Survival in patients with primary dermatofibrosarcoma protuberans: National Cancer Database analysis

Trofymenko, Oleksandr; Bordeaux, Jeremy S.; Zeitouni, Nathalie C.

doi:10.1016/j.jaad.2017.11.030

Cited by 31 publications

(36 citation statements)

References 26 publications

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“…Retrospective analysis of the US National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) database, which included 3,686 DFSP patients, showed age, male gender and tumor size to be significantly associated with an unfavorable prognosis . Another retrospective analysis of the US National Cancer Database (5,249 DFSP cases) revealed the following factors to be relevant in terms of prognosis: insurance status, anaplastic histology and positive postoperative margins .…”

Section: Prognosis and Stagingmentioning

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: Prognosis and Stagingmentioning

confidence: 99%

S1 guidelines for dermatofibrosarcoma protuberans (DFSP) – update 2018

Ugurel

Kortmann

Mohr

et al. 2019

J Deutsche Derma Gesell

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“…Nevertheless, mortality rates compared to other high‐grade sarcomas are lower, and patients have a higher chance of prolonged survival. A recent large study addressed survival in patients with dermatofibrosarcoma protuberans using the national cancer database . In this study, positive margins and “poorly differentiated” or “anaplastic” histology had an increased risk of mortality, whereas tumor size did not impact survival .…”

Section: Discussionmentioning

confidence: 94%

Dermatofibrosarcoma protuberans with fibrosarcomatous transformation: our experience, molecular evaluation of selected cases, and short literature review

et al. 2019

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Dermatofibrosarcoma protuberans with fibrosarcomatous transformation (DFSP-FS) is a higher grade tumor arising from dermatofibrosarcoma protuberans (DFSP). Recent literature highlights its impact on recurrence rates, metastatic rates, and survival. In this article, we aim to describe our experience with 13 cases of DFSP-FS in terms of pathologic findings, molecular alterations, clinical outcomes, management, and also perform a short recent literature review.

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“…Our patient's lesion was widely excised with 5 cm margins and required skin grafting for closure. While wide local excision with 3–5 cm margins has historically been the gold standard for DFSP treatment, new reports support Mohs surgery as the treatment of choice . Mohs surgery offers improved tissue preservation with more narrow margins of 1–2 cm, lower recurrence rates, and decreased usage of postsurgical radiation therapy .…”

Section: Discussionmentioning

confidence: 99%

“…While wide local excision with 3–5 cm margins has historically been the gold standard for DFSP treatment, new reports support Mohs surgery as the treatment of choice . Mohs surgery offers improved tissue preservation with more narrow margins of 1–2 cm, lower recurrence rates, and decreased usage of postsurgical radiation therapy . Technical limitations of processing large tissue blocks and patient‐specific factors may prohibit the use of Mohs surgery in individual cases.…”

Section: Discussionmentioning

confidence: 99%