Materiały źródłowe

• #1 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. This condition can present as a slowly developing, robust plaque, exhibiting shades of pink or violet, underscoring the challenge of timely diagnosis. […] DFSP is an uncommon soft tissue sarcoma primarily found on the trunk and proximal extremities that typically appears as a slowly progressing, firm, violet-red, or blue plaque. Because DFSP is a slow-growing tumor, the diagnosis is often delayed for months to years. […] DFSP typically manifests as an asymptomatic, skin-colored to red-brown firm plaque, eventually progressing to multiple raised nodules with a violaceous to red-brown hue. […] Lesions exhibit slow growth over months to years, occasionally resembling keloids or dermatofibromas, leading to misdiagnosis, particularly in the early stages.

• #2 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. It has a high local recurrence rate but low metastatic potential. In early stages of DFSP, the patient typically notices a slow-growing, small, firm, painless, skin-colored dermal plaque, subcutaneous thickening or atrophic non-protuberant lesion. The early non-protuberant lesions gradually enlarge to form protuberant, indurated, reddish-blue or violaceous nodules in protuberant stages. If untreated, the tumors can locally invade more deeply into the fascia, muscle, periosteum, bone and occasionally metastasize to other organs in advanced stages. The tumor cells most frequently metastasize to lung, brain, bone, visceral organs, lymph nodes and soft tissues. This insidious growing process takes several months or years to as long as 60 years. The reported tumor sizes vary in a wide range from 0.5 to 10 cm in diameter, with a mean of 23.5 cm. Most DFSPs are localized on the trunk (40-50%), followed by proximal extremities (30-40%), and then the head and neck (10-15%).

• #3 Dermatofibrosarcoma Protuberans – SFA

  https://curesarcoma.org/sarcoma-subtypes/dermatofibrosarcoma-protuberans/

  Dermatofibrosarcoma protuberans is a rare, locally aggressive skin tumor characterized by fibroblastic proliferation and a distinctive genetic fusion. […] DFSP typically presents as a nodular or multinodular cutaneous mass, often with a history of slow but persistent growth. Early lesions may show a plaque-like growth with peripheral red discoloration. These neoplasms may show rapid enlargement during pregnancy or due to tumor progression to fibrosarcomatous DFSP. […] DFSP is characterized by locally aggressive growth and frequent, often repeated local recurrences unless widely excised. The rate of local recurrences varies from 20% to 50% in the setting of inadequate margins. In contrast, ordinary DFSP almost never metastasizes. Higher-grade fibrosarcomatous progression is seen in 5% of cases. Fibrosarcomatous DFSP exhibits more aggressive behavior than ordinary DFSP, and 10–15% of patients develop distant metastases, most often to the lungs. Histological grading has not been shown to be prognostic in fibrosarcomatous DFSP.

• #4 Skin cancer types: Dermatofibrosarcoma protuberans signs & symptoms

  https://www.aad.org/public/diseases/skin-cancer/types/common/dfsp/symptoms

  This skin cancer tends to grow slowly, so it often goes unnoticed for months or even years. When dermatofibrosarcoma protuberans (DFSP) first appears on the skin, a person may notice: […] The first sign of this skin cancer may be reddish brown or pink patch of raised skin that looks like a scar. […] As DFSP grows inside the middle layer of skin, it tends to push on the top layer of skin. You may see a lump, also known as a protuberan. The lump may feel hard or rubbery. As the lump grows, it stretches the skin. You may notice that the affected skin: […] Over time, more protuberans (lumps) can appear. Once these appear, DFSP tends to grow quickly. In adults, the protuberans often range in color from reddish brown to violet. In young patients, DFSP tends to be blue or red in color. […] Given time to grow, many protuberans can appear on the surface of the skin. […] If you are worried about a growth on your skin, you should see a dermatologist. […] DFSP tends to occur between the ages of 20 and 50.

• #5 Dermatofibrosarcoma Protuberans (DFSP) – Rare Cancers Australia

  https://knowledge.rarecancers.org.au/knowledgebase/cancer-types/260/dermatofibrosarcoma-protuberans-dfsp

  Dermatofibrosarcoma protuberans (DFSP) is a rare type of sarcoma cancer arising from bones and/or soft tissue that starts in the skin. […] DFSP is slightly more common in males, and tends to develop in people between 20-59 years of age. However, anyone can develop this disease. […] Early symptoms of DFSP may include: Painless plaque (thickened area of skin) or nodule on skin. Rubbery or firm nodule on skin. Red-brown, pink, or blueish discolouration of nodule. Soft, depressed (or indented) area of skin (rare). Ulceration of nodule over time. […] Not everyone with the symptoms above will have cancer but see your general practitioner (GP) if you are concerned.

