Dermatofibrosarcoma protuberans
Note: these are regimens tested in subtype-specific populations, please see the main soft tissue sarcoma page for other regimens.
Section editor | |
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Elizabeth J. Davis, MD Vanderbilt University Nashville, TN, USA |
Last updated on 2024-07-23: 1 regimens on this page
2 variants on this page
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Guidelines
Given the rapid change in evidence in many areas of hematology/oncology, readers are encouraged to consider any guideline published 5+ years ago to be for historical purposes, only.
NCCN
- NCCN Guidelines - Dermatofibrosarcoma Protuberans (DFSP)
- 2014: Bichakjian et al. Dermatofibrosarcoma protuberans, version 1.2014 PubMed
- 2012: Miller et al. Dermatofibrosarcoma protuberans. PubMed
- 2007: Miller et al. Dermatofibrosarcoma protuberans. PubMed
- 2004: Authors not listed. Dermatofibrosarcoma protuberans. Clinical practice guidelines in oncology. PubMed
All lines of therapy
Imatinib monotherapy
Regimen variant #1, 400 mg/day
Study | Dates of enrollment | Evidence |
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Rutkowski et al. 2010 (SWOG S0345) | 2005-2006 | Phase 2, fewer than 20 pts |
Regimen variant #2, 800 mg/day
Study | Dates of enrollment | Evidence |
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Rutkowski et al. 2010 (EORTC 62027) | 2004-2007 | Phase 2, fewer than 20 pts (RT) |
Targeted therapy
- Imatinib (Gleevec) 400 mg PO twice per day
Continued indefinitely, unless complete (R0) surgical resection became possible
References
- EORTC 62027: Rutkowski P, Van Glabbeke M, Rankin CJ, Ruka W, Rubin BP, Debiec-Rychter M, Lazar A, Gelderblom H, Sciot R, Lopez-Terrada D, Hohenberger P, van Oosterom AT, Schuetze SM; EORTC Soft Tissue and Bone Sarcoma Group; SWOG. Imatinib mesylate in advanced dermatofibrosarcoma protuberans: pooled analysis of two phase II clinical trials. J Clin Oncol. 2010 Apr 1;28(10):1772-9. Epub 2010 Mar 1. link to original article dosing details in manuscript have been reviewed by our editors link to PMC article PubMed NCT00085475
- SWOG S0345: Rutkowski P, Van Glabbeke M, Rankin CJ, Ruka W, Rubin BP, Debiec-Rychter M, Lazar A, Gelderblom H, Sciot R, Lopez-Terrada D, Hohenberger P, van Oosterom AT, Schuetze SM; EORTC Soft Tissue and Bone Sarcoma Group; SWOG. Imatinib mesylate in advanced dermatofibrosarcoma protuberans: pooled analysis of two phase II clinical trials. J Clin Oncol. 2010 Apr 1;28(10):1772-9. Epub 2010 Mar 1. link to original article dosing details in manuscript have been reviewed by our editors link to PMC article PubMed NCT00084630