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%, respec-

tively (P < 0.0001).