1a和CD21呈阴性反应。影像学检查显示颈部和腋窝淋巴结肿大、皮下结节以及鼻腔软组织病变。采用ABVD方案治疗8个周期化疗后完全缓解。
