Materiały źródłowe

• #1 Dermatofibrosarcoma protuberans: surgical management of a challenging mesenchymal tumor | World Journal of Surgical Oncology | Full Text

  https://wjso.biomedcentral.com/articles/10.1186/s12957-019-1627-3

  Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade malignant mesenchymal tumor of the soft tissue, characterized by slow infiltrative growth and common local recurrence, with rare distant metastases. […] The greatest clinical challenge in the management of DFSP is achieving local control. Complete excision of the tumor with surgical margin widths of at least 2 cm is recommended. […] Surgical excision remains the standard of therapy. Main surgical treatment options consist of wide excision or Mohs micrographic surgery. […] According to Guidelines by the German Cancer Society and German Dermatologic Society, excision margins usually range from 1 to 5 cm. […] The Clinical Practice Guidelines in Oncology for Dermatofibrosarcoma Protuberans published by the National Comprehensive Cancer Network recommends 2- to 4-cm margins to investing fascia if wide local excision is applied.

• #1 Dermatofibrosarcoma protuberans: Treatment – UpToDate

  https://www.uptodate.com/contents/dermatofibrosarcoma-protuberans-treatment

  Dermatofibrosarcoma protuberans: Treatment […] The preferred initial treatment for a localized dermatofibrosarcoma protuberans (DFSP) is resection with pathologically negative margins. […] The status of the surgical margins is the most important prognostic factor in patients with DFSP.

• #1 Dermatofibrosarcoma Protuberans Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/1100203-treatment

  Based on the knowledge that constitutively activated PDGFB-PDGFR-beta signaling pathway plays a central role in the proliferation of DFSP tumor cells, the development of molecularly targeted therapy holds promise as an additional treatment option. […] Surgical excision remains the mainstay of treatment for dermatofibrosarcoma protuberans (DFSP). Despite controversy, Mohs micrographic surgery has become accepted as the treatment of choice. […] Because of its infiltrating growth pattern, DFSP commonly extends far beyond the clinical margins; this accounts in part for the high recurrence rate after standard surgical excision. Hence, a wide excision of 2-3 cm or more of the margins beyond clinically identifiable tumor border, down to and including the fascia, is recommended for the treatment of DFSP.

• #1 Dermatofibrosarcoma Protuberans: Update on the Diagnosis and Treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7355835/

  Dermatofibrosarcoma protuberans (DFSP) is a slow growing, low- to intermediate-grade dermal soft-tissue tumor. The standard treatment is complete surgical excision. The surgical procedures include wide local excision (WLE) with tumor free margins, Mohs micrographic surgery (MMS) and amputation. Unresectable DFSPs are treated with radiation therapy and/or targeted therapy. […] The standard treatment of resectable DFSPs is complete surgical excision with either wide local excision with tumor free margins or Mohs micrographic surgery, or, rarely, amputation. Each procedure has advantages and drawbacks. Unresectable DFSPs are treated with radiation therapy and/or targeted therapy. […] Surgical excision is the standard treatment of DFSP including stage I and II, even III and IV whenever feasible.

• #1 Dermatofibrosarcoma protuberans – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/dermatofibrosarcoma-protuberans/diagnosis-treatment/drc-20576979

  Dermatofibrosarcoma protuberans treatment typically involves surgery to remove the cancer. Other treatments may be used to kill cancer cells that might remain after surgery. […] Treatment options may include: […] Surgery to remove the cancer. Dermatofibrosarcoma protuberans tends to grow in a shape that’s not regular. This makes the cancer hard to remove completely. Your healthcare professional may recommend a procedure to remove the cancer and some of the healthy tissue around it. This makes it more likely that all the cancer cells are removed. […] Mohs surgery is a type of surgery that involves cutting away thin layers of cancer-containing skin until only cancer-free tissue is left. After each layer of skin is removed, it’s examined for signs of cancer. The process keeps going until there are no signs of cancer. Mohs surgery may be helpful for treating larger cancers.

• #1 Dermatofibrosarcoma Protuberans: Update on the Diagnosis and Treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7355835/

  Surgical techniques include wide local excision (WLE) with tumor-free margins, Mohs micrographic surgery (MMS), partial or total amputation if the tumor is located on the upper or lower digits. […] Multiple studies have shown that MMS can significantly lower the risk of recurrence of DFSP, compared with WLE. […] The FS-DFSP variant is a much more aggressive tumor with local recurrence in more than 50% of patients and metastasis in 10% to 15% of patients. FS-DFSP treatment needs consultation with a multidisciplinary specialized soft-tissue sarcoma tumor board. Aggressive treatments for FS-DFSP include adequate WLE with clear surgical margins or MMS. Adjuvant radiotherapy and targeted therapy may be used to reduce the incidence of both local recurrence and metastasis. […] Unresectable DFSPs include advanced stage tumors, recurrent tumors without any possibility for further resection due to the size and/or location, or tumors in which further resection is likely to cause severe functional or cosmetic defects. These tumors should be treated with adjuvant radiation and/or targeted therapy in consultation with a multidisciplinary specialized soft-tissue sarcoma tumor board.

• #1 Dermatofibrosarcoma Protuberans Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/1100203-treatment

  With limited clinical data to date, a response rate of approximately 65% has been achieved among DFSP patients treated with imatinib. A small subset of DFSP patients lacking the classic t(17,22) gene aberration seems to have no response to imatinib. […] Neoadjuvant imatinib therapy for DFSP has been proposed. Using imatinib as a preoperative therapy agent in locally advanced or recurrent DFSP may decrease tumor load, promote tumor cell apoptosis, and subsequently reduce the extent of surgery. […] Radiation therapy may be recommended for patients if the margins of resection are positive or for situations in which adequate wide excision alone may result in major cosmetic or functional deficits. Postoperative adjuvant RT may reduce the risk of recurrence when clear surgical margins are not confident. The complete radiation therapy dose ranges from 50-70 Gy. Overall, the risk of severe complications from RT is low.

