Advanced Dermatofibrosarcoma Protuberans Treatment With Imatinib: Experience From a Dedicated Sarcoma Medical Oncology Clinic in India

Abstract

PurposeAdvanced dermatofibrosarcoma protuberans (DFSP) is an exceptionally uncommon disease with scarce literature, especially from developing countries. Molecular testing is unfortunately not available in India, and expert diagnosis by a sarcoma pathologist is available only in tertiary care centers.Materials and MethodsWe retrospectively analyzed consecutive patients with inoperable DFSP (on the basis of expert histopathology only) who presented to our sarcoma medical oncology clinic from January 2016 to July 2017.ResultsThere were a total of seven patients, with median age of 35 years, predominantly males (85.7%). Fibrosarcomatous variant and metastatic disease were present in six (85.7%) patients. Partial response rates were 71.4%, and overall disease control was 85.7%. Median progression-free survival was 14 months.ConclusionDFSP diagnosis on the basis of expert histopathology in the absence of translocation can help out in targeted therapy–based treatment until translocation testing becomes available. The fibrosarcomatous variant has poor outcome, and further research is needed to help this group of patients.

Dermatofibrosarcoma Protuberans Re-excision and Recurrence Rates in the Netherlands Between 1989 and 2016

“…Only a few cases were treated with postoperative radiotherapy in our study, probably because it is still unclear whether radiotherapy is effective in slowly growing tumours, such as DFSP. Also, only a few cases were treated with tyrosine kinase inhibitors (imatinib), probably because systemic treatment for DFSP is indicated only for metastasized tumours or for tumours that could not be treated surgically, which is rarely the case for DFSPs (18,19). We observed that, in our large population-based sample, 51% of DFSPs were re-excised and 10% recurred.…”

Recurrent Dermatofibrosarcoma Protuberant of the Hand: A Case Report

“…Radiation therapy can be used as adjuvant therapy in cases with positive margins or with recurrent disease, and as palliative radiation therapy for incurable disease [12]. The targeted drug imatinib can be used in patients with inoperable primary tumors, locally recurrent and metastatic DFSP patients, and as a new neoadjuvant drug for preoperative reduction of tumor volume [13]. In addition, studies have shown that PD-1 monoclonal antibodies and CDK4/6 inhibitors may be used as new targeted drugs [14].…”

The evolving role of precision medicine in the management of advanced sarcomas – A mini review

“…Fibrosarcomatous areas occasionally occur in DFSP, which are associated with increased chance of metastasis and P53 mutations. [28] It is characterized by a t(17;22) translocation involving COL1A1 and PDGFB genes. The consequent hyperactivation of PDGFRB rendering them sensitive to targeted therapy with the tyrosine kinase inhibitors (TKI) imatinib.…”