title: Neoadjuvant treatment of Dermatofibrosarcoma Protuberans of pancreas with Imatinib: case report and systematic review of literature.
abstract: Dermatofibrosarcoma Protuberans (DFSP) is a rare skin tumor, characterized by frequent local recurrence but is seldom metastatic. It is histologically characterized by storiform arrangement of spindle cells. Cytogenetically, most tumors are characterized by translocation 17:22 leading to overexpression of tyrosine kinase PDGFB which can be targeted with tyrosine kinase inhibitor, Imatinib. We describe the first case of unresectable pancreatic metastases from DFSP treated with neoadjuvant Imatinib and subsequently R0 metastectomy. Additionally, a comprehensive systematic review of DFSP pancreatic metastases and the current published data on the use of Imatinib in DFSP is summarized.
authors.affiliation: Department of Surgery, Surgical Oncology, 986345 University of Nebraska Medical Center, Omaha, NE 68198-6345, USA.
authors.forename: Mashaal
authors.initials: M
authors.lastname: Dhir
authors.affiliation: Department of Hematology and Oncology, University of Nebraska Medical Center, Omaha, NE, USA.
authors.forename: David G
authors.initials: DG
authors.lastname: Crockett
authors.affiliation: Department of Pathology, University of Missouri, Kansas City, MO, USA.
authors.forename: Todd M
authors.initials: TM
authors.lastname: Stevens
authors.affiliation: Department of Hematology and Oncology, Creighton University Medical Center, Omaha, NE, USA.
authors.forename: Peter T
authors.initials: PT
authors.lastname: Silberstein
authors.affiliation: Department of Anatomic Pathology, Creighton University Medical Center, Omaha, NE, USA.
authors.forename: William J
authors.initials: WJ
authors.lastname: Hunter
authors.affiliation: Department of Surgery, Surgical Oncology, 986345 University of Nebraska Medical Center, Omaha, NE 68198-6345, USA.
authors.forename: Jason M
authors.initials: JM
authors.lastname: Foster
keywords: DFSP; Imatinib; Pancreatic metastases; Systematic review