• #1 Dermatofibrosarcoma Protuberans – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK513305/

  Current NCCN guidelines recommend the use of adjuvant radiation in the following settings: Positive margins after surgical resection, Negative margins, with the closest margin being less than 1 cm in patients who did not undergo MMS, For recurrent or metastatic disease when surgical excision is not feasible. […] Imatinib inhibits the PDGF receptor and other receptor tyrosine kinases such as c-KIT. Imatinib has the United States Food and Drug Administration (FDA) approval for unresectable, recurrent, or metastatic DFSP. […] Patients should participate in regular self-examination, and the primary site should be examined every 6 months for 3 to 5 years. Lifelong examination annually follows.

• #1 Dermatofibrosarcoma Protuberans: Update on the Diagnosis and Treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7355835/

  Multiple studies have shown that DFSP is a radio-responsive tumor and adjuvant radiation therapy is effective to control tumor growth and reduce the incidence of postoperative recurrence. […] Imatinib mesylate (IM) is a potent and specific protein tyrosine kinase inhibitor interfering with the phosphorylation and activation of the PDGF receptor which is constitutively activated due to translocation and fusion between PDGFB and COL1A1 genes. […] The efficacy of IM was observed in localized and metastatic DFSP with t(17; 22), but not in FS-DFSP lacking t(17; 22). […] In the cases of IM resistance, other multikinase inhibitors, including sunitinib, sorafenib and pazopanib, can be considered since treatment with these inhibitors showed effectiveness in IM resistant DFSP patients.

• #1 Skin cancer types: Dermatofibrosarcoma protuberans diagnosis & treatment

  https://www.aad.org/public/diseases/skin-cancer/dermatofibrosarcoma-protuberans

  The results from a small study show that radiation may reduce the risk of DFSP from returning. Researchers followed 14 patients who received radiation treatments, mostly after surgery. Most patients, 86%, remained cancer-free. The average follow-up period exceeded 10 years. […] Some patients cannot undergo surgery. When this happens, other treatment options are used. Some patients have radiation treatments. Other options include: […] Imatinib mesylate: This is a chemotherapy medicine. The U.S. Food and Drug Administration (FDA) has approved this medicine to treat DFSP in adults when the cancer: […] Cannot be treated with surgery […] Returns after treatment […] Has spread to other parts of the body […] Some chemotherapy medicines kill both cancerous and healthy cells. Imatinib mesylate works differently. It is designed to target specific cancer-causing molecules. This gives it the power to kill cancer cells while preventing serious damage to non-cancerous cells.

• #1 Dermatofibrosarcoma protuberans – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/dermatofibrosarcoma-protuberans/diagnosis-treatment/drc-20576979

  Radiation therapy uses powerful energy beams, such as X-rays and protons, to kill cancer cells. Your health professional may recommend radiation therapy if all the cancer couldn’t be removed during surgery. […] Targeted therapy medicines attack specific chemicals present in cancer cells. By blocking these chemicals, targeted therapy medicines cause cancer cells to die. Some people with dermatofibrosarcoma protuberans have cancer cells that produce an excess protein. A medicine called imatinib (Gleevec) can target those cells and cause them to die. Your health professional may recommend this treatment if surgery is not an option or if your cancer returns after surgery. […] Clinical trials to test new treatments may be an option. Ask your health professional whether you’re eligible to participate in a clinical trial.

• #1 Dermatofibrosarcoma Protuberans Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/1100203-treatment

  Surgical excision remains the mainstay of treatment. Mohs micrographic surgery or other forms of peripheral and deep en face margin assessment (PDEMA) is the treatment of choice. Wide excision can be considered if PDEMA is unavailable. Radiation therapy may be administered if margins are positive and further resection is not feasible or following wide excision if margins are considered narrow. […] In cases of recurrence, repeated resection is preferred. Radiotherapy is an alternative if not previous given or if resection is not feasible. When the disease is unresectable, treatment with imatinib can be considered. […] On October 19, 2006, the US Food and Drug Administration granted approval for imatinib mesylate (Gleevec) as a single agent for the treatment of DFSP. Imatinib mesylate is indicated for the treatment of adult patients with unresectable, recurrent, and/or metastatic DFSP. The recommended oral dose is 800 mg/d. NCCN guidelines recommend imatinib be considered for recurrent unresectable disease.

• #1

  https://journals.lww.com/crst/fulltext/2019/02020/head_and_neck_dermatofibrosarcoma_protuberans_.34.aspx

  Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive, cutaneous, malignant tumor characterized by a high propensity for local relapse and low metastatic potential. […] The authors have also reported the use of imatinib as neoadjuvant therapy in borderline operable head-and-neck DFSP, yielding 66% partial response rates with all the three patients achieving wide excision with negative margins. […] This is in congruence with the results of an European Organisation for Research and Treatment of Cancer (EORTC) study in which out of 16 patients of DFSP treated for 14 weeks with 800 mg daily of neoadjuvant imatinib, 4 (25%) achieved complete remission after wide local excision. […] The cons of neoadjuvant imatinib should be also kept in mind, one of which being long-term outcomes with respect to local margin control and disease-free survival.

• #1

  https://journals.lww.com/crst/fulltext/2019/02020/head_and_neck_dermatofibrosarcoma_protuberans_.34.aspx

  In a pooled analysis of EORTC and SWOG studies, it was observed that a daily dose of 400 mg has similar efficacy to 800 mg daily. […] The authors have reported the Grade 3 toxicities in 33.3% of patients which included anemia, anorexia, and fatigue. […] Use of these simple measures can reduce toxicities such as anemia and fatigue which are an important cause of poor compliance and possible poor outcomes.

