Materiały źródłowe

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

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

  Dermatofibrosarcoma protuberans (DFSP) is a rare type of skin cancer. It starts in connective tissue cells in the middle layer of the skin (dermis). […] The cause of dermatofibrosarcoma protuberans often isn’t known. […] This skin cancer happens when cells in the skin develop changes. The changes happen in the cells’ chromosomes. Chromosomes are threadlike structures inside cells that contain DNA and proteins. The changes in the chromosomes cause the cells to make many more cells quickly. The cancer cells form a growth that starts underneath the skin and may push up to create a lump over time. Other lumps may appear as well.

• #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. […] The chromosomal translocation t(17;22)(q22;q13) leads to the formation of the abnormal fusion protein COL1A1-PDGFB, contributing to its pathogenesis. […] Ninety percent of patients with DFSP, including those who develop the fibrosarcomatous variant (DFSP-FS), have the t(17;22)(q22;q13) translocation. […] An alternative translocation involving the platelet-derived growth factor subunit B (PDGFB) gene on chromosome 22 may be present in patients without the t(17;22)(q22;q13) translocation. […] In certain instances, the progression from DFSP to DFSP-FS is associated with genomic gain affecting the PDGFB/COL1A1 fusion gene, although this occurrence is variable.

• #3 Dermatofibrosarcoma protuberans – Dermatology Advisor

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

  DFSP is a rare tumor, with an estimated incidence of 4.1 cases per million person-years, but still the most common sarcoma of the skin. […] The etiology of DFSP is unknown. Recent advances in genetic research have implicated a specific chromosomal translocation in the pathogenesis of DFSP arising between chromosome regions 17q22 and 22q13. This t17:22 translocation is present in 90% of all DFSPs and represents a fusion product of the type I collagen (COL1A1) promoter region and the platelet-derived growth factor-beta (PDGF) receptor. The fusion product results in a ringed chromosome, which results in continuous activation of the PDGF signal transduction pathway.

• #4 Dermatofibrosarcoma protuberans (DFSP) | Acclaim Dermatology

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

  People of all ages get this rare skin cancer. Most people are diagnosed when they are between 20 and 50 years of age. […] Scientists do not yet know what causes DFSP. […] In treating patients with DFSP, dermatologists have learned that this cancer sometimes begins on skin that was badly injured. The injured skin may have a scar from a burn or surgery. Sometimes, DFSP forms where a person received many radiation treatments or vaccines. […] More research is needed to know whether a skin injury plays a role in causing DFSP.

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

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

  People of all ages get this rare skin cancer. Most people are diagnosed when they are between 20 and 50 years of age. […] Although unusual, children get DFSP. Sometimes, a child is born with DFSP. In newborns, this skin cancer often looks like a birthmark. […] Scientists do not yet know what causes DFSP. […] In treating patients with DFSP, dermatologists have learned that this cancer sometimes begins on skin that was badly injured. The injured skin may have a scar from a burn or surgery. Sometimes, DFSP forms where a person received many radiation treatments or vaccines. […] More research is needed to know whether a skin injury plays a role in causing DFSP.

• #6 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. […] The chromosomal translocation t(17;22)(q22;q13) leads to the formation of the abnormal fusion protein COL1A1-PDGFB, contributing to its pathogenesis. […] Ninety percent of patients with DFSP, including those who develop the fibrosarcomatous variant (DFSP-FS), have the t(17;22)(q22;q13) translocation. […] An alternative translocation involving the platelet-derived growth factor subunit B (PDGFB) gene on chromosome 22 may be present in patients without the t(17;22)(q22;q13) translocation. […] In certain instances, the progression from DFSP to DFSP-FS is associated with genomic gain affecting the PDGFB/COL1A1 fusion gene, although this occurrence is variable.

