Long-term results of treatment of advanced dermatofibrosarcoma protuberans (DFSP) with imatinib mesylate – The impact of fibrosarcomatous transformation

Rutkowski, Piotr; Klimczak, Anna; Ługowska, Iwona; Jagielska, Beata; Wągrodzki, Michał; Dębiec‐Rychter, Maria; Pieńkowska-Grela, Barbara; Świtaj, Tomasz

doi:10.1016/j.ejso.2017.03.011

Cited by 61 publications

(50 citation statements)

References 41 publications

(19 reference statements)

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“…To date, there has been no clinical study directly comparing various imatinib doses in the treatment of DFSP. A real‐world study of 31 patients treated with imatinib for advanced DFSP showed a progression‐free 5‐year survival rate of 58 % and an overall 5‐year survival rate of 64 % (median follow‐up of 5.3 years) . Shorter survival times on imatinib were associated with the histological subtype of fibrosarcomatous DFSP (DFSP‐FS) as well as with the presence of metastases .…”

Section: Treatmentmentioning

confidence: 99%

S1 guidelines for dermatofibrosarcoma protuberans (DFSP) – update 2018

Ugurel

Kortmann

Mohr

et al. 2019

J Deutsche Derma Gesell

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Summary While dermatofibrosarcoma protuberans (DFSP) is a rare cancer entity overall, it is nevertheless the most common type of cutaneous sarcoma. The tumor is of fibroblastic origin and characterized by slow, undermining and locally destructive growth. Metastatic spread is very rare. Given its nonspecific clinical appearance, diagnosis is frequently delayed. Biopsy and subsequent histopathology are key diagnostic tools. Standard treatment for primary tumors consists of complete excision with surgical margins of 1 to 2 cm. Smaller margins are associated with high local recurrence rates. Inoperable and metastatic DFSP may be treated with radiation therapy. Approximately 80–90 % of DFSP lesions harbor a fusion gene that results in continuous activation of the PDGF‐β signaling pathway. Consequently, molecular targeted therapy inhibiting PDGF‐β is an effective option for advanced (inoperable) and metastatic DFSP. The first agent to be approved for systemic treatment of DFSP is the multikinase inhibitor imatinib, showing objective response rates of about 50 % in clinical trials.

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Section: Treatmentmentioning

confidence: 99%

S1 guidelines for dermatofibrosarcoma protuberans (DFSP) – update 2018

Ugurel

Kortmann

Mohr

et al. 2019

J Deutsche Derma Gesell

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“…In addition, radiotherapy may be useful in patients with positive surgical margins, to reduce the risk of local recurrence, and in cases where surgery would result in major cosmetic defects. Several published reports have indicated that imatinib mesylate can be considered as an optimal therapeutic choice in patients with metastatic or locally advanced DFSP . Tsai et al reported 13 pediatric DFSP patients who received a wide local excision (25 mm‐30 mm), and neither recurrence nor metastasis was observed from 20 months to 19 years after the surgery.…”

Section: Discussionmentioning

confidence: 99%

Pediatric atrophic dermatofibrosarcoma protuberans

Liu

Zhang

Han

et al. 2017

Pediatric Investigation

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“…The dose of imatinib (400 vs 800 mg) did not have a significant differential treatment effect 67. Furthermore, the presence of a fibrosarcomatous component in DFSP was correlated with worse imatinib activity in terms of PFS and OS 68…”

Section: Part 1 Developed Genomic-guided Precision Therapy For Stsmentioning

confidence: 96%

Genomic-guided precision therapy for soft tissue sarcoma

Chen

2020

ESMO Open

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Soft tissue sarcoma (STS), although heterogeneous in histopathology presentation, has mostly been treated with chemotherapy agents as one entity. Our understanding of crucial genomic alterations in different STS histologies and the advent of molecular-targeted agents have reshaped the treatment paradigm for advanced STS. Small-molecule inhibitors of c-KIT, plate-derived growth factor receptor alpha, c-MET, BRAF, anaplastic lymphoma kinase, ROS1 and colony-stimulating factor-1 receptor have been successfully validated in clinical studies to yield practice-changing results. Inhibitors of other novel genomic targets including mouse double minute 2 homolog, cyclin-dependent kinase 4/6, mitogen-activated protein kinase and epigenetic regulators are expected to be developed in the near future. Furthermore, with the advancement and accessibility of molecular diagnosis and next-generation sequencing, a genomic-based therapeutic approach should be widely applicable to advanced STS patients. This review will focus on the progress of genomic-guided therapy tailored to each molecular alteration of different STS histologies.

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Long-term results of treatment of advanced dermatofibrosarcoma protuberans (DFSP) with imatinib mesylate – The impact of fibrosarcomatous transformation

Cited by 61 publications

References 41 publications

S1 guidelines for dermatofibrosarcoma protuberans (DFSP) – update 2018

S1 guidelines for dermatofibrosarcoma protuberans (DFSP) – update 2018

Pediatric atrophic dermatofibrosarcoma protuberans

Genomic-guided precision therapy for soft tissue sarcoma

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