• #1 Imatinib Found to Be Effective in Unresectable Dermatofibrosarcoma Protuberans- Cancer Therapy Advisor

  https://www.cancertherapyadvisor.com/news/imatinib-found-to-be-effective-in-unresectable-dermatofibrosarcoma-protuberans/

  In other words, 88.1% of patients with advanced DFSP demonstrated some degree of clinical benefit with imatinib, the researchers wrote. […] Among studies that included data on surgery, 60.3% of patients were able to undergo surgery after treatment with imatinib. […] Almost three-quarters (73.5%) of patients experienced adverse events, with approximately 15% of patients experiencing severe adverse events.

• #1 Dermatofibrosarcoma Protuberans | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/20385

  Imatinib inhibits the PDGF receptor and other receptor tyrosine kinases such as c-KIT. Imatinib has the United States Food and Drug Administration (FDA) approval for unresectable, recurrent, or metastatic DFSP. […] Patients should participate in regular self-examination, and the primary site should be examined every 6 months for 3 to 5 years. […] The initial treatment for localized DFSP is surgical resection with negative margins. […] The risk of inadequate initial resection is high due to the delay in diagnosis and the tumor’s ability to invade surrounding tissues. […] For these reasons, pathological negative margins are necessary. […] The NCCN recommends margins of 2 to 4 cm with clear pathologic margins when clinically feasible. […] Some studies propose that MMS may result in lower local recurrence rates than WLE for DFSP.

• #1 Dermatofibrosarcoma protuberans (DFSP) | Sarcoma UK

  https://sarcoma.org.uk/about-sarcoma/what-is-sarcoma/types-of-sarcoma/dermatofibrosarcoma-protuberans-dfsp/

  This treatment uses anti-cancer drugs to destroy cancer cells. It is sometimes used in patients that have a high risk of the cancer coming back. […] In DFSP, researchers have been learning more about treatment known as immunotherapy. […] Researchers have found that some targeted therapies and immunotherapies have worked well in clinical trials. […] After treatment, you will have regular follow-up appointments for several years. […] If DFSP does reappear, it is important to get treatment as quickly as possible. This could involve further surgery or radiotherapy. […] If you are worried about DFSP returning, speak to your doctor or a clinical nurse specialist. […] But if it does, treatment may involve surgery, targeted therapy, radiotherapy, chemotherapy, or a combination of these treatments.

• #1 Skin cancer types: Dermatofibrosarcoma protuberans diagnosis & treatment

  https://www.aad.org/public/diseases/skin-cancer/dermatofibrosarcoma-protuberans

  This medicine is not right for every patient who has DFSP. For this medicine to work, patients must have certain DNA. Testing is required to find out whether a patient has that DNA. […] If your doctor prescribes imatinib mesylate, you will need to be carefully supervised. […] After taking imatinib mesylate, some patients are able to have surgery because the drug shrinks the DFSP enough so that the cancer can be surgically removed. […] Clinical trial: Some patients are encouraged to join a clinical trial. A clinical trial is a type of research study. This study tests how well new a treatment or a new way of treating a disease works. For some patients, joining a clinical trial may be the best treatment option. […] Lifelong follow-up with your doctors is essential though. DFSP can return after treatment. If DFSP returns, it is often treated with one of the surgeries described above. Some patients receive radiation treatments after surgery. Taking the drug imatinib mesylate may be an option for some patients.

• #1 Skin cancer types: Dermatofibrosarcoma protuberans diagnosis & treatment

  https://www.aad.org/public/diseases/skin-cancer/dermatofibrosarcoma-protuberans

  To reduce the risk of DFSP returning after surgery, your dermatologist may include a second treatment. The second treatment helps to kill cancer cells. […] Dermatologists also are studying new treatment options. One way to reduce DFSP from returning may be to treat patients with both excision and Mohs surgery. In one small study, the cancer did not return when patients received both excision and Mohs. […] More research is needed to find out whether this can reduce the risk of DFSP returning. […] Reconstructive surgery: DFSP can grow deep, so some patients need reconstructive surgery to repair the wound caused by the surgery. […] Radiation treatments: After surgery, some patients receive radiation treatments. These treatments can destroy cancer cells. Because of the long-term risks involved in getting radiation treatments, doctors carefully consider the benefits and the risks of this treatment.

• #1 Dermatofibrosarcoma Protuberans (DFSP): Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/24068-dermatofibrosarcoma-protuberans

  Dermatologists perform Mohs surgery to treat DFSP. […] With proper treatment, DFSP has a high survival rate. […] Surgical removal is the treatment of choice for DFSP. Dermatologists perform Mohs surgery to remove DFSP tumors. […] Healthcare providers use imatinib to treat metastatic DFSP or tumors that are too large or difficult to surgically remove. Imatinib can also shrink the tumor, making surgical removal possible. Radiation treatment can be done for incompletely excised DFSP or inoperable DFSP. […] A prompt diagnosis and treatment are critical to improving your outlook.