• #7 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Dermatofibrosarcoma-Protuberans-(DFSP)-Causes-and-Risk-Factors.aspx

  Dermatofibrosarcoma protuberans (DFSP) is now known to be associated in almost 90% of patients with a translocation of chromosomes 17 and 22 t(17;22). This is recirpocral abnormality is not inherited, but occurs in tumor cells, after birth. […] The reciprocal translocation of genetic material between the chromosomes 17 and 22, involves the genes COL1A1 and PDGFB. This translocation results in the formation of an extra or supernumerary chromosome. […] The fusion of these genes causes an abnormal proliferation of cells from the affected or mutated cell. This is because the fused gene gives rise to excessive amounts of an abnormal fusion protein which, just like PDGF, acts as a growth and proliferation promoter. This in turn leads to tumor formation. […] It is currently thought that DFSP is the result of severe trauma to the skin cells in some cases. For instance, some tumors began on skin that was badly burned or injured, and others on irradiated skin or skin that showed vaccination scars. The nature of this risk is not yet clear.

• #8 Dermatofibrosarcoma Protuberans | Encyclopedia MDPI

  https://encyclopedia.pub/entry/5315

  Dermatofibrosarcoma protuberans is associated with a rearrangement (translocation) of genetic material between chromosome 17 and chromosome 22. This translocation, written as t(17;22), fuses part of the COL1A1 gene from chromosome 17 with part of the PDGFB gene from chromosome 22. […] The translocation is acquired during a person’s lifetime and the chromosomes containing the translocation are present only in the tumor cells. This type of genetic change is called a somatic mutation. […] The abnormally fused COL1A1-PDGFB gene provides instructions for making an abnormal combined (fusion) protein that researchers believe ultimately functions like the PDGFB protein. The gene fusion leads to the production of an excessive amount of protein that functions like the PDGFB protein. In excess, this fusion protein stimulates cells to proliferate and differentiate abnormally, leading to the tumor formation seen in dermatofibrosarcoma protuberans. […] The COL1A1-PDGFB fusion gene is found in more than 90 percent of dermatofibrosarcoma protuberans cases. In the remaining cases, changes in other genes may be associated with this condition. These genes have not been identified.

• #9 Dermatofibrosarcoma Protuberans | Encyclopedia MDPI

  https://encyclopedia.pub/entry/5315

  Dermatofibrosarcoma protuberans is associated with a rearrangement (translocation) of genetic material between chromosome 17 and chromosome 22. This translocation, written as t(17;22), fuses part of the COL1A1 gene from chromosome 17 with part of the PDGFB gene from chromosome 22. […] The translocation is acquired during a person’s lifetime and the chromosomes containing the translocation are present only in the tumor cells. This type of genetic change is called a somatic mutation. […] The abnormally fused COL1A1-PDGFB gene provides instructions for making an abnormal combined (fusion) protein that researchers believe ultimately functions like the PDGFB protein. The gene fusion leads to the production of an excessive amount of protein that functions like the PDGFB protein. In excess, this fusion protein stimulates cells to proliferate and differentiate abnormally, leading to the tumor formation seen in dermatofibrosarcoma protuberans. […] The COL1A1-PDGFB fusion gene is found in more than 90 percent of dermatofibrosarcoma protuberans cases. In the remaining cases, changes in other genes may be associated with this condition. These genes have not been identified.

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

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

  As many as 9 in 10 people who develop DFSP have a gene change (mutation) that causes the condition. This gene change occurs in cells after you’re born. You don’t inherit a gene mutation from a parent that causes DFSP. […] In addition to racial factors, a skin injury or scars may increase your risk of DFSP. Causes of skin injuries include: Burns, Radiation therapy, Surgical incisions, Tattoos.

