New Study Evaluates Practice Patterns and Management of Patients With Dermatofibrosarcoma Protuberans

An international team of researchers led by Fox Chase Cancer Center published a study evaluating practice patterns and management of patients with dermatofibrosarcoma protuberans (DFSP), a rare type of skin cancer.

DFSP is a soft-tissue cancer with an incidence rate of only 4.1 cases per million. This makes it challenging to study, which results in varied care due to a paucity of research-backed guidance.

As a step toward standardizing care, Leah Winer, MD, the study’s lead author, decided to explore the issue. Winer worked on the study during a recently completed Complex General Surgical Oncology Fellowship at Fox Chase. She was joined by two Fox Chase colleagues, Jeffrey Farma, MD, FACS, Chair of Surgery, and Anthony Villano, MD, an Assistant Professor in the Department of Surgical Oncology.

They analyzed records for 347 patients across 10 institutions in eight different countries who underwent surgery for DFSP between 2003 and 2021. The researchers’ goals were to determine how DFSP patients are being managed and to advance knowledge of best practices around surgical margins — how much tissue to remove around DFSP tumors.

The research indicated that 1- to 2-centimeter surgical margins are safe and yield favorable results for patients. These margins are narrower than the 2- to 5-centimeter margins historically used by surgeons and require fewer, less intensive follow-on reconstructive surgeries.

“This is confirming a paradigm shift that has been happening over the last few decades that says yes, using narrower margins is safe,” said Winer.

The reason the surgical margins are under debate is because DFSP tumors can extend, almost like tentacles, through the fat underneath the skin. Because the reach of these “tentacles” is difficult to detect, surgeons have historically taken a wide margin, removing up to two inches of additional tissue around the tumor to minimize the chance of leaving behind any disease.

However, when DFSP tumors occur on the head, neck, and extremities, there is greater risk to important structures like blood vessels, nerves, and facial features. This forces healthcare providers to weigh the risks and benefits of leaving the cancer behind versus taking so much unnecessary tissue that the patient will incur greater risk and require major reconstructive surgery.

In their analysis, Winer and her colleagues found that most healthcare providers operating on DFSP patients were using less than 3-centimeter circumferential margins.

“We found that the rate at which you can clear all the disease in one surgery does not increase much above 2-centimeter margins. However, at margins greater than 2 centimeters, you have higher complication rates and lower rates of being able to close the incision just by sewing it shut. So, based on the outcomes of this study, we concluded that a 1- to 2-centimeter margin is an appropriate recommendation,” said Winer.

She credits advances in pathology with these shifts in care. “Sarcoma care has improved as pathological assessment has gotten better and more precise. This has informed the way the surgeon can do their work and results in better outcomes for patients,” she said.

The paper, “Current Patterns of Care and Outcomes for Dermatofibrosarcoma Protuberans: An International Multi-Institutional Collaborative,” was published in Cancer, a publication of the American Cancer Society.