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Dermatofibrosarcoma protuberans (DFSP): Predictors of Recurrence and the Use of Systemic Therapy

  • Bone and Soft Tissue Sarcomas
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript

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.

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Acknowledgment

Supported by Grants from the National Cancer Institute CA47179 (LXQ, RGM, SS, MFB), CA148260 (RGM), the National Cancer Institute-American Society of Clinical Oncology Cancer Foundation Clinical Investigator Team Leadership Supplemental Award (RGM), the Cycle for Survival (RGM), and the Shuman Fund for GIST Research (RGM).

Disclosure

RGM serves on an advisory board for Novartis.

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Authors and Affiliations

  1. Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY, USA

    Ryan C. Fields MD, Nicole Moraco MA, Samuel Singer MD & Murray F. Brennan MD

  2. Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY, USA

    Meera Hameed MD

  3. Department of Epidemiology and Biostatistics, Memorial Sloan-Kettering Cancer Center, New York, NY, USA

    Li-Xuan Qin PhD & Xiaoyu Jia MS

  4. Department of Medical Oncology, Memorial Sloan-Kettering Cancer Center, New York, NY, USA

    Robert G. Maki MD, PhD

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  1. Ryan C. Fields MD
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  2. Meera Hameed MD
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  4. Nicole Moraco MA
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Correspondence to Ryan C. Fields MD.

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Fields, R.C., Hameed, M., Qin, LX. et al. Dermatofibrosarcoma protuberans (DFSP): Predictors of Recurrence and the Use of Systemic Therapy. Ann Surg Oncol 18, 328–336 (2011). https://doi.org/10.1245/s10434-010-1316-5

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  • DOI: https://doi.org/10.1245/s10434-010-1316-5

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