• #6 Dermatofibrosarcoma Protuberans (DFSP) – Dawes Fretzin Dermatology Group

  https://dawesfretzin.com/terminology/dermatofibrosarcoma-protuberans-dfsp/

  As the lump grows, it stretches the skin. You may notice that the affected skin: Becomes tender. Cracks and bleeds. Feels hard, and the lump seems cemented in the skin. […] Over time, more protuberans (lumps) can appear. Once these appear, DFSP tends to grow quickly. […] DFSP can form anywhere on the skin. It is, however, more likely to develop on the trunk (chest, back, abdomen, shoulder, buttocks) and arm or leg. […] If you are worried about a growth on your skin, you should see a dermatologist. […] DFSP tends to occur between the ages of 20 and 50. […] Although unusual, children get DFSP. Sometimes, a child is born with DFSP. […] In treating patients with DFSP, dermatologists have learned that this cancer sometimes begins on skin that was badly injured. […] If the diagnosis is DFSP, you will need a thorough physical exam.

• #7 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Dermatofibrosarcoma-Protuberans-(DFSP)-Signs-and-Symptoms.aspx

  Dermatofibrosarcoma protuberans (DFSP) is a rare tumor affecting the deep layers of the skin. […] The general lack of symptoms in DfSP causes the diagnosis of this disease to often occur well after tumor development begins in most patients. In fact, it is estimated that less than a fifth of all DSFP patients report pain or redness. […] The first sign of DFSP is a soft to firm skin patch that slightly changes over the months or years in most cases. It is flesh-colored, reddish or purple, and either flat, raised, or even depressed, in some patients. […] After several years, the tumor may start to grow more aggressively, swell or become painful, or bleed. […] The DSFP lesion is usually first noticed when it starts to grow rapidly, or becomes more prominent. At this point, the growing tumor pushes up against the superficial skin to form a firm or rubbery patch, over which the skin is stretched and may become tender, ulcerate, bleed, or feel fixed to the tumor. […] The tumor may also enter a rapid growth phase during pregnancy.

• #8 Dermatofibrosarcoma Protuberans: Practice Essentials, Background, Pathophysiology

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

  Dermatofibrosarcoma protuberans (DFSP) is a relatively uncommon soft tissue neoplasm of intermediate- to low-grade malignancy of unknown etiology. DFSP presents as a slow-growing, firm, multilobular nodule or plaque with irregular margins. […] A small subset of DFSP patients presents with fibrosarcomatous progression. This fibrosarcomatous progression DFSP variant (FS-DFSP) is more aggressive in nature, and the clinical outcome can be poor. […] DFSP is a very slowly growing tumor and the diagnosis is often delayed for months to years and misdiagnosis can occur due to inadequate tissue sampling or superficial biopsy. […] Because of the high local recurrence rate, patients require close follow-up care after treatment. Most recurrences occur within 3 years of the primary excision. […] Dermatofibrosarcoma protuberans (DFSP) is characterized by its aggressive local invasion. The tumor invades local tissue by extending tentaclelike projections underneath healthy skin, rendering complete removal of the tumor very difficult. Incomplete removal of these neoplastic cells results in a high local recurrence rate.

• #9 Dermatofibrosarcoma protuberans – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/dermatofibrosarcoma-protuberans/symptoms-causes/syc-20576872

  As dermatofibrosarcoma protuberans grows, lumps of tissue (protuberans) may form near the surface of the skin. […] Dermatofibrosarcoma protuberans might look like a pimple or feel like a firm patch of skin at first. As it grows, lumps of tissue (protuberans) may form near the surface of the skin. This skin cancer often forms on the arms, legs and trunk. […] Symptoms of dermatofibrosarcoma protuberans include: A lump or lumps on the skin that look like pimples, scars or birthmarks. A lump or lumps on the skin that may feel hard to the touch or rubberlike. A patch of skin that feels firm to the touch. […] The lumps typically appear on the arms, legs or trunk. They rarely form on the head or neck. […] Dermatofibrosarcoma protuberans grows slowly and rarely spreads beyond the skin.