• #11 Orphanet: Dermatofibrosarcoma protuberans

  https://www.orpha.net/en/disease/detail/31112

  Dermatofibrosarcoma protuberans (DFSP) is a rare infiltrating soft tissue sarcoma, generally of low grade malignancy, arising from the dermis of the skin and characteristically associated with a specific chromosomal translocation t(17;22). […] DFSP is most likely fibroblastic in origin: over 90% of cases are associated with dysregulated platelet-derived growth factor (PDGF) production resulting from chromosomal translocation or a supernumerary ring chromosome derived from t(17;22). The translocation breakpoint most often involves the second exon of the PDGFB gene on chromosome 22 (22q13.1), with fusion to the collagen, type I, alpha 1 gene (COL1A1) on chromosome 17 (17q21.33). This chromosomal translocation results in the upregulation of the PDGFB gene in the form of a fused proto-oncogene COL1A1/PDGFB.

• #12 Dermatofibrosarcoma Protuberans | Encyclopedia MDPI

  https://encyclopedia.pub/entry/5315

  Dermatofibrosarcoma protuberans is associated with a rearrangement (translocation) of genetic material between chromosome 17 and chromosome 22. This translocation, written as t(17;22), fuses part of the COL1A1 gene from chromosome 17 with part of the PDGFB gene from chromosome 22. […] The translocation is acquired during a person’s lifetime and the chromosomes containing the translocation are present only in the tumor cells. This type of genetic change is called a somatic mutation. […] The abnormally fused COL1A1-PDGFB gene provides instructions for making an abnormal combined (fusion) protein that researchers believe ultimately functions like the PDGFB protein. The gene fusion leads to the production of an excessive amount of protein that functions like the PDGFB protein. In excess, this fusion protein stimulates cells to proliferate and differentiate abnormally, leading to the tumor formation seen in dermatofibrosarcoma protuberans. […] The COL1A1-PDGFB fusion gene is found in more than 90 percent of dermatofibrosarcoma protuberans cases. In the remaining cases, changes in other genes may be associated with this condition. These genes have not been identified.

• #13 Azthena logo with the word Azthena

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

  The risk factors for this tumor are yet to be identified, but some cases have been noted to arise in skin that was previously burned or suffered severe trauma. […] The underlying etiology of DSFP appears to be genetic, with the translocation t17;22 being found in 90% of all patients affected. This mutation is commonly found as a supernumerary ring chromosome carrying parts of the genes COL1A1 and PDGFB, from chromosome 17 and 22 respectively, in fused form. […] While COL1A1 is responsible for the production of collagen type 1, providing support and structural strength to many tissues, the other is a powerful growth factor that enables many cells to grow, proliferate and differentiate. The fused gene results in the production of large amounts of PDGFB, which promotes the proliferation of the mutated clone of cells to form a DFSP tumor.

• #14 Dermatofibrosarcoma protuberans (overview) – Altmeyers Encyclopedia – Department Dermatology

  https://www.altmeyers.org/en/dermatology/dermatofibrosarcoma-protuberans-overview-119036

  The etiology is unclear in detail. Chromosomal translocation mutations („ring chromosomes”) resulting from a fusion of chromosomal regions 17q22 and 22q13 have been described. […] The now fused COL1A1-PDGF gene is outsourced from the other genes as a „ring chromosome”. As a consequence, excessive amounts of functional growth factor PDGF-beta are produced under the influence of transcription factors, driving the tumor to grow autocrine via PDGF receptor stimulation. This dysfunction is highly characteristic for DFSP (detection in 90%) and at the same time key for molecular targeted therapy with imatinib (Gleevec) type tyrosine kinase inhibitors. […] The rare fibrosarcomatous dermatofibrosarcoma protuberans has a greater tendency to metastasis than the non-fibrosarcomatous DFSP. Hence the larger safety margin of 3.0-5.0 cm.

• #15 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. […] The chromosomal translocation t(17;22)(q22;q13) leads to the formation of the abnormal fusion protein COL1A1-PDGFB, contributing to its pathogenesis. […] Ninety percent of patients with DFSP, including those who develop the fibrosarcomatous variant (DFSP-FS), have the t(17;22)(q22;q13) translocation. […] An alternative translocation involving the platelet-derived growth factor subunit B (PDGFB) gene on chromosome 22 may be present in patients without the t(17;22)(q22;q13) translocation. […] In certain instances, the progression from DFSP to DFSP-FS is associated with genomic gain affecting the PDGFB/COL1A1 fusion gene, although this occurrence is variable.