• #1 Dermatofibrosarcoma Protuberans: Symptoms & Treatment | NewYork-Presbyterian

  https://www.nyp.org/cancer/skin-cancer/dermatofibrosarcoma-protuberans

  If your tumor has invaded fat, muscle, or bone, you may have other treatments besides surgery to destroy cancer. […] Radiation therapy. We use radiation to treat some large or oddly situated DFSP. We also use it after surgery in some people to kill any remaining cancer cells. NewYork-Presbyterian’s radiation treatment centers are state-of-the-art facilities featuring the latest highly focused radiation technologies. […] Oral anticancer therapy. Imatinib is an oral drug (taken by mouth) used to treat some people with DFSP tumors that cannot be treated with surgery, have come back after prior therapy, or have spread to other parts of the body. In some cases, imatinib shrinks an inoperable tumor enough so that is can be surgically removed. Your doctor will tell you if imatinib is part of your treatment plan and advise you on its use. […] Because DFSP has a tendency to come back, your doctor will want to see you periodically to check you for recurrence. Your team at NewYork-Presbyterian is here to monitor you for as long as you need.

• #1 Guidelines for Diagnosis and Treatment of Cutaneous Sarcomas: Dermatofibrosarcoma Protuberans | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/en-guidelines-for-diagnosis-treatment-cutaneous-articulo-S1578219018303548

  In our experience, MMS is essential in patients with DFSP with positive margins and radiation therapy should not be administered. […] In Europe, imatinib mesylate is approved for the treatment of inoperable primary tumors, inoperable local recurrent tumors, and metastatic DFSP. […] Imatinib appears to be useful as a neoadjuvant treatment for reducing tumor size and facilitating surgery in locally advanced cases with extensive, difficult-to-access lesions.

• #2 Dermatofibrosarcoma Protuberans – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK513305/

  Dermatofibrosarcoma protuberans (DFSP) stands as a rare soft tissue sarcoma that emerges in the dermis and is often found on the trunk and proximal extremities. […] Surgical excision, whether through Mohs micrographic surgery or wide local excision, remains the cornerstone of treatment. However, the potential for local recurrence necessitates vigilant monitoring posttreatment, with radiation therapy sometimes employed to mitigate this risk. […] The initial treatment for localized DFSP is surgical resection with negative margins. […] Wide local excision (WLE) is an option for resection. […] Mohs micrographic surgery (MMS) involves the progressive horizontal slicing of tissue during resection, coupled with immediate microscopic evaluation via frozen section analysis. […] DFSP is radiosensitive tumor. Radiation therapy is rarely used alone in the treatment of DFSP.

• #2 Dermatofibrosarcoma Protuberans: Update on the Diagnosis and Treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7355835/

  Surgical techniques include wide local excision (WLE) with tumor-free margins, Mohs micrographic surgery (MMS), partial or total amputation if the tumor is located on the upper or lower digits. […] Multiple studies have shown that MMS can significantly lower the risk of recurrence of DFSP, compared with WLE. […] The FS-DFSP variant is a much more aggressive tumor with local recurrence in more than 50% of patients and metastasis in 10% to 15% of patients. FS-DFSP treatment needs consultation with a multidisciplinary specialized soft-tissue sarcoma tumor board. Aggressive treatments for FS-DFSP include adequate WLE with clear surgical margins or MMS. Adjuvant radiotherapy and targeted therapy may be used to reduce the incidence of both local recurrence and metastasis. […] Unresectable DFSPs include advanced stage tumors, recurrent tumors without any possibility for further resection due to the size and/or location, or tumors in which further resection is likely to cause severe functional or cosmetic defects. These tumors should be treated with adjuvant radiation and/or targeted therapy in consultation with a multidisciplinary specialized soft-tissue sarcoma tumor board.

• #2 Dermatofibrosarcoma Protuberans FAQs Houston TX – DFSP GA

  https://www.mohssurgery.org/skin-cancer-faqs/dermatofibrosarcoma-protuberans-faqs/

  For many patients with dermatofibrosarcoma protuberans, Mohs surgery is recommended to remove cancer. […] Mohs surgery is now the most common treatment for dermatofibrosarcoma protuberans. […] The traditional method of treating dermatofibrosarcoma protuberans has been the excision technique. […] One alternative treatment for dermatofibrosarcoma protuberans involves targeted drug therapy and radiation. […] Dermatofibrosarcoma protuberans that is treated with traditional excision, where the lesion and a surrounding margin of presumed healthy tissue are removed, has had a history of high rates of recurrence. […] Because Mohs removes the lesion and a surrounding margin, and that margin is then immediately examined for the presence of remaining cancer cells, there is instant feedback for the surgeon. […] Treating DFSP with Mohs micrographic surgery has a 98 percent cure rate. […] Even with recurrent dermatofibrosarcoma protuberans, Mohs surgery has a 98 percent cure rate. […] The recurrence rate for these growths is near zero percent when Mohs surgery is used to remove them.

• #2 Dermatofibrosarcoma protuberans (DFSP) – Phoenix Surgical Dermatology Group

  https://www.psdermgroup.com/skin-cancer/dermatofibrosarcoma-protuberans-phoenix-az/

  Dermatofibrosarcoma protuberans (DFSP) is a rare type of skin cancer that start from the connective tissue cells in the middle layer of the skin. […] At Phoenix Surgical Dermatology Group, our fellowship-trained and board-certified Mohs micrographic surgeon, Dr. Ramin Fathi, is an expert at removing and managing this type of cancer. […] This tumor is most amenable to treatment with Mohs micrographic surgery, as the Mohs technique evaluates 100% of the margin to give the highest chance at cure. […] Many cases of DFSP are curable. Treatment with Mohs micrographic surgery has reported as high as a 98% cure rate. […] According to the National Comprehensive Cancer Network (NCCN), Mohs surgery is considered the first-line treatment option for DFSP. […] After surgery, some patients may be candidates for radiation treatments. This may reduce the risk of DFSP from returning. […] Some patients cannot undergo surgery; hence chemotherapy may be an option (imatinib mesylate). In cases where surgery is deemed too high-risk, our team will work with medical oncologists to consider other treatment options.

