Zhou Jianfeng, Zhou Weixun, Bai Chunmei, Zhou Yanping, Wang Yuzhou
Department of Medical Oncology, Peking Union Medical College, Beijing, China.
Onkologie. 2011;34(11):634-7. doi: 10.1159/000334212. Epub 2011 Oct 28.
Interdigitating dendritic cell sarcoma (IDCS) is an exceedingly rare tumor. The characteristics of IDCS and its optimal therapeutic approach have not been fully clarified.
We report the case of a 53-year-old Chinese male patient presenting with a subcutaneous nodule in the right chest wall. The histological and immunohistochemical features of the nodule confirmed the diagnosis of IDCS. Complementary examination excluded other involvement of the tumor. The patient was alive without evidence of disease 1 year after tumor resection followed by radiotherapy.
With regard to the literature, IDCS presents with a wide spectrum of clinical manifestations, and its correct diagnosis requires awareness of this rare disease and the use of appropriate markers. Surgery with curative potential might remain the first treatment option, and current data do not support adjuvant therapy. Systemic chemotherapy is mainly suggested for extensive disease while the long-term efficacy is unsatisfactory. The prognosis of IDCS seems to be associated with the initial stage of disease.
指状突树突状细胞肉瘤（IDCS）是一种极为罕见的肿瘤。IDCS的特征及其最佳治疗方法尚未完全阐明。
我们报告一例53岁中国男性患者，其右胸壁出现皮下结节。结节的组织学和免疫组化特征确诊为IDCS。辅助检查排除了肿瘤的其他受累情况。该患者在肿瘤切除并接受放疗后1年仍存活，无疾病证据。
根据文献，IDCS具有广泛的临床表现，其正确诊断需要认识到这种罕见疾病并使用适当的标志物。具有治愈潜力的手术可能仍是首选治疗方案，目前的数据不支持辅助治疗。对于广泛病变主要建议进行全身化疗，但其长期疗效并不理想。IDCS的预后似乎与疾病的初始阶段有关。