• #16 Dermatofibrosarcoma Protuberans | Encyclopedia MDPI

  https://encyclopedia.pub/entry/5315

  Dermatofibrosarcoma protuberans is associated with a rearrangement (translocation) of genetic material between chromosome 17 and chromosome 22. This translocation, written as t(17;22), fuses part of the COL1A1 gene from chromosome 17 with part of the PDGFB gene from chromosome 22. […] The translocation is acquired during a person’s lifetime and the chromosomes containing the translocation are present only in the tumor cells. This type of genetic change is called a somatic mutation. […] The abnormally fused COL1A1-PDGFB gene provides instructions for making an abnormal combined (fusion) protein that researchers believe ultimately functions like the PDGFB protein. The gene fusion leads to the production of an excessive amount of protein that functions like the PDGFB protein. In excess, this fusion protein stimulates cells to proliferate and differentiate abnormally, leading to the tumor formation seen in dermatofibrosarcoma protuberans. […] The COL1A1-PDGFB fusion gene is found in more than 90 percent of dermatofibrosarcoma protuberans cases. In the remaining cases, changes in other genes may be associated with this condition. These genes have not been identified.

• #17 Dermatofibrosarcoma protuberans, fibrosarcomatous – Wikipedia

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

  DFSP-FS tumors are related to DFSP. […] The tumor cells in DFSP and DFSP-FS harbor one or more fusion gene mutations, i.e. mutations that merge two previously independent genes. The COL1A1-PDGFB fusion gene is the most common fusion gene found in both tumor types. […] While the COL1A1-PDGFB fusion gene is present in 90% of DFSP-FS and DFSP cases, recent studies have also found the COL6A3-PDGFD and EMILIN2-PDGFD fusion genes in 2% of DFSP-FS or DFSP cases, the TNC-PDGFD fusion gene in one case of DFSP-FS, the COL1A2-PDGFB fusion gene in one case of DFSP, and the CSPG2-PTK2B fusion gene in one case of DFSP-FS. […] These events are considered to underlie the development and/or progression of DFSP and DFSP-FS tumors.

• #18 Dermatofibrosarcoma Protuberans – StatPearls – NCBI Bookshelf

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

  Furthermore, researchers have noted the occurrence of microsatellite instability, characterized by alterations in the number of repeated deoxyribonucleic acid (DNA) bases within microsatellites or short, repetitive DNA sequences compared to the inherited state. […] Subsequently, these instances of microsatellite instability are accompanied by the acquisition of tumor protein p53 (TP53) mutations, particularly in cases displaying features of high-grade sarcoma.

• #19 Dermatofibrosarcoma Protuberans – MD Searchlight

  https://mdsearchlight.com/cancer/dermatofibrosarcoma-protuberans/

  Moreover, scientists have observed something known as microsatellite instability in some cases. This is characterized by changes in the number of times certain small sequences of DNA are repeated compared to what is typically inherited. After these changes, commonly the cancer acquires mutations in a protein called TP53, especially in instances where the cancer features characteristics of a highly aggressive type of cancer called high-grade sarcoma.

• #20 Dermatofibrosarcoma Protuberans – MD Searchlight

  https://mdsearchlight.com/cancer/dermatofibrosarcoma-protuberans/

  Dermatofibrosarcoma protuberans, or DFSP, is a rare type of skin cancer usually found on the torso and upper limbs. […] Most instances of DFSP are connected with a particular genetic shift which generates a specific protein called COL1A1-PDGFB. […] Ninety percent of patients with a type of skin cancer known as DFSP, as well as those who develop a variant of it called DFSP-FS, usually show a specific change in their genes, or a translocation. This involves a swap between two specific regions of DNA on chromosomes 17 and 22. […] But in some patients, this typical translocation isn’t present. Instead, these patients might show a different translocation involving a gene known as PDGFB on chromosome 22. […] In certain cases, the transformation from standard DFSP to the DFSP-FS variant is related to an increase in the activity of the PDGFB/COL1A1 fusion gene, although this doesn’t happen all the time.