• #2 Dermatofibrosarcoma Protuberans – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK513305/

  Current NCCN guidelines recommend the use of adjuvant radiation in the following settings: Positive margins after surgical resection, Negative margins, with the closest margin being less than 1 cm in patients who did not undergo MMS, For recurrent or metastatic disease when surgical excision is not feasible. […] Imatinib inhibits the PDGF receptor and other receptor tyrosine kinases such as c-KIT. Imatinib has the United States Food and Drug Administration (FDA) approval for unresectable, recurrent, or metastatic DFSP. […] Patients should participate in regular self-examination, and the primary site should be examined every 6 months for 3 to 5 years. Lifelong examination annually follows.

• #2 The Role of Postoperative Radiotherapy in the Management of Dermatofibrosarcoma Protuberans: A Multidisciplinary Systematic Review

  https://www.mdpi.com/2077-0383/13/6/1798

  Dermatofibrosarcoma protuberans (DFSP) is a superficial soft tissue sarcoma, and surgical excision is the first-line treatment. […] The role of integrated approaches, such as radiotherapy (RT) and/or systemic treatment (i.e., imatinib mesylate), has been reported, both in the neoadjuvant and adjuvant setting, as well as for the definitive treatment in patients not eligible for surgery. […] After the primary surgical management of DFSP, postoperative radiotherapy may either be considered as adjuvant treatment (presence of risk factors, i.e., close margins, recurrent tumours, aggressive histological subtypes) or as salvage treatment (positive margins) and should be assessed within the frame of multidisciplinary evaluation.

• #2 Skin cancer types: Dermatofibrosarcoma protuberans diagnosis & treatment

  https://www.aad.org/public/diseases/skin-cancer/dermatofibrosarcoma-protuberans

  The results from a small study show that radiation may reduce the risk of DFSP from returning. Researchers followed 14 patients who received radiation treatments, mostly after surgery. Most patients, 86%, remained cancer-free. The average follow-up period exceeded 10 years. […] Some patients cannot undergo surgery. When this happens, other treatment options are used. Some patients have radiation treatments. Other options include: […] Imatinib mesylate: This is a chemotherapy medicine. The U.S. Food and Drug Administration (FDA) has approved this medicine to treat DFSP in adults when the cancer: […] Cannot be treated with surgery […] Returns after treatment […] Has spread to other parts of the body […] Some chemotherapy medicines kill both cancerous and healthy cells. Imatinib mesylate works differently. It is designed to target specific cancer-causing molecules. This gives it the power to kill cancer cells while preventing serious damage to non-cancerous cells.

• #2 Dermatofibrosarcoma Protuberans Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/1100203-treatment

  Surgical excision remains the mainstay of treatment. Mohs micrographic surgery or other forms of peripheral and deep en face margin assessment (PDEMA) is the treatment of choice. Wide excision can be considered if PDEMA is unavailable. Radiation therapy may be administered if margins are positive and further resection is not feasible or following wide excision if margins are considered narrow. […] In cases of recurrence, repeated resection is preferred. Radiotherapy is an alternative if not previous given or if resection is not feasible. When the disease is unresectable, treatment with imatinib can be considered. […] On October 19, 2006, the US Food and Drug Administration granted approval for imatinib mesylate (Gleevec) as a single agent for the treatment of DFSP. Imatinib mesylate is indicated for the treatment of adult patients with unresectable, recurrent, and/or metastatic DFSP. The recommended oral dose is 800 mg/d. NCCN guidelines recommend imatinib be considered for recurrent unresectable disease.

• #2 Dermatofibrosarcoma Protuberans Medication: Molecular-targeted therapy

  https://emedicine.medscape.com/article/1100203-medication

  Although medical therapy is not a first-line treatment for localized dermatofibrosarcoma protuberans (DFSP), the approved molecular-targeted drug, imatinib mesylate, is an effective oral medication for unresectable, recurrent, and/or metastatic DFSP. […] Before starting imatinib therapy, cytogenetic studies to confirm PDGFB gene rearrangement may be necessary for predicting the clinical response. […] Imatinib mesylate is an inhibitor of receptor tyrosine kinase for PDGF. It inhibits PDGF-B receptor-mediated cellular events, the key pathogenetic pathway in DFSP. […] Neoadjuvant imatinib therapy for dermatofibrosarcoma protuberans. […] Efficacy and biological activity of imatinib in metastatic dermatofibrosarcoma protuberans (DFSP). […] Imatinib mesylate as a preoperative therapy in dermatofibrosarcoma: results of a multicenter phase II study on 25 patients.

• #2 Skin cancer types: Dermatofibrosarcoma protuberans diagnosis & treatment

  https://www.aad.org/public/diseases/skin-cancer/dermatofibrosarcoma-protuberans

  This medicine is not right for every patient who has DFSP. For this medicine to work, patients must have certain DNA. Testing is required to find out whether a patient has that DNA. […] If your doctor prescribes imatinib mesylate, you will need to be carefully supervised. […] After taking imatinib mesylate, some patients are able to have surgery because the drug shrinks the DFSP enough so that the cancer can be surgically removed. […] Clinical trial: Some patients are encouraged to join a clinical trial. A clinical trial is a type of research study. This study tests how well new a treatment or a new way of treating a disease works. For some patients, joining a clinical trial may be the best treatment option. […] Lifelong follow-up with your doctors is essential though. DFSP can return after treatment. If DFSP returns, it is often treated with one of the surgeries described above. Some patients receive radiation treatments after surgery. Taking the drug imatinib mesylate may be an option for some patients.

