From the Guidelines
Dermatofibrosarcoma protuberans (DFSP) should be treated with wide local excision with 2-4 cm margins or Mohs micrographic surgery to ensure complete removal of the tumor, as it rarely metastasizes but is locally aggressive and has high recurrence rates if not completely removed 1. The standard treatment for DFSP is surgical excision, with the goal of achieving negative margins to minimize recurrence.
- Wide local excision with 2-4 cm margins is the preferred treatment, as it allows for complete removal of the tumor and reduces the risk of recurrence.
- Mohs micrographic surgery is also an option, particularly for tumors in critical anatomical sites, as it can help preserve tissue and reduce functional loss.
- For larger or recurrent tumors, neoadjuvant therapy with imatinib (Gleevec), a tyrosine kinase inhibitor, may be used at doses of 400-800 mg daily for several months before surgery to shrink the tumor 1.
- Imatinib targets the COL1A1-PDGFB fusion gene present in most DFSP cases, making it an effective treatment option for patients with this type of tumor.
- Radiation therapy may be recommended for positive surgical margins or when complete excision isn't possible, as it can help reduce the risk of recurrence and improve outcomes.
- Regular follow-up examinations are essential, typically every 3-6 months for the first few years after treatment, then annually, as recurrences can happen years after initial treatment.
- Patients should perform regular self-examinations of the surgical site and surrounding areas to monitor for potential recurrence, and seek medical attention if they notice any changes or symptoms.
From the Research
Overview of Dermatofibrosarcoma Protuberans (DFSP)
- DFSP is a rare cutaneous tumor that is locally highly invasive and aggressive, although it rarely metastasizes 2.
- It occurs most commonly on the trunk and proximal extremities, affects all races, and often develops between the second and fifth decade of life 3.
- The tumor grows slowly, typically over years, and has several variants, including pigmented, myxoid, myoid, granular cell, sclerotic, atrophic, giant cell fibroblastoma, and DFSP with fibrosarcomatous areas 3.
Treatment Options for DFSP
- Traditional treatment has been wide and deep local surgical excision, but this has resulted in a local recurrence rate of 11% even with 3-cm-wide local excision margins 2.
- Mohs micrographic surgery has emerged as a treatment of choice for DFSP, with a recurrence rate of 2.4% 2 and 2% 4.
- Imatinib mesylate is a safe and effective treatment for DFSP, especially in cases of local advanced or metastatic disease 3, 5.
- Radiation therapy is warranted for inoperable disease or for cases with positive margins where re-excision is not possible 5.
Surgical Approaches for DFSP
- Wide local excision and Mohs micrographic surgery are the preferred approaches for localized disease 5, 6.
- The choice between wide local excision and Mohs micrographic surgery should be governed by the attainment of three goals: complete excision of the tumor with negative margins, preservation of function and cosmesis, and minimization of cost and inconvenience to the patient and the health care system 6.
- Mohs micrographic surgery allows for the detection and excision of microscopic tumor elements, resulting in a low recurrence rate 4.