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

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

  Ninety percent of patients with DFSP, including those who develop the fibrosarcomatous variant (DFSP-FS), have the t(17;22)(q22;q13) translocation. […] An alternative translocation involving the platelet-derived growth factor subunit B (PDGFB) gene on chromosome 22 may be present in patients without the t(17;22)(q22;q13) translocation. […] In certain instances, the progression from DFSP to DFSP-FS is associated with genomic gain affecting the PDGFB/COL1A1 fusion gene, although this occurrence is variable. […] Furthermore, researchers have noted the occurrence of microsatellite instability, characterized by alterations in the number of repeated deoxyribonucleic acid (DNA) bases within microsatellites or short, repetitive DNA sequences compared to the inherited state. […] Subsequently, these instances of microsatellite instability are accompanied by the acquisition of tumor protein p53 (TP53) mutations, particularly in cases displaying features of high-grade sarcoma.

• #22 Dermatofibrosarcoma protuberans: Epidemiology, pathogenesis, clinical presentation, diagnosis, and staging – UpToDate

  https://www.uptodate.com/contents/dermatofibrosarcoma-protuberans-epidemiology-pathogenesis-clinical-presentation-diagnosis-and-staging

  Dermatofibrosarcoma protuberans (DFSP) is an uncommon locally aggressive cutaneous soft tissue sarcoma. Approximately 85 to 90 percent of DFSPs are low grade, while the remainder contain a high-grade sarcomatous component (which is usually a fibrosarcoma, designated DFSP-FS) and are considered to be intermediate-grade sarcomas. […] The fibrosarcomatous variant of DFSP (DFSP-FS) accounts for approximately 5 to 15 percent of DFSPs. […] The so-called giant cell fibroblastoma is considered to be the juvenile form of DFSP, given its similar morphology, identical chromosomal translocation, and CD34 positivity.

• #23 Dermatofibrosarcoma Protuberans: An Updated Review of the Literature

  https://www.mdpi.com/2072-6694/16/18/3124

  Dermatofibrosarcoma protuberans (DFSP) is a rare proliferative condition representing skin sarcomas which is known to locally recur yet very rarely metastasizes. Its genetic background is a reciprocal translocation t(17;22)(q22;q13) that produces COL1A1-PDGFB gene fusion. […] Malignant transformation within DFSP (or high histologic grade), older age, being female, large primary tumor size (≥10 cm), narrow surgical margins of excision (<3 cm), surgical margin positivity for tumor cells, short time to recurrence, numerous recurrences, tumor that was recently rapidly enlarging, and presence of pain in the tumor have all been proposed as clinicopathological risk factors for recurrence and metastasis. [...] Approximately 85–90% of all DFSPs are low-grade tumors, whereas the remaining 10–15% contain a high-grade tumor component. This transformation, when presenting in more than 5% of the tumor’s volume, leads to a higher incidence of local relapse and distant metastasis.

• #24 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. […] Laboratory studies have shown that chromosomal aberrations may contribute to the pathogenesis of DFSP; however, no evidence of hereditary or familial predisposition exists. In 10-20% of patients with this tumor, trauma at the site seems to be incriminated. Surgical and burn scars and sites of vaccinations have been reported as sites of DFSP. […] The cause of dermatofibrosarcoma protuberans (DFSP) is unknown.

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

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

  There are no definitive causes identified for DFSP, although a small percentage of cases have been linked to prior skin trauma. […] In a minority of patients, about 10 percent, some type of trauma, such as a burn, scar, or injection site, has occurred at the site where the disease develops. […] Laboratory studies indicate that a type of chromosome mutation tends to help the previous skin damage evolve into this type of cancer. […] However, many people with this chromosome mutation do not develop DFSP, and many who have DFSP do not have this chromosome mutation.

