Dermatofibrosarcoma protuberans (DFSP): Predictors of recurrence and the use of systemic therapy Journal Article


Authors: Fields, R. C.; Hameed, M.; Qin, L. X.; Moraco, N.; Jia, X.; Maki, R. G.; Singer, S.; Brennan, M. F.
Article Title: Dermatofibrosarcoma protuberans (DFSP): Predictors of recurrence and the use of systemic therapy
Abstract: Background: Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue malignancy that typically presents with local invasion but rarely metastasizes. We examine clinicopathologic factors associated with disease-free survival (DFS) in patients with primary and recurrent DFSP and evaluate responses to multimodality therapy. Materials and Methods: Patients treated for DFSP were identified in a prospectively maintained database. Clinicopathologic factors associated with DFS were analyzed using univariate and multivariate analysis. Results: A total of 244 patients with DFSP were identified. Median follow-up was 50 months. A total of 14 patients had local recurrence (LR), and 2 patients had distant recurrence (DR), with a median time to recurrence of 35 months. At time of last follow-up, 70% and 47% of patients showed no evidence of disease (NED) in the primary (n = 197) and recurrent groups (n = 47), respectively. On univariate analysis, tumor location and depth were associated with DFS in the primary group, while margin status (R1 vs. R0) was associated with DFS in the LR group. On multivariate analysis, only depth (primary group) and margin status (LR group), remained significant. Also, 22 patients had therapy other than surgical resection: 14 radiotherapy, 4 tyrosine kinase inhibitor (TKI) only, 2 conventional chemotherapy only, and 2 chemotherapy plus TKI. Responses to other therapies were variable. Conclusions: DFS after treatment for DFSP is strongly predicted by tumor depth in the primary setting and margin status in recurrent tumors. The treatment for DFSP in the primary or recurrent setting is excision with negative margins, resulting in low recurrence rates and infrequent metastatic spread. Multimodality treatment, especially TKI use, can be effective, but is not curative. © 2010 Society of Surgical Oncology.
Journal Title: Annals of Surgical Oncology
Volume: 18
Issue: 2
ISSN: 1068-9265
Publisher: Springer  
Date Published: 2011-02-01
Start Page: 328
End Page: 336
Language: English
DOI: 10.1245/s10434-010-1316-5
PROVIDER: scopus
PUBMED: 20844969
PMCID: PMC4310211
DOI/URL:
Notes: --- - "Export Date: 4 March 2011" - "CODEN: ASONF" - "Source: Scopus"
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MSK Authors
  1. Meera Hameed
    284 Hameed
  2. Murray F Brennan
    1059 Brennan
  3. Xiaoyu Jia
    46 Jia
  4. Robert Maki
    241 Maki
  5. Li-Xuan Qin
    193 Qin
  6. Ryan Courtney Fields
    19 Fields
  7. Samuel Singer
    337 Singer
  8. Nicole Heather Moraco
    16 Moraco