title: Impact of surgery, radiation and systemic therapy on the outcomes of patients with dendritic cell and histiocytic sarcomas.
abstract: BACKGROUND
  Neoplasms of histiocytic and dendritic cell origin, including follicular dendritic cell sarcoma (FDCS), histiocytic sarcoma (HS) and interdigitating dendritic cell sarcoma (IDCS), are extremely rare, and data on their natural history and treatment outcomes are sparse. We evaluated the impact of surgery, radiation and systemic therapies on overall survival (OS).
  
  
  METHODS
  We conducted a retrospective chart review of patients with FDCS, IDCS and HS treated at Memorial Sloan Kettering Cancer Center between 1995 and 2014.
  
  
  RESULTS
  We identified 31, 15 and 7 patients with FDCS, HS and IDCS, respectively. Median age was 48.7, 42.3 and 58.8years for FDCS, HS and IDCS, respectively. Only a slight disparity in gender distribution existed for FDCS and HS; however, IDCS predominantly affected males (6:1). The most common sites of presentation were abdomen and pelvis (42%), extremities (33%) and head and neck (57%) for FDCS, HS and IDCS, respectively. At diagnosis, 74%, 40% and 86% of patients presented with localised disease in FDCS, HS and IDCS, respectively. Patients with localised disease had significantly improved OS than those with metastatic disease in FDCS (P=0.04) and IDCS (P=0.014) but not in HS (P=0.95). In FDCS and HS, adjuvant or neo-adjuvant therapy was not associated with improved OS compared with observation. In IDCS, surgery alone provided a 5-year overall survival rate of 71%.
  
  
  CONCLUSIONS
  Adjuvant or neo-adjuvant treatment in FDCS and HS did not affect OS. Patients with IDCS had an excellent outcome with surgery. In the metastatic setting, chemotherapy and small molecule inhibitors may provide benefit.
authors: Department of Medicine, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, NY 10065, USA. Electronic address: gounderm@mskcc.org.
authors: Mrinal
authors: M
authors: Gounder
authors: Department of Medicine, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, NY 10065, USA.
authors: Ved
authors: V
authors: Desai
authors: Department of Biostatistics, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, NY 10065, USA.
authors: Deborah
authors: D
authors: Kuk
authors: Department of Pathology, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, NY 10065, USA.
authors: Narasimhan
authors: N
authors: Agaram
authors: Department of Pathology, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, NY 10065, USA.
authors: Maria
authors: M
authors: Arcila
authors: Department of Pathology, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, NY 10065, USA.
authors: Benjamin
authors: B
authors: Durham
authors: Department of Medicine, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, NY 10065, USA.
authors: Mary L
authors: ML
authors: Keohan
authors: Department of Medicine, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, NY 10065, USA.
authors: Mark A
authors: MA
authors: Dickson
authors: Department of Medicine, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, NY 10065, USA.
authors: Sandra P
authors: SP
authors: D'Angelo
authors: Department of Pediatrics, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, NY 10065, USA.
authors: Neerav
authors: N
authors: Shukla
authors: Department of Medicine, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, NY 10065, USA.
authors: Craig
authors: C
authors: Moskowitz
authors: Department of Medicine, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, NY 10065, USA.
authors: Ariela
authors: A
authors: Noy
authors: Icahn School of Medicine at Mount Sinai, 1428 Madison Avenue, New York, NY, 10029, USA.
authors: Robert G
authors: RG
authors: Maki
authors: Department of Medicine, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, NY 10065, USA.
authors: Diego Adrianzen
authors: DA
authors: Herrera
authors: Department of Medicine, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, NY 10065, USA.
authors: Armando
authors: A
authors: Sanchez
authors: Department of Medicine, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, NY 10065, USA.
authors: Anita
authors: A
authors: Krishnan
authors: Department of Medicine, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, NY 10065, USA.
authors: Andrew
authors: A
authors: Pourmoussa
authors: Department of Biostatistics, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, NY 10065, USA.
authors: Li-Xuan
authors: LX
authors: Qin
authors: Department of Medicine, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, NY 10065, USA.
authors: William D
authors: WD
authors: Tap
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D017024:Chemotherapy, Adjuvant; D054740:Dendritic Cell Sarcoma, Follicular / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D054739:Dendritic Cell Sarcoma, Interdigitating / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D018450:Disease Progression; D018572:Disease-Free Survival; D005260:Female; D054747:Histiocytic Sarcoma / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D020360:Neoadjuvant Therapy; D009362:Neoplasm Metastasis; D009519:New York City; D016016:Proportional Hazards Models; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome
keywords: Adjuvant therapy; Dendritic sarcoma; Histiocytic neoplasm