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

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

  As many as 9 in 10 people who develop DFSP have a gene change (mutation) that causes the condition. This gene change occurs in cells after you’re born. You don’t inherit a gene mutation from a parent that causes DFSP. […] In addition to racial factors, a skin injury or scars may increase your risk of DFSP. Causes of skin injuries include: Burns, Radiation therapy, Surgical incisions, Tattoos.

• #27 Dermatofibrosarcoma Protuberans: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/dermatofibrosarcoma-protuberans-causes-symptoms-and-treatment?srsltid=AfmBOoqwhWMPInoT_ks9wIi9ugbWPKPBSHDD1twsqLZMUEhhZxhdO1id

  Dermatofibrosarcoma protuberans (DFSP) is a rare type of soft tissue sarcoma of the skin that is characterised by the formation of malignant tumours in the connective tissue cells of the dermis (the skins middle layer). […] Just like any other form of soft tissue sarcoma, the absolute cause of DFSP is yet unknown but some clinical trials and scientific data indicate that this condition might occur due to a rearrangement or translocation of genetic material between chromosome 17 and chromosome 22. This rearrangement. This somatic mutation triggers the healthy cells to proliferate and differentiate abnormally, leading to a huge mass of irregular cells which combine to form tumours as seen in dermatofibrosarcoma protuberans. […] Certain causative factors that aggravate the chances of getting dermatofibrosarcoma protuberans include: Age: Although DFSp is usually noticed in adults between 20 – 50, even children and teens have some form of DFSP. Race: The risk of DFSP is more in people of Black origin than in other ethnicities. Skin Conditions: People having any skin injury, scars, burns or tattoos have a higher incidence of getting DFSP. Medical Treatments: People who underwent radiation therapy or any surgical incision have an aggravated risk of this type of cancer.

• #28 Dermatofibrosarcoma Protuberans FAQs Houston TX – DFSP GA

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

  Dermatofibrosarcoma protuberans does involve a mutation of the genetic material between chromosomes 17 and 22, but it is not a hereditary condition. […] The causes of this rare form of skin cancer are unknown. There is some thought that dermatofibrosarcoma protuberans can begin on the skin that was badly injured from a burn or from surgery. There is not a link between sun exposure and this rare skin cancer. […] It is not clear what causes this type of skin cancer. As mentioned above, its thought the scarring that develops after a burn or surgery may increase a persons risk for developing dermatofibrosarcoma protuberans (DFSP). But its impossible for a person to prevent scarring after a burn or surgical incision. Women and African Americans are at a higher risk, but again there isnt any prevention protocol, such as applying sunscreen to ward off other skin cancers. DFSP is not caused by sun exposure.

• #29 Dermatofibrosarcoma Protuberans (DFSP) – DoveMed

  https://www.dovemed.com/diseases-conditions/dermatofibrosarcoma-protuberans-dfsp

  Dermatofibrosarcoma Protuberans is a rare tumor of the subcutaneous tissue of skin. […] Currently, no definitive risk factors have been reported for the development of Dermatofibrosarcoma Protuberans. However, the tumor has been linked to the following: Site of trauma, Region of extensive burns, Surgical incision sites, Vaccinations sites (BCG vaccination), Arsenic poisoning. […] The exact cause of development of Dermatofibroma Protuberans is unknown. Occasionally, certain genetic mutations have been reported; but these are still being researched.

• #30 Dermatofibrosarcoma protuberans (DFSP) | Acclaim Dermatology

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

  People of all ages get this rare skin cancer. Most people are diagnosed when they are between 20 and 50 years of age. […] Scientists do not yet know what causes DFSP. […] In treating patients with DFSP, dermatologists have learned that this cancer sometimes begins on skin that was badly injured. The injured skin may have a scar from a burn or surgery. Sometimes, DFSP forms where a person received many radiation treatments or vaccines. […] More research is needed to know whether a skin injury plays a role in causing DFSP.

