Patients with interdigitating dendritic cell sarcoma (IDCS) had an excellent outcome with surgery, while adjuvant or neoadjuvant treatment did not improve overall survival in patients with follicular dendritic cell sarcoma (FDCS) or histiocytic sarcoma (HS), a recent study published online ahead of print in the European Journal of Cancer has shown.

FDCS, HS, and IDCS are extremely rare neoplasms of histocytic and dendritic cell origin, and data on the natural history and treatment outcomes of these diseases are limited. Therefore, researchers retrospectively analyzed the impact of surgery, radiation, and systemic therapies on overall survival.

For the study, researchers reviewed the charts of 31, 15, and seven patients with FDCS, HS, and IDCS, respectively, who were treated at Memorial Sloan Kettering Cancer Center in New York, NY, between 1995 and 2014.

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Results showed that the median age of patients with FDCS, HS, and IDCS was 48.7, 42.3, and 58.8 years, respectively, and IDCS predominantly occurred in men. Researchers found that the most common sites of cancer presentation were abdomen and pelvis, extremities, and head and neck, respectively. 

At the time of diagnosis, 74%, 40%, and 86% of patients with FDCS, HS, and IDCS, respectively presented with localized disease.

Researchers found that patients with FDCS and IDCS who presented with localized disease rather than metastatic disease had significantly improved overall survival.

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In regard to treatment outcomes, the study demonstrated that adjuvant or neoadjuvant therapy was not associated with improved survival vs observation in patients with FDCS and HS; however, in patients with IDCS, surgery alone provided a 71% 5-year overall survival rate.

“In the metastatic setting, chemotherapy and small molecule inhibitors may provide benefit,” the authors conclude.


  1. Gounder M, Desai V, Kuk D, et al. Impact of surgery, radiation and systemic therapy on the outcomes of patients with dendritic cell and histiocytic sarcomas. E J Cancer. 2015. [epub ahead of print]. doi: 10.1016/j.ejca.2015.06.109.