Impact of surgery, radiation and systemic therapy on the outcomes of patients with dendritic cell and histiocytic sarcomas Journal Article


Authors: Gounder, M.; Desai, V.; Kuk, D.; Agaram, N.; Arcila, M.; Durham, B.; Keohan, M. L.; Dickson, M. A.; D'Angelo, S. P.; Shukla, N.; Moskowitz, C.; Noy, A.; Maki, R. G.; Adrianzen Herrera, D.; Sanchez, A.; Krishnan, A.; Pourmoussa, A.; Qin, L. X.; Tap, W. D.
Article 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 58.8 years 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. © 2015 Elsevier Ltd. All rights reserved.
Keywords: adult; aged; major clinical study; overall survival; prednisone; sorafenib; doxorubicin; sunitinib; systemic therapy; alpha interferon; gemcitabine; adjuvant therapy; cancer adjuvant therapy; cancer radiotherapy; pelvis; carboplatin; metastasis; dendritic cell; etoposide; cyclophosphamide; vincristine; medical record review; retrospective study; ifosfamide; docetaxel; irinotecan; abdomen; follicular dendritic cell sarcoma; pazopanib; rapamycin; alemtuzumab; histiocytic sarcoma; brivanib; human; male; female; priority journal; article; dendritic sarcoma; histiocytic neoplasm; interdigitating dendritic cell sarcoma
Journal Title: European Journal of Cancer
Volume: 51
Issue: 16
ISSN: 0959-8049
Publisher: Elsevier Inc.  
Date Published: 2015-11-01
Start Page: 2413
End Page: 2422
Language: English
DOI: 10.1016/j.ejca.2015.06.109
PROVIDER: scopus
PUBMED: 26298731
PMCID: PMC5087129
DOI/URL:
Notes: Export Date: 2 November 2015 -- Source: Scopus
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MSK Authors
  1. Craig Moskowitz
    371 Moskowitz
  2. Ariela Noy
    204 Noy
  3. Narasimhan P Agaram
    117 Agaram
  4. Li-Xuan Qin
    114 Qin
  5. Mary Louise Keohan
    64 Keohan
  6. Mrinal M Gounder
    68 Gounder
  7. Mark Andrew Dickson
    77 Dickson
  8. Maria Eugenia Arcila
    336 Arcila
  9. Deborah Kuk
    81 Kuk
  10. Neerav Shukla
    69 Shukla
  11. William Douglas Tap
    122 Tap
  12. Benjamin Heath Durham
    60 Durham
  13. Ved Dinkar Desai
    2 Desai