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

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

  Dermatofibrosarcoma protuberans appears as firm lumps of reddish-brown tissue on persons scalp. Dermatofibrosarcoma protuberans is a rare, slow-growing skin cancer. Early symptoms of DFSP are easy to dismiss or not notice. The tumors typically appear on your chest, back, shoulders, abdomen or buttocks. Tumors can also form on your arms, legs, scalp and inside of your mouth. At first, you may notice a small patch of skin that looks like a bruise. The spot is flat and painless. It may feel rough and look discolored. DFSP symptoms become more noticeable as the cancer grows. The growing tumor pushes into the top layer of skin (epidermis). Firm lumps (nodules) of tissue appear on the skin (protuberans). You may notice that the nodules are: Easy to crack open or bleed. Firmly attached to your skin (dont move). Getting bigger and stretching your skin. Hard or rubbery. Reddish-brown to violet, blue or red. Tender.

• #11 Dermatofibrosarcoma Protuberans: Symptoms and Treatment

  https://www.healthline.com/health/skin-cancer/dermatofibrosarcoma-protuberans

  Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing skin cancer. People with DFSP have a highly favorable outlook, provided they can treat the cancer before it spreads. […] DFSP usually starts as a small, firm patch of skin that can feel rubbery or hard to the touch. It can look like a dermatofibroma, a harmless and common skin growth. […] Common symptoms of DFSP include: a small bump or patch on the skin that grows slowly, a firm, raised nodule that can be purple, red, or brown, pain or tenderness around the affected area, bleeding from the affected area, though this is rare. […] Because DFSP grows slowly, it often takes years for people to seek medical attention, which can delay diagnosis and treatment. […] The outlook for people with DFSP is generally good, especially with early detection and surgical removal. DFSP rarely spreads to other parts of the body. The overall 10-year survival rate for DFSP is 99%.

• #12 Dermatofibrosarcoma protuberans

  https://dermnetnz.org/topics/dermatofibrosarcoma-protuberans

  Dermatofibrosarcoma protuberans usually presents as a painless thickened area of skin (plaque) and/or nodule that feels rubbery or firm to touch and is fixed to the underlying skin. It may be red-brown or skin coloured. It usually grows very slowly over months to years. […] Rarely it presents as a soft depressed area of the skin making the diagnosis even more difficult. Dermatofibrosarcoma protuberans may range in size from 0.525 cm in diameter. Fifty to sixty per cent of tumours arise on the trunk, often in the shoulder and chest area. The remaining 35% of tumours are found on the limbs and 10 to 15% on the head and neck region. […] Dermatofibrosarcoma protuberans is often diagnosed when it enters a more rapid growth phase giving rise to larger lesions. Neglected tumours may reach large proportions.

• #13 Dermatofibrosarcoma Protuberans – Cancer Diagnosis

  https://www.leiomyosarcoma.org/dermatofibrosarcoma-protuberans/

  DFSP is typically first noticed as a small, firm patch of skin with a diameter of one to five centimeters. […] Typically the first sign of DFSP that people notice is a flat or slightly raised patch of skin, which feels rubbery or hard to the touch. […] As DFSP develops, a violet, red-brown or colorless tumor will appear. […] If a tumor has appeared, then DFSP is growing more quickly and the tumors can crack open, bleed and become painful. […] You should see a dermatologist if the growth does not go away within two weeks, a scar changes or anything on your skin is growing or changing quickly.

• #14 Dermatofibrosarcoma protuberans

  https://dermnetnz.org/topics/dermatofibrosarcoma-protuberans

  The absence of symptoms often leads to a delay in diagnosis of dermatofibrosarcoma protuberans. Redness and pain only occur in 15% of cases. It is often mistaken for other skin conditions, particularly in the early stages. […] The tumour only metastasises in 5% of cases. It spreads in 1% via lymphatic vessels to the regional lymph glands and in 4% via the bloodstream, most commonly to the lung followed by the brain, bone and heart. […] Local recurrences arise in 1120% of cases, usually within 3 years of initial surgery, so follow-up is important. The recurrences are treated surgically as described for the original primary tumour.

• #15 Dermatofibrosarcoma protuberans: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/dermatofibrosarcoma-protuberans/

  Dermatofibrosarcoma protuberans is a rare type of cancer that causes a tumor in the deep layers of skin. This condition is a type of soft tissue sarcoma, which are cancers that affect skin, fat, muscle, and similar tissues. […] In dermatofibrosarcoma protuberans, the tumor most often starts as a small, firm patch of skin, usually 1 to 5 centimeters in diameter, that is usually purplish, reddish, or flesh-colored. The tumor typically grows slowly and can become a raised nodule. Occasionally, the tumor begins as a flat or depressed patch of skin (plaque). Tumors are most commonly found on the torso and can also be found on the arms, legs, head, or neck. Affected individuals usually first show signs of this condition in their thirties, but the age at which a tumor appears varies widely. […] In dermatofibrosarcoma protuberans, the tumor has a tendency to return after being removed. However, it does not often spread to other parts of the body (metastasize).