• #2 Dermatofibrosarcoma Protuberans: Update on the Diagnosis and Treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7355835/

  Multiple studies have shown that DFSP is a radio-responsive tumor and adjuvant radiation therapy is effective to control tumor growth and reduce the incidence of postoperative recurrence. […] Imatinib mesylate (IM) is a potent and specific protein tyrosine kinase inhibitor interfering with the phosphorylation and activation of the PDGF receptor which is constitutively activated due to translocation and fusion between PDGFB and COL1A1 genes. […] The efficacy of IM was observed in localized and metastatic DFSP with t(17; 22), but not in FS-DFSP lacking t(17; 22). […] In the cases of IM resistance, other multikinase inhibitors, including sunitinib, sorafenib and pazopanib, can be considered since treatment with these inhibitors showed effectiveness in IM resistant DFSP patients.

• #2

  https://journals.lww.com/md-journal/fulltext/2015/05010/target_therapy_of_unresectable_or_metastatic.26.aspx

  Dermatofibrosarcoma protuberans (DFSP) is a rare, plaque-like tumor of the cutaneous tissue occurring more on the trunk than the extremities and neck. […] Surgery is the optimal treatment for DFSP, but less effective in locally advanced or metastatic patients, as is the case with chemotherapy and radiotherapy. […] The aim of this study was to assess retrospectively the therapeutic activity and safety of imatinib on 22 Chinese patients with locally inoperative or metastatic DFSP at a single institution. […] Imatinib therapy is well tolerated with a safety profile and is the therapy of choice in locally inoperative or metastatic DFSP. Neoadjuvant treatment of locally advanced or metastatic DFSP with imatinib improves surgical outcomes and may facilitate resection of difficult tumors. […] As the molecular pathogenesis of DFSP was elucidated, imatinib target therapy, which is a tyrosine kinase inhibitor specifically directed at BCR/ABL, KIT, and PDGFR- and PDGFR-, was introduced into the treatment of DFSP.

• #2 Dermatofibrosarcoma protuberans – Dermatology Advisor

  https://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/dermatofibrosarcoma-protuberans/

  Radiation therapy (RT) has been used adjuvantly in patients with recurrent tumors and positive margins on excision in whom more extensive surgery is deemed functionally or cosmetically unacceptable. […] A new molecular-based therapy for DFSP has been developed in the form of the imatinib mesylate (STI-571, trade name GleevecTM), a multikinase inhibitor targeting the PDGF pathway. […] Imatinib has been used in the treatment of unresectable locally advanced, recurrent, and metastatic DFSP, showing a response rate of about 70% in preliminary trials. […] More recently, neo-adjuvant Imatinib therapy has been employed to reduce preoperative tumor size, facilitating resection of previously unresectable disease in a few cases. […] Sunitinib has also been shown helpful in the treatment of patients with locally advanced or inoperable DFSP after imatinib failure, helping to slow disease progression and decrease tumor size in several reported patients.

• #2 Dermatofibrosarcoma protuberans | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/dermatofibrosarcoma-protuberans?lang=us

  Dermatofibrosarcoma protuberans has an excellent prognosis after complete resection but has a marked tendency to recur locally if inadequate surgical resection margins are obtained. […] Wide excision with 2 cm margins or Micrographic Mohs surgery with smaller margins is the treatment of choice for FS-DFSP, to avoid local recurrence and metastasis. […] Radiation has a role in cases with incomplete surgical resection, whereas the PDGFR-inhibitor imatinib is effective in irresectable, locally advanced and metastatic cases.

• #2 Dermatofibrosarcoma protuberans – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/dermatofibrosarcoma-protuberans/diagnosis-treatment/drc-20576979

  Radiation therapy uses powerful energy beams, such as X-rays and protons, to kill cancer cells. Your health professional may recommend radiation therapy if all the cancer couldn’t be removed during surgery. […] Targeted therapy medicines attack specific chemicals present in cancer cells. By blocking these chemicals, targeted therapy medicines cause cancer cells to die. Some people with dermatofibrosarcoma protuberans have cancer cells that produce an excess protein. A medicine called imatinib (Gleevec) can target those cells and cause them to die. Your health professional may recommend this treatment if surgery is not an option or if your cancer returns after surgery. […] Clinical trials to test new treatments may be an option. Ask your health professional whether you’re eligible to participate in a clinical trial.

• #2 Dermatofibrosarcoma protuberans (DFSP) | Sarcoma UK

  https://sarcoma.org.uk/about-sarcoma/what-is-sarcoma/types-of-sarcoma/dermatofibrosarcoma-protuberans-dfsp/

  This treatment uses anti-cancer drugs to destroy cancer cells. It is sometimes used in patients that have a high risk of the cancer coming back. […] In DFSP, researchers have been learning more about treatment known as immunotherapy. […] Researchers have found that some targeted therapies and immunotherapies have worked well in clinical trials. […] After treatment, you will have regular follow-up appointments for several years. […] If DFSP does reappear, it is important to get treatment as quickly as possible. This could involve further surgery or radiotherapy. […] If you are worried about DFSP returning, speak to your doctor or a clinical nurse specialist. […] But if it does, treatment may involve surgery, targeted therapy, radiotherapy, chemotherapy, or a combination of these treatments.

