Neoadjuvant treatment of Dermatofibrosarcoma Protuberans of pancreas with Imatinib: case report and systematic review of literature

Dhir, Mashaal; Crockett, David G.; Stevens, Todd M.; Silberstein, Peter T.; Hunter, William J.; Foster, Jason M.

doi:10.1186/2045-3329-4-8

Cited by 10 publications

(10 citation statements)

References 58 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Consistent with findings observed in 1 DFSP case with an unresectable pancreatic metastasis being treated in a neoadjuvant fashion with imatinib and an R0 resection was successfully performed. 20 Our study documents that the utility of imatinib neoadjuvant therapy has the potential to transform patients with surgically challenging or recurrent tumors into better operative candidates, and the response observed in histologic findings following neoadjuvant imatinib may be useful in determining the best postoperative adjuvant therapy. In our study, all the patients, who received complete excision after imatinib neoadjuvant therapy always maintained adjuvant therapy for 1 year and until the recent follow-up, live with no signs of recurrent or distant metastases after stopping imatinib treatment.…”

Section: Discussionmentioning

confidence: 56%

Target Therapy of Unresectable or Metastatic Dermatofibrosarcoma Protuberans With Imatinib Mesylate

et al. 2015

View full text Add to dashboard Cite

Dermatofibrosarcoma protuberans (DFSP) is a rare, plaque-like tumor of the cutaneous tissue occurring more on the trunk than the extremities and neck. More than 95% of DFSP present anomalies on the 17q22 and 22q13 chromosomal regions leading to the fusion of COL1A1 and PDGFB genes. Surgery is the optimal treatment for DFSP, but less effective in locally advanced or metastatic patients, as is the case with chemotherapy and radiotherapy. The aim of this study was to assess retrospectively the therapeutic activity and safety of imatinib on 22 Chinese patients with locally inoperative or metastatic DFSP at a single institution.In the collected data of 367 Chinese patients with DFSP, we analyzed retrospectively 22 patients with locally advanced or metastatic DFSP, all of whom received imatinib therapy at 1 center from January 2009 to October 2014. Patients were administered with imatinib at an initial dose of 400 mg and escalated to 800 mg daily after they developed imatinib resistance. The median follow-up time was 36 months, and the median treatment time was 15 months.The results showed that 10 locally advanced DFSP patients and 12 metastatic DFSP patients received imatinib therapy. Apart from 1 patient who developed primary imatinib resistance, 15 patients achieved partial remission (PR), and 6 patients achieved stable disease (SD). Both fibrosarcomatous DFSP and classic DFSP patients demonstrated similar response to imatinib. Median PFS was estimated to be 19 months. Median overall survival (OS) has not been reached, and estimated 1- and 3-year OS rates were 95.5% (21/22) and 77.3% (17/22), respectively. Four out of 10 patients with primarily unresectable DFSP received complete surgical resection after neoadjuvant treatment of imatinib.Imatinib therapy is well tolerated with a safety profile and is the therapy of choice in locally inoperative or metastatic DFSP. Neoadjuvant treatment of locally advanced or metastatic DFSP with imatinib improves surgical outcomes and may facilitate resection of difficult tumors.

show abstract

Section: Discussionmentioning

confidence: 56%

Target Therapy of Unresectable or Metastatic Dermatofibrosarcoma Protuberans With Imatinib Mesylate

et al. 2015

View full text Add to dashboard Cite

show abstract

“…Under these conditions, the canine COL3A1/PDGFB should produce a chimeric precursor protein, transformed into a mature growth factor after proteolysis and forming an autocrine loop, as for the human COL1A1/PDGFB fusion (11). As the chimeric protein COL1A1/PDGFB is targeted by imatinib for human DFSP (7,10), regarding the genetic and biological similarities between human DFSP and canine DFSP-like, we predict similarities in treatment responses. Finally, we propose the COL3A1 gene as a new candidate partner gene for human DFSP, like the recently discovered COL1A2 (12).…”

Section: -A Col3a1-pdgfb Fusion In a Canine Dfsp-likementioning

confidence: 99%

“…In human DFSP, the translocation t(17;22) leading to the fusion of the collagen type 1 alpha 1 (COL1A1) and plateletderived growth factor B chain (PDGFB) genes is considered as pathognomonic of DFSP (10). Thus, we analyzed a canine DFSPlike case ( Supplementary Fig.…”

Section: -A Col3a1-pdgfb Fusion In a Canine Dfsp-likementioning

confidence: 99%

Discovery of Human-Similar Gene Fusions in Canine Cancers

et al. 2017

View full text Add to dashboard Cite

Canine cancers represent a tremendous natural resource due to their incidence and striking similarities to human cancers, sharing similar clinical and pathologic features as well as oncogenic events, including identical somatic mutations. Considering the importance of gene fusions as driver alterations, we explored their relevance in canine cancers. We focused on three distinct human-comparable canine cancers representing different tissues and embryonic origins. Through RNA-Seq, we discovered similar gene fusions as those found in their human counterparts: - in B-cell lymphoma, - in glioma, and - in dermatofibrosarcoma protuberans-like. We showed not only similar partner genes but also identical breakpoints leading to oncogene overexpression. This study demonstrates similar gene fusion partners and mechanisms in human-dog corresponding tumors and allows for selection of targeted therapies in preclinical and clinical trials with pet dogs prior to human trials, within the framework of personalized medicine. .

show abstract

“…Lesions appear as smooth, flesh coloured nodules in or immediately beneath the skin and generally manifest in patients between 20 and 50 years of age 1. Cytogenetically the majority of DFSP displays the t(17:22)(q22:q13) translocation, which fuses the COL1A1 and platelet-derived growth factor B (PDGFB) genes and accounts for its PDGFB over expression 2. Translocation of t(17:22) has been found in 90% of DFSP.…”

Section: Introductionmentioning

confidence: 99%

“…Adjuvant radiation therapy has been used effectively after resection for recurrences. Because of the PDGFB upregulation, patients with unresectable disease may be treated with neoadjuvant imatinib 2…”

Section: Introductionmentioning

confidence: 99%

Pancreatic metastasis of dermatofibrosarcoma protuberans: a rare case

et al. 2020

View full text Add to dashboard Cite

Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue malignancy which is locally aggressive, slow growing. It has a very low metastatic potential and has high risk of local recurrence. We report a 65-year-old man with recurrent DFSP of thigh with pancreatic metastasis. Apart from our patient, only four other cases of pancreatic metastasis of DFSP have been reported. Our patient had a solitary metastasis to pancreas and was treated with distal pancreaticosplenectomy. Outcome of the patient was good. We present this case report to emphasise that resection may be considered for solitary metastasis of DFSP and can be managed successfully.

show abstract

Neoadjuvant treatment of Dermatofibrosarcoma Protuberans of pancreas with Imatinib: case report and systematic review of literature

Cited by 10 publications

References 58 publications

Target Therapy of Unresectable or Metastatic Dermatofibrosarcoma Protuberans With Imatinib Mesylate

Target Therapy of Unresectable or Metastatic Dermatofibrosarcoma Protuberans With Imatinib Mesylate

Discovery of Human-Similar Gene Fusions in Canine Cancers

Pancreatic metastasis of dermatofibrosarcoma protuberans: a rare case

Contact Info

Product

Resources

About