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

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

  As many as 9 in 10 people who develop DFSP have a gene change (mutation) that causes the condition. This gene change occurs in cells after you’re born. You don’t inherit a gene mutation from a parent that causes DFSP. […] In addition to racial factors, a skin injury or scars may increase your risk of DFSP. Causes of skin injuries include: Burns, Radiation therapy, Surgical incisions, Tattoos.

• #32 Dermatofibrosarcoma protuberans of the breast: A case study

  https://www.spandidos-publications.com/10.3892/mco.2021.2212

  Dermatofibrosarcoma protuberans (DFSP) is a superficial mesenchymal neoplasm that originates from the dermal fibroblasts and tends to be locally aggressive. […] The etiology is not completely known. A history of previous trauma has been suggested as predisposing factor in approximately 10-20% of the cases. The reported cases included lesions in surgical scars, burns, tattoo skin regions and even bacillus Calmette-Guerin vaccination scar. […] the vast majority of cases arise from the rearrangement of chromosomes 17 and 22, creating a supernumerary ring chromosome composed of hybrid material derived from t (17;22). This translocation leads to a continuous activation of platelet derived growth factor receptor -protein tyrosine kinase due to a fusion of PDGFB gene with collagen Type Ia1 gene (COL1A1).

• #33 Dermatofibrosarcoma Protuberans (DFSP) – DoveMed

  https://www.dovemed.com/diseases-conditions/dermatofibrosarcoma-protuberans-dfsp

  Dermatofibrosarcoma Protuberans is a rare tumor of the subcutaneous tissue of skin. […] Currently, no definitive risk factors have been reported for the development of Dermatofibrosarcoma Protuberans. However, the tumor has been linked to the following: Site of trauma, Region of extensive burns, Surgical incision sites, Vaccinations sites (BCG vaccination), Arsenic poisoning. […] The exact cause of development of Dermatofibroma Protuberans is unknown. Occasionally, certain genetic mutations have been reported; but these are still being researched.

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

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

  As many as 9 in 10 people who develop DFSP have a gene change (mutation) that causes the condition. This gene change occurs in cells after you’re born. You don’t inherit a gene mutation from a parent that causes DFSP. […] In addition to racial factors, a skin injury or scars may increase your risk of DFSP. Causes of skin injuries include: Burns, Radiation therapy, Surgical incisions, Tattoos.

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

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

  Dermatofibrosarcoma protuberans (DFSP), also known as giant cell fibroblastoma, is a type of skin cancer that begins in the middle layer of the skin. In rare cases, it can spread to the muscles, connective tissues, or even to the bones. […] Although experts are unsure about what exactly causes DFSP, they have observed some associated conditions: […] It sometimes develops on badly injured skin or scar tissue. Burn scars, surgery scars, skin damaged by radiation treatments, and tattoos may be among the affected areas. […] Studies have also shown that a chromosomal translocation, or an unusual arrangement of chromosomes, could link to a DFSP occurrence.

• #36 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

  Notably, 6 out of 19 patients recounted tumor growth on cutis that previously had been exposed to trauma, such as chronic irritation, laceration, or burns. […] Interestingly, there are several case reports that describe DFSP arising from a preexisting trauma to the skin, such as vaccination, tattoos, radiation, or burns. […] We suggest to abstain from citing the trunk as the most common tumor localization of DFSP. Instead, we propose to describe the tumor as a ubiquitous soft tissue sarcoma with a tendency to grow in skin that has been exposed to prior trauma. […] Patients diagnosed with DFSP who underwent a complete excision have an excellent prognosis with a 5-year-survival rate of 99%. […] The follow-up should be continued well beyond 5 years since recurrences after this period are not uncommon in DFSP.