• #2 Dermatofibrosarcoma Protuberans, Version 1.2025, NCCN Clinical Practice Guidelines In Oncology in: Journal of the National Comprehensive Cancer Network Volume 23 Issue 1 (2025)

  https://jnccn.org/abstract/journals/jnccn/23/1/article-e250001.xml

  Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous soft tissue sarcoma and affects an estimated 1,500 people annually in the United States. Initial treatment is through surgical excision, and care should be taken to ensure that negative margins are achieved to minimize recurrence. If achieving negative margins or resection is not feasible, radiation therapy or systemic treatment are options that may be considered by a multidisciplinary team. The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) outline recommended treatment options available for DFSP. […] Initial treatment of DFSP is surgical. Because of its proclivity for irregular and frequently deep subclinical extensions, every effort should be made to completely remove this tumor at initial therapy. Excision with Mohs micrographic surgery (Mohs) or other forms of peripheral and deep en face margin assessment (PDEMA) is recommended over WLE. When anatomic structures at the deep margin (eg, major vessels, nerves, bone) preclude complete histologic evaluation of the marginal surface via Mohs or other forms of PDEMA, these surgical techniques should be used to evaluate as much of the marginal surface as feasible.

• #2 Dermatofibrosarcoma protuberans (DFSP) | Sarcoma UK

  https://sarcoma.org.uk/about-sarcoma/what-is-sarcoma/types-of-sarcoma/dermatofibrosarcoma-protuberans-dfsp/

  If you have been diagnosed with DFSP, you should be referred to a specialist sarcoma multidisciplinary team (MDT) for treatment. […] In most cases, surgery is used to treat DFSP. The surgeon will remove the tumour and will aim to take out an area of normal tissue too this is known as taking a margin. This is to make sure that all the cancer has been removed. […] In rare cases, DFSP cannot be removed through surgery in a safe way. This may be because it has come back after surgery, it is too large to be removed by surgery, or it has spread to other parts of the body. […] Targeted therapy is a treatment that targets specific characteristics within cancer cells to stop them from growing and spreading. […] A drug called imatinib is sometimes used in these cases. […] This treatment uses high-energy radiation to destroy cancer cells. Radiotherapy is used to treat DFSP that cannot be removed through surgery or that has come back after surgery.

• #3 Dermatofibrosarcoma Protuberans Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/1100203-treatment

  Surgical excision remains the mainstay of treatment. Mohs micrographic surgery or other forms of peripheral and deep en face margin assessment (PDEMA) is the treatment of choice. Wide excision can be considered if PDEMA is unavailable. Radiation therapy may be administered if margins are positive and further resection is not feasible or following wide excision if margins are considered narrow. […] In cases of recurrence, repeated resection is preferred. Radiotherapy is an alternative if not previous given or if resection is not feasible. When the disease is unresectable, treatment with imatinib can be considered. […] On October 19, 2006, the US Food and Drug Administration granted approval for imatinib mesylate (Gleevec) as a single agent for the treatment of DFSP. Imatinib mesylate is indicated for the treatment of adult patients with unresectable, recurrent, and/or metastatic DFSP. The recommended oral dose is 800 mg/d. NCCN guidelines recommend imatinib be considered for recurrent unresectable disease.

• #3 Skin cancer types: Dermatofibrosarcoma protuberans diagnosis & treatment

  https://www.aad.org/public/diseases/skin-cancer/dermatofibrosarcoma-protuberans

  Mohs surgery: For many DFSP patients, Mohs (pronounced „Moes”) surgery may be recommended. This specialized surgery is only used to treat skin cancer. This surgery allows the Mohs surgeon to remove less tissue than is removed during excision (the surgery described above.) […] During Mohs surgery, the Mohs surgeon cuts out the tumor plus a very small amount of healthy-looking tissue surrounding the tumor. While the patient waits, the Mohs surgeon uses a microscope to look at what was removed. The surgeon is looking for cancer cells. […] If the Mohs surgeon finds cancer cells at the edge of the removed tissue, the surgeon will remove another small amount of tissue and look at it under the microscope. This process continues until the surgeon no longer sees cancer cells along the edge of the removed tissue. Your Mohs surgeon may refer to this edge as the margin. When the Mohs surgeon no longer sees cancer cells along the edges, the surgeon may tell you that the margins look clear.

• #3 Dermatofibrosarcoma protuberans – Dermatology Advisor

  https://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/dermatofibrosarcoma-protuberans/

  Surgery remains the therapeutic modality of choice for DFSP, with the accuracy of the initial procedure being the main prognostic factor for locoregional recurrence and overall survival. […] Mohs micrographic surgery (MMS) and wide local excision (WLE) are the two most commonly performed surgical treatment modalities. […] MMS is regarded as the best treatment for DFSP by many investigators, and has been shown to be the superior modality when compared to WLE. […] Standard surgical excision continues to be used, but must take into account clinically unapparent tumor fascicles, which can extend into adjacent tissue for long distances in a horizontal plane. […] Thus, the standard surgical margin for the treatment of DFSP is 2 to 3 cm. […] DFSP is a radio-sensitive tumor, but results have been mixed.

• #3 Dermatofibrosarcoma Protuberans, Version 1.2025, NCCN Clinical Practice Guidelines In Oncology in: Journal of the National Comprehensive Cancer Network Volume 23 Issue 1 (2025)

  https://jnccn.org/abstract/journals/jnccn/23/1/article-e250001.xml

  Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous soft tissue sarcoma and affects an estimated 1,500 people annually in the United States. Initial treatment is through surgical excision, and care should be taken to ensure that negative margins are achieved to minimize recurrence. If achieving negative margins or resection is not feasible, radiation therapy or systemic treatment are options that may be considered by a multidisciplinary team. The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) outline recommended treatment options available for DFSP. […] Initial treatment of DFSP is surgical. Because of its proclivity for irregular and frequently deep subclinical extensions, every effort should be made to completely remove this tumor at initial therapy. Excision with Mohs micrographic surgery (Mohs) or other forms of peripheral and deep en face margin assessment (PDEMA) is recommended over WLE. When anatomic structures at the deep margin (eg, major vessels, nerves, bone) preclude complete histologic evaluation of the marginal surface via Mohs or other forms of PDEMA, these surgical techniques should be used to evaluate as much of the marginal surface as feasible.

