Interdigitating dendritic cell sarcoma is a rare haematological neoplasm with high debatable management protocols. The data extracted from 127 case reports published between 1981 and 2018 were analysed. The median age at diagnosis was 58 years with a male to female ratio of 1.65:1. The median OS and PFS of IDCS were 12 and 6 months, respectively, with a disease-specific mortality rate of 36.4%. Two-thirds of patients had a localised disease, while 30% had a disseminated form with 1-year mortality rates of 21.1% and 78.9%, respectively. Twenty per cent of cases were associated with other malignancies. Histologically, the proliferation of large spindle-shaped cells with fascicular growth was described in 84.3% of cases. Based on Cox-regression model, surgical resection was the only treatment modality linked to survival improvement with no recorded survival benefits of radiotherapy and chemotherapy. The 1-year mortality rates in resected and non-resected disease were 17.8% and 63.2%, respectively (P < 0.0001).

中文翻译：

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相互交叉的树突状细胞肉瘤合并分析和文献回顾的新见解。

叉指状树突状细胞肉瘤是一种罕见的血液肿瘤，具有高度争议的治疗方案。分析了从1981年至2018年发布的127个病例报告中提取的数据。诊断时的中位年龄为58岁，男女之比为1.65：1。IDCS的中位OS和PFS分别为12个月和6个月，特定疾病死亡率为36.4％。三分之二的患者患有局部疾病，而30％的患者为散播性疾病，一年死亡率分别为21.1％和78.9％。20％的病例与其他恶性肿瘤有关。从组织学上讲，有84.3％的病例描述了具有束状生长的大纺锤形细胞的增殖。基于Cox回归模型，手术切除是唯一与生存改善相关的治疗方式，没有放疗和化疗的生存获益。切除和未切除疾病的1年死亡率分别为17.8％和63.2％（P <0.0001）。