Targeting Oncogenic Pathways in Dermatofibrosarcoma Protuberans and Inflammatory Myofibroblastic Tumor

B. P. Ferreira*, A. Gullet, S. M. Pollack, R. L. Jones

*Corresponding author for this work

Research output: Chapter in Book/Report/Conference proceedingChapter


Dermatofibrosarcoma protuberans (DFSP) and inflammatory myofibroblastic tumor (IMT) are rare maLignancies with the capacity to be locally invasive but with low metastatic potential. DFSP is characterized by the translocation t(17;22)(q22;q13), resulting in the COl1A1-PDGFβ fusion protein. Imatinib mesylate has shown activity in the treatment of unresectable or metastatic DFSP, achieving an RECIST response rate close to 50% in phase II trials. These results have estabLished imatinib as standard systemic therapy for patients with unresectable or metastatic DFSP.IMT is also a rare mesenchymal neoplasm that tends to occur in children and young adults, typically affecting the lungs, retroperitoneum, abdomen, and pelvis. Rearrangements in the anaplastic lymphoma kinase (. ALK) locus on chromosome 2p23 are present in almost half of IMTs. ALK inhibition is a promising therapeutic approach in patients with inoperable IMT. For both DFSP and IMT, surgery remains the mainstay therapy for locaLized disease. However, advances in the understanding of the underlying biology of these diseases have led to the appLication of effective and well-tolerated systemic therapy.

Original languageEnglish (US)
Title of host publicationPathobiology of Human Disease
Subtitle of host publicationA Dynamic Encyclopedia of Disease Mechanisms
PublisherElsevier Inc
Number of pages8
ISBN (Electronic)9780123864567
ISBN (Print)9780123864574
StatePublished - Jan 1 2014
Externally publishedYes


  • ALK mutation
  • COl1A1-PDGFβ
  • Crizotinib
  • Dermatofibrosarcoma protuberans
  • Imatinib
  • Inflammatory myofibroblastic tumor
  • T(17;22)(q22;q13)
  • Tyrosine kinase inhibitors

ASJC Scopus subject areas

  • Medicine(all)

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