• #3 Dermatofibrosarcoma Protuberans: Update on the Diagnosis and Treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7355835/

  Multiple studies have shown that DFSP is a radio-responsive tumor and adjuvant radiation therapy is effective to control tumor growth and reduce the incidence of postoperative recurrence. […] Imatinib mesylate (IM) is a potent and specific protein tyrosine kinase inhibitor interfering with the phosphorylation and activation of the PDGF receptor which is constitutively activated due to translocation and fusion between PDGFB and COL1A1 genes. […] The efficacy of IM was observed in localized and metastatic DFSP with t(17; 22), but not in FS-DFSP lacking t(17; 22). […] In the cases of IM resistance, other multikinase inhibitors, including sunitinib, sorafenib and pazopanib, can be considered since treatment with these inhibitors showed effectiveness in IM resistant DFSP patients.

• #3 Dermatofibrosarcoma Protuberans, Version 1.2025, NCCN Clinical Practice Guidelines In Oncology in: Journal of the National Comprehensive Cancer Network Volume 23 Issue 1 (2025)

  https://jnccn.org/abstract/journals/jnccn/23/1/article-e250001.xml

  DFSP can be treated by targeted platelet-derived growth factor receptors. Imatinib mesylate, a protein tyrosine kinase inhibitor, has shown clinical activity against DFSP, which has led to its approval by the US FDA for the treatment of unresectable, recurrent, and/or metastatic DFSP in adult patients. […] Imatinib mesylate should be considered in cases where the disease is unresectable after multiple resections, or if unacceptable functional or cosmetic outcomes will occur with further resection.

• #3 Dermatofibrosarcoma protuberans: from translocation to targeted therapy | Cancer Biology & Medicine

  https://www.cancerbiomed.org/content/12/4/375

  DFSP is generally perceived as being resistant to conventional chemotherapy. There is some anecdotal evidence of response to methotrexate, although the experience is limited. A breakthrough in the management of metastatic patients was possible following the discovery of the translocation t(17;22)(q22;q13) (COL1A1;PDGFB), whereby efforts were directed towards targeting PDGFR. Imatinib is an inhibitor of PDGFR, ABL and KIT, and blocks PDGF signaling, interfering with phosphorylation of the receptor tyrosine kinase. […] The clinical activity of imatinib was subsequently described in several other case reports. […] Imatinib was also studied in a neo-adjuvant setting with the aim to improve on surgical outcomes. […] Patients lacking the t(17;22) translocation do not usually respond to imatinib. Furthermore, the pigmented-variant DFSP seems to be unresponsive to imatinib despite cases presenting the translocation, although larger cohorts will be needed to validate these findings. To our knowledge, there are currently no trials investigating novel agents designed specifically for DFSP. Treatment options available for translocation negative or imatinib-resistant DFSP are limited.

• #3

  https://journals.lww.com/md-journal/fulltext/2015/05010/target_therapy_of_unresectable_or_metastatic.26.aspx

  The aim of this study was to assess retrospectively the therapeutic activity and safety of imatinib on 22 Chinese patients with locally unresectable or metastatic DFSP at a single institution. […] The intent of neoadjuvant therapy should be to convert the unresectable tumor into a resectable tumor with negative margins or to limit the extent of reconstruction and comorbidities from excision. […] Our study documents that the utility of imatinib neoadjuvant therapy has the potential to transform patients with surgically challenging or recurrent tumors into better operative candidates, and the response observed in histologic findings following neoadjuvant imatinib may be useful in determining the best postoperative adjuvant therapy. […] In conclusion, imatinib in standard dosage allowed prompt and impressive clinical improvement, so that imatinib mesylate appears to be the therapy of choice in patients with metastatic or locally advanced disease not amenable to surgery.

• #3

  https://link.springer.com/article/10.1007/s11864-022-00979-9

  Complete remission of a periorbital dermatofibrosarcoma protuberans with adjuvant imatinib mesylate in a child. […] A favorable outcome of advanced dermatofibrosarcoma protuberans under treatment with sunitinib after imatinib failure. […] Dermatofibrosarcoma protuberans (DFSP) successfully treated with sorafenib: case report. […] Chemosensitivity in pediatric dermatofibrosarcoma protuberans.

• #3 Dermatofibrosarcoma Protuberans: Symptoms & Treatment | NewYork-Presbyterian

  https://www.nyp.org/cancer/skin-cancer/dermatofibrosarcoma-protuberans

  Dermatofibrosarcoma protuberans (DFSP) is a rare type of slow-growing skin cancer that arises from the fibrous tissue of the skin. If left untreated, it can invade deeper structures under the skin, such as fat, muscle, or bone and can even metastasize to other organs such as the lungs so the sooner it is found and treated, the better the outcome. NewYork-Presbyterian’s skin cancer and sarcoma specialists are experts in the treatment of DFSP and offer a full range of therapies to meet your needs. […] Your treatment for DFSP will most likely include surgery to remove the tumor. NewYork-Presbyterian’s skin cancer surgeons often use Mohs surgery, which must be performed by specially trained physicians. During this procedure, the tumor is removed layer by layer and microscopically examined each time to ensure elimination of cancer while preserving as much healthy skin as possible.

• #3 Dermatofibrosarcoma Protuberans Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/1100203-treatment

  Although some Mohs surgeons consider it unnecessary, taking an extra layer of tissue around the surgical defect at the completion of Mohs surgery for permanent pathology section and/or CD34 immunostaining may potentially enhance the cure rate. […] Studies have demonstrated a low recurrence rate after surgery for DFSP if a multidisciplinary approach and careful pathology margin assessment are used.

Zobacz więcej