• #16 Dermatofibrosarcoma Protuberans FAQs Houston TX – DFSP GA

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

  The first sign of dermatofibrosarcoma protuberans is usually a small bump on the skin. The area of skin will feel rubbery or hard to the touch. This will be red and may resemble a deep-seated pimple. As it grows, it will turn into a reddish-brown and slightly raised patch of skin. By the time the red bump becomes the brown patch, the cancer is growing more rapidly and may cause pain or bleed. This only occurs in roughly 15 percent of people with dermatofibrosarcoma protuberans. […] Left untreated, dermatofibrosarcoma protuberans tend to grow into surrounding fat, muscle, and even bone. This is most common on the torso, but it can also be seen on the arms, legs, head, and neck. Because DFSP starts as a small lesion, usually from 1-5 centimeters in diameter, it is often overlooked. It also tends to grow very slowly, so changes aren’t obvious to the person.

• #17 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. It has a high local recurrence rate but low metastatic potential. In early stages of DFSP, the patient typically notices a slow-growing, small, firm, painless, skin-colored dermal plaque, subcutaneous thickening or atrophic non-protuberant lesion. The early non-protuberant lesions gradually enlarge to form protuberant, indurated, reddish-blue or violaceous nodules in protuberant stages. If untreated, the tumors can locally invade more deeply into the fascia, muscle, periosteum, bone and occasionally metastasize to other organs in advanced stages. The tumor cells most frequently metastasize to lung, brain, bone, visceral organs, lymph nodes and soft tissues. This insidious growing process takes several months or years to as long as 60 years. The reported tumor sizes vary in a wide range from 0.5 to 10 cm in diameter, with a mean of 23.5 cm. Most DFSPs are localized on the trunk (40-50%), followed by proximal extremities (30-40%), and then the head and neck (10-15%).

• #18 Dermatofibrosarcoma Protuberans Clinical Presentation: History, Physical Examination

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

  Mostly the tumor is mobile upon palpation; however, fixation to deeper structures such as fascia, muscle, and bone may occur in the later stage of the tumor. […] DFSP most commonly occurs on the trunk (42-72%), followed by the proximal extremities (16-30%). DFSP rarely occurs above the neck (10-16%) and extremely rarely on the breast.

• #19 Dermatofibrosarcoma protuberans (DFSP) | Sarcoma UK

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

  DFSP can occur anywhere in the body, but it most often occurs in the torso. […] Symptoms of DFSP can vary depending on the size and location of your tumour. You may experience all, some or none of these symptoms before you are diagnosed with DFSP: A slow growing and often painless lump under the skin […] If DFSP is left for several years, it can grow through the top layer of the skin and develop into an ulcer. […] DFSP tends to grow slowly, and very rarely spreads to other parts of the body. But, it is important to get correct treatment as soon as possible. Without the correct treatment, DFSP can grow deep into the fat, muscle and bone, which makes it more difficult to treat. […] DFSP can sometimes come back in the same area where a previous tumour has been removed. This is called a recurrence. If DFSP does reappear, it is important to get treatment as quickly as possible. […] It is very rare for DFSP to spread to another part of the body. But if it does, treatment may involve surgery, targeted therapy, radiotherapy, chemotherapy, or a combination of these treatments. This will be assessed on an individual basis.

• #20 Dermatofibrosarcoma protuberans (DFSP) | Acclaim Dermatology

  https://acclaimdermatology.com/blog/dermatofibrosarcoma-protuberans-dfsp

  As DFSP grows inside the middle layer of skin, it tends to push on the top layer of skin. You may see a lump, also known as a protuberan (pro-to-bur-an). The lump may feel hard or rubbery. As the lump grows, it stretches the skin. You may notice that the affected skin: Becomes tender. Cracks and bleeds. Feels hard, and the lump seems cemented in the skin. […] When a woman is pregnant, DFSP tends to grow more quickly. […] Over time, more protuberans (lumps) can appear. Once these appear, DFSP tends to grow quickly. In adults, the protuberans often range in color from reddish brown to violet. In young patients, DFSP tends to be blue or red in color. […] This skin cancer rarely spreads to other parts of the body, so people often live for many years after treatment. […] 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. […] It is very important to keep all follow-up appointments.

