Treatment of advanced dermatofibrosarcoma protuberans with imatinib mesylate with or without surgical resection

J Eur Acad Dermatol Venereol. 2011 Mar;25(3):264-70. doi: 10.1111/j.1468-3083.2010.03774.x.

Abstract

Background: Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma of the skin characterized by the presence of specific COL1A1-PDGFB fusion protein, which appears as a consequence of the t(17;22) (q22;q13) translocation.

Objective: The aim of the study was to perform an analysis of patients with advanced DFSP treated with imatinib, with or without surgery, in clinical practice outside trials.

Patients and methods: We analysed the data of 15 patients (6 male, 9 female; median age 56 years) with locally advanced/initially inoperable and/or metastatic DFSP treated with imatinib 400-800 mg daily between 12/2004 and 06/2009. All diagnoses were ascertained cytogenetically (fluorescent in situ hybridization). Median follow-up time was 16 months (range: 4-81).

Results: Metastases were present in six cases (two lungs, two soft tissue, two lymph nodes). Fibrosarcomatous transformation (FS-DFSP) was confirmed in seven patients (47%). A 2-year progression-free survival (PFS) rate was 60%, and a 2-year overall survival (OS) rate was 78% (median time for PFS/OS was not reached). The best overall responses were: 10 partial responses (67%, including 5 FS-DFSP-1 progressed during the follow-up), 2 stable diseases (13%) and 3 progressive diseases (20%). Seven patients (47%) underwent resection of residual disease and remained free of disease.

Conclusions: We have confirmed the profound anti-tumour effect of imatinib in DFSP harbouring t(17;22) with long-term responses. Imatinib therapy may in some cases lead to tumour resectability of lesser disfiguration.

Publication types

  • Comparative Study

MeSH terms

  • Antineoplastic Agents / therapeutic use
  • Benzamides
  • Chromosomes, Human, Pair 17
  • Chromosomes, Human, Pair 22
  • Collagen Type I / metabolism
  • Combined Modality Therapy
  • Dermatofibrosarcoma / drug therapy
  • Dermatofibrosarcoma / metabolism
  • Dermatofibrosarcoma / surgery
  • Disease-Free Survival
  • Female
  • Follow-Up Studies
  • Humans
  • Imatinib Mesylate
  • Male
  • Middle Aged
  • Oncogene Proteins, Fusion / metabolism
  • Piperazines / therapeutic use*
  • Proto-Oncogene Proteins c-sis / metabolism
  • Pyrimidines / therapeutic use*
  • Retrospective Studies
  • Sarcoma / drug therapy*
  • Sarcoma / metabolism
  • Sarcoma / surgery*
  • Skin Neoplasms / drug therapy*
  • Skin Neoplasms / metabolism
  • Skin Neoplasms / surgery*
  • Treatment Outcome

Substances

  • Antineoplastic Agents
  • Benzamides
  • Collagen Type I
  • Oncogene Proteins, Fusion
  • Piperazines
  • Proto-Oncogene Proteins c-sis
  • Pyrimidines
  • collagen type I, alpha 1 chain
  • Imatinib Mesylate