• #21 Dermatofibrosarcoma Protuberans: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/dermatofibrosarcoma-protuberans-causes-symptoms-and-treatment?srsltid=AfmBOoo9U8bQWSoz8KMEVE4wF_dpnPQT5Jzx6OX9FqXajmqepmgvSVdS

  The initial symptoms of DFSP are hard to notice. They usually appear as a small patch of bruised skin, a flat and painless spot which is rough and discoloured. These nodules chiefly appear on the chest, back, shoulders, arms, legs, scalp, abdomen, buttocks and inside of your mouth. […] These nodules become more prominent as the tumour grows in size. The growing tumour then pushes into the epidermis or the top layer of skin and appears on the skin (protuberans). […] Characteristics of the nodules: Easy to bleed or crack open, Reddish-brown to blue, violet, or red, Tender to the touch, Firmly attached to the epidermal layer of the skin, Getting bigger and stretching the surface of the skin, Hard or rubbery surface of the nodule.

• #22 Dermatofibrosarcoma protuberans (DFSP): Foothill Dermatology Medical Center: Dermatology

  https://www.foothillderm.com/blog/dermatofibrosarcoma-protuberans-dfsp

  As DFSP grows inside the middle layer of skin, it tends to push on the top layer of skin. You may see a lump, also known as a protuberan (pro-to-bur-an). The lump may feel hard or rubbery. As the lump grows, it stretches the skin. You may notice that the affected skin: Becomes tender. Cracks and bleeds. Feels hard, and the lump seems cemented in the skin. […] Over time, more protuberans (lumps) can appear. Once these appear, DFSP tends to grow quickly. In adults, the protuberans often range in color from reddish brown to violet. In young patients, DFSP tends to be blue or red in color. […] If you are worried about a growth on your skin, you should see a dermatologist. […] DFSP tends to occur between the ages of 20 and 50. […] This skin cancer rarely spreads to other parts of the body, so people often live for many years after treatment.

• #23 Dermatofibrosarcoma Protuberans Treatment – Virginia Beach Dermatologist

  https://virginiabeachdermatologist.com/skincancer/dermatofibrosarcoma-protuberans/

  Dermatofibrosarcoma Protuberans (DFSP) is a rare type of soft tissue sarcoma that primarily affects the deeper layers of the skin. It is known for its slow-growing nature and often presents as a firm nodule on the skin’s surface. Although DFSP is generally non-metastatic, meaning it doesn’t easily spread to other body parts, it requires medical attention due to its potential to invade surrounding tissues over time. […] The primary indicator of DFSP is the emergence of a raised, firm lump on the skin, which might exhibit shades of reddish, purple, or tan. DFSP can also contribute to skin thinning and breakdown, leading to ulcer formation (open sores). […] Additional symptoms associated with DFSP encompass: Pain, Itching, Burning sensation, Tingling, Numbness. […] Given its gradual growth, DFSP often remains unnoticed for extended periods, potentially spanning months or even years. In its initial stages, DFSP may manifest as: A pimple-like growth or rough skin patch, Minimal to no pain or tenderness at the site of the growth or patch, Minor alterations in the growth or patch’s appearance. […] Yes, DFSP tends to recur even after successful treatment. This emphasizes the importance of regular follow-up appointments with a medical professional to monitor for potential recurrence.

• #24 Patient Education | Peters Dermatology Center | Dermatologist in Bend, OR

  https://www.petersderm.com/articles/aad_education_library/920158-dermatofibrosarcoma-protuberans-dfsp/

  DFSP is a type of skin cancer that begins in the deep layers of the skin. It most commonly occurs on the trunk (chest or back) and the arms, legs, or head. DFSP is usually slow-growing and may not cause any symptoms for years. However, as it grows larger, it can form a raised, firm lump on the skin that may be reddish, purple, or tan. DFSP can also cause the skin to thin and break down, resulting in an ulcer (open sore). […] The most common symptom of DFSP is the development of a raised, firm lump on the skin. This lump may be reddish, purple, or tan. DFSP can also cause the skin to thin and break down, resulting in an ulcer (open sore). […] Other symptoms of DFSP include: Pain, Itching, Burning sensation, Tingling, Numbness. […] DFSP is a type of skin cancer that can be very aggressive and difficult to treat. However, with early diagnosis and treatment, most people with DFSP can expect to have a good outcome.

• #25 Dermatofibrosarcoma Protuberans: Causes, Symptoms, and Treatments

  https://resources.healthgrades.com/right-care/skin-cancer/dermatofibrosarcoma-protuberans

  DFSP is slow-growing cancer, and you may not notice it for months or even years. […] In its early stages, DFSP typically appears as a flesh-colored to a reddish-brown hardened area of skin. […] Eventually, the affected area develops into multiple violet to reddish-brown bumps or growths, which can grow to several centimeters in diameter. […] There is no pain or tenderness at first, but as it grows and stretches the top layer of skin, you may start to notice: tenderness, hard bumps, open sores, bleeding, cracks, pain. […] These changes occur very slowly at first. However, once other patches and bumps appear, the cancer begins to grow more quickly. If it reaches the lowest level of the skin, it may branch out into the tissue like the roots of a tree, making treatment more difficult. […] Although it is a slow-growing cancer, it can spread to fat, muscle, and even bone without diagnosis and treatment. Once it reaches muscle or bone, it becomes much more challenging to treat.

• #26 Dermatofibrosarcoma Protuberans (DFSP) – Dawes Fretzin Dermatology Group

  https://dawesfretzin.com/terminology/dermatofibrosarcoma-protuberans-dfsp/

  Most treatment plans include surgery to remove the cancer. […] When DFSP grows into the lower layer of skin, the cancer often spreads out like the roots of a plant. […] To reduce the risk of DFSP returning after surgery, your dermatologist may include a second treatment. […] After surgery, some patients receive radiation treatments. […] This skin cancer rarely spreads to other parts of the body, so people often live for many years after treatment. […] DFSP can return after treatment. If DFSP returns, it is often treated with one of the surgeries described above.

• #27 Dermatofibrosarcoma protuberans: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/dermatofibrosarcoma-protuberans/

  Rarely, the tumors involved in the different types of dermatofibrosarcoma protuberans can have regions that look similar to fibrosarcoma, a more aggressive type of soft tissue sarcoma. In these cases, the condition is called fibrosarcomatous dermatofibrosarcoma protuberans or FS-DFSP. FS-DFSP tumors are more likely to metastasize than tumors in the other types of dermatofibrosarcoma protuberans.

• #28 Dermatofibrosarcoma Protuberans: Practice Essentials, Background, Pathophysiology

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

  Despite the local invasiveness, DFSP rarely metastasizes. For the classic form of DFSP, the risk is assumed to be only 0.5%. […] A small subset of DFSP patients presents with fibrosarcomatous progression. This fibrosarcomatous progression DFSP variant (FS-DFSP) is more aggressive in nature, and the clinical outcome can be poor. […] The extent of surgical excision determines the prognosis for the patient. To reduce the local recurrence rate, a wide surgical excision with adequate margins or Mohs technique are used. The latter imparts a better outcome. […] Age older than 50 years is also a risk factor associated with a poor clinical outcome.

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

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

  A small percentage of people with DFSP have an aggressive type called fibrosarcomatous dermatofibrosarcoma protuberans (DFSP-FS). These tumors are more likely to spread and come back after surgical removal. […] 20% to 30% or more of people experience a recurrence of DFSP within three years after wide excision, and up to 4% to 5% recur after Mohs surgery. But tumors can recur for 10 years or more.

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

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

  […] The frequencies of local recurrences ranged from 20% to 50%, especially with positive margins. The median time from excision to local recurrence was reported from 32 to 38 months. […] […] 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.

• #31 Dermatofibrosarcoma protuberans, fibrosarcomatous – Wikipedia

  https://en.wikipedia.org/wiki/Dermatofibrosarcoma_protuberans,_fibrosarcomatous

  Painless mass in the dermis […] DFSP-FS tumors are often diagnosed in tumors that have recurred at the sites where DFSP tumors were surgically removed one or more times. In general, they are more rapidly growing and invasive (e.g. rare DFSP-FS dermal tumors in the chest or abdominal skin have respectively invaded the thoracic or abdominal cavities) than DFSP tumors. About 10 to 15% of individuals initially or subsequently present with metastases, most commonly in the lung, less commonly in the regional lymph nodes draining the site of the primary dermal tumor, or, rarely, in the liver, kidney, soft tissue sites outside of the primary dermal tumor’s areas, mediastinum, or brain. […] Various studies find that DFSP-FS tumors have higher rates of recurrence after surgical removal than DFSP tumors and may metastasize (i.e. spread to distant tissues).

• #32 Dermatofibrosarcoma protuberans, fibrosarcomatous – Wikipedia

  https://en.wikipedia.org/wiki/Dermatofibrosarcoma_protuberans,_fibrosarcomatous

  Follow-ups in patients treated for DFSP-FS are aimed at the early detection of local recurrences and metastatic disease. One study recommends that the treatment regimen should include physical examinations and imaging studies (see Diagnosis section) that are routinely conducted following initial therapy every 6 months for 5 years and yearly thereafter. […] Retrospective reviews of patients treated for DFSP-FS find average 5-year recurrence-free survival rates of 42%-52% with a 10%-15% risk of having or developing metastatic disease.

• #33

  https://www.orthobullets.com/pathology/8066/dermatofibrosarcoma-protuberans

  Dermatofibrosarcoma Protuberans are rare, low-grade fibrogenic cutaneous sarcomas that occur in early to mid-adult life. […] Symptoms include usually a slowly enlarging painless mass composed of firm irregular nodules that may have dark red or blue discoloration. […] Local recurrence is common. Distant metastasis is rare (5%), with the lung being the most common site of distant metastasis, occurring via hematogenous spread and usually preceded by multiple local recurrences. […] Worse prognosis is seen with regional lymph node involvement, fibrosarcomatous progression DFSP variant, and histologic features such as a high number of mitotic figures, increased cellularity, DNA aneuploidy, TP53 gene overexpression, and the presence of fibrosarcomatous changes.

• #34 Dermatofibrosarcoma Protuberans – StatPearls – NCBI Bookshelf

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

  As the tumor progresses, it may reach several centimeters in diameter, potentially accompanied by telangiectasia in the surrounding or overlying skin. […] Patients require vigilant monitoring for local recurrence following curative treatment. […] The prognosis for DFSP is generally favorable, with a 10-year survival rate of 99.1%. However, patients with metastatic disease typically survive approximately 2 years post-diagnosis. […] While metastasis remains uncommon, local recurrence poses a more prevalent concern for morbidity. Features that increase the risk of a poor prognosis are patients older than 50, DFSP-FS, a high mitotic index, and increased cellularity. […] Nearly 50% of patients will experience local recurrence with simple excision. Recurrent tumors are more likely to invade fascia, muscle, or bone and result in distant metastases.

• #35 Dermatofibrosarcoma Protuberans: Symptoms and Treatment

  https://www.healthline.com/health/skin-cancer/dermatofibrosarcoma-protuberans

  However, recurrence is common. Almost half of people with DFSP have their cancer return after a simple excision. […] In cases where DFSP is more advanced (spread to other tissues), the outlook can vary. Some patients may require additional treatments, such as radiation or targeted therapy. […] Life expectancy for people with DFSP that has spread is about 2 years after diagnosis. […] DFSP is a serious condition because it is a form of cancer. However, it grows slowly and is usually not life threatening with early treatment. […] However, left untreated, DFSP can damage the surrounding tissues. Life expectancy after DFSP spreads is typically about 2 years. […] DFSP typically does not metastasize, but its possible. When it grows to invade surrounding tissues, complete surgical removal can be challenging. […] In very rare cases, untreated DFSP can spread into distant tissues and organs (metastasis). Metastatic DFSP can be potentially life threatening.

• #36 Dermatofibrosarcoma protuberans (DFSP) | EBSCO Research Starters

  https://www.ebsco.com/research-starters/health-and-medicine/dermatofibrosarcoma-protuberans-dfsp

  DFSP does not generally spread beyond the original tumor site. However, the tumors are very aggressive in moving out underneath healthy skin. The tumor may be much larger under the surface than it appears from the top of the skin. This makes DFSP a difficult cancer to eliminate because the distance the tumor has spread underneath the top layer of skin may make it difficult to remove the entire tumor, and cells that are left behind tend to grow into another tumor. In rare cases, this cancer may spread to lymph nodes or into the bloodstream, where it may spread to the lungs, heart, brain, or bones. […] This type of cancer spreads in only one out of twenty patients, but it has a high rate of recurrence. In 20 to 30 percent of DFSP patients, the cancer returns in the same place. Usually, this is because the first occurrence of the cancer was not completely removed. Because of this high recurrence rate, patients must continue to receive follow-up care. Recurrence usually happens within three years, and patients should be seen regularly during that period to assess any tumor sites to ensure that cancerous cells left behind during surgery have not begun to grow.

• #37 Dermatofibrosarcoma protuberans: A clinical analysis

  https://www.spandidos-publications.com/10.3892/ol.2018.8802

  Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous tumor with a high incidence of misdiagnosis. DFSP has a high rate of recurrence but a low rate of metastasis. The early clinical symptoms of DFSP are non-specific, making diagnosis difficult and leading to a high incidence of misdiagnosis. The main clinical manifestations were pale red or brown irregular indurations, showing slow growth. They appeared either as solitary nodules or as multiple scattered confluent masses. DFSP demonstrated a high recurrence rate, with the degree of malignancy increasing following multiple recurrences. FSDFSP had a higher risk of local recurrence and distant metastasis, and a higher degree of malignancy than classic DFSP. The total postoperative recurrence rate in the present study was 25.7%. The recurrence rate was significantly higher among patients with FS-DFSP compared with those with DFSP. The recurrence rate was significantly higher among the referred cases compared with the newly diagnosed cases.

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

  https://www.mdpi.com/2077-0383/9/6/1752

  In addition to a thorough history and physical examination, diagnosis of suspected DFSP ideally requires a generous biopsy (punch biopsy or excisional biopsy) for a pathologic diagnosis. The pathology report should note the presence of fibrosarcomatous change or other high-risk features. Examination of lymph nodes and imaging studies are important for staging and surgical planning. […] The prognoses of the patients with DFSP, after surgical resection with negative and sometimes even positive microscopic margins, are generally good. The five- and ten-year recurrence-free survival rates of DFSP are 86% and 76%, respectively. Increased age, high mitotic index, positive margins and increased cellularity are predictors of poor clinical outcome. Local recurrence is a major concern after surgical excision. The frequencies of local recurrences ranged from 20% to 50%, especially with positive margins. The median time from excision to local recurrence was reported from 32 to 38 months. Therefore, mandatory long-term surveillance, at an interval of 6 to 12 months, is recommended.

• #39 Dermatofibrosarcoma Protuberans: Symptoms & Causes | Qwark

  https://qwarkhealth.com/conditions/dermatofibrosarcoma-protuberans

  Dermatofibrosarcoma Protuberans develops slowly over time, usually starting as a small lump on the skin that gradually grows in size. As the abnormal cells multiply and invade surrounding tissue, the lump may become more visible or prominent, and may begin to cause other symptoms. […] The typical survival rate for DFSP patients varies depending on several factors such as tumor size, location, and invasion depth. However, studies have reported a 5-year survival rate of approximately 96-100%, indicating a high survival rate among patients diagnosed with DFSP. […] The likelihood of DFSP recurrence after initial treatment varies depending on several factors, such as the adequacy of surgical excision, the presence of positive margins, and the histologic subtype. Studies have reported recurrence rates ranging from 1% to 30%, with most recurrences occurring within the first 5 years after initial treatment.

• #40 Dermatofibrosarcoma Protuberans – StatPearls – NCBI Bookshelf

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

  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. […] Radiation therapy can decrease local recurrence, particularly in cases where wide margins are impractical. […] 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.

• #41 Dermatofibrosarcoma protuberans (DFSP): Foothill Dermatology Medical Center: Dermatology

  https://www.foothillderm.com/blog/dermatofibrosarcoma-protuberans-dfsp

  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. […] It is very important to keep all follow-up appointments.

• #42 Dermatofibrosarcoma protuberans (DFSP) – BAD Patient Hub

  https://www.skinhealthinfo.org.uk/condition/dermatofibrosarcoma-protuberans-dfsp/

  DFSP is a very rare type of soft tissue cancer. It usually occurs on the trunk, often the chest and shoulders; however, it can also affect the limbs, head and neck and rarely the genitalia. […] It can often be completely removed surgically by including a margin of normal tissue, or with a specialized form of surgery called Mohs surgery. […] DFSP is usually a slow growing, painless, thickened lumpy area of skin. It can be skin coloured, pink/red, brown/black or occasionally blue/purple. […] Most people with DFSP do not have any symptoms. They may notice thick skin, skin changing colour, a sunken area or a lump increasing in size. […] DFSP can be difficult even for doctors to detect. After having had a DFSP, you should do regular checks of your scar at home; if you notice any changes in the scar, such as new lumps which do not settle within a week or two, you should contact your primary care physician, dermatologist or department leading your care.

Zobacz więcej