• #37 Dermatofibrosarcoma protuberans associated with trauma: A case report

  https://www.spandidos-publications.com/10.3892/mco.2020.2121

  A case of dermatofibrosarcoma protuberans (DFSP) in a 22year old female associated with blunt trauma of the midupper back is presented in the current study with a review of the literature. […] The literature search revealed that both noncongenital mutation as well as trauma serve a role in the development of this dermal neoplasm, but the exact mechanism by which trauma may predispose to development of DFSP is unknown. […] Based on the literature search and our current findings, the authors conclude that both non congenital mutation as well as trauma play a role in the development of this dermal neoplasm. Future research into the relationship between trauma and DFSP on a cellular level is suggested, as there seems to be direct link.

• #38 Dermatofibrosarcoma Protuberans: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/dermatofibrosarcoma-protuberans-causes-symptoms-and-treatment?srsltid=AfmBOoqwhWMPInoT_ks9wIi9ugbWPKPBSHDD1twsqLZMUEhhZxhdO1id

  Dermatofibrosarcoma protuberans (DFSP) is a rare type of soft tissue sarcoma of the skin that is characterised by the formation of malignant tumours in the connective tissue cells of the dermis (the skins middle layer). […] Just like any other form of soft tissue sarcoma, the absolute cause of DFSP is yet unknown but some clinical trials and scientific data indicate that this condition might occur due to a rearrangement or translocation of genetic material between chromosome 17 and chromosome 22. This rearrangement. This somatic mutation triggers the healthy cells to proliferate and differentiate abnormally, leading to a huge mass of irregular cells which combine to form tumours as seen in dermatofibrosarcoma protuberans. […] Certain causative factors that aggravate the chances of getting dermatofibrosarcoma protuberans include: Age: Although DFSp is usually noticed in adults between 20 – 50, even children and teens have some form of DFSP. Race: The risk of DFSP is more in people of Black origin than in other ethnicities. Skin Conditions: People having any skin injury, scars, burns or tattoos have a higher incidence of getting DFSP. Medical Treatments: People who underwent radiation therapy or any surgical incision have an aggravated risk of this type of cancer.

• #39 Dermatofibrosarcoma Protuberans – Cancer Diagnosis

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

  It is unknown what causes this particular type of skin cancer. However, a postoperative scar or a scar from a burn can increase the risk for DFSP. […] In addition, women and blacks are more likely to develop DFSP.

• #40 Dermatofibrosarcoma Protuberans – Cancer Diagnosis

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

  It is unknown what causes this particular type of skin cancer. However, a postoperative scar or a scar from a burn can increase the risk for DFSP. […] In addition, women and blacks are more likely to develop DFSP.

• #41 Dermatofibrosarcoma Protuberans | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/en-dermatofibrosarcoma-protuberans-articulo-S1578219012002740

  Dermatofibrosarcoma protuberans is the most common skin sarcoma, although its incidence is very low compared with other skin tumors. […] As for most sarcomas, DFSP does not have a well-established risk factor and its etiology is unknown. In 1951, Pack and Tabah suggested that local trauma in the region of the tumor was an etiologic factor on the basis that such an event was reported by 13% of the patients in their series. […] Several cases of DFSP have been reported in women in whom tumor growth starts or accelerates during pregnancy. […] What does seem clear is that tumor onset is not related to sun exposure as no studies report such an association.

• #42 Dermatofibrosarcoma Protuberans | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/en-dermatofibrosarcoma-protuberans-articulo-S1578219012002740

  Dermatofibrosarcoma protuberans is the most common skin sarcoma, although its incidence is very low compared with other skin tumors. […] As for most sarcomas, DFSP does not have a well-established risk factor and its etiology is unknown. In 1951, Pack and Tabah suggested that local trauma in the region of the tumor was an etiologic factor on the basis that such an event was reported by 13% of the patients in their series. […] Several cases of DFSP have been reported in women in whom tumor growth starts or accelerates during pregnancy. […] What does seem clear is that tumor onset is not related to sun exposure as no studies report such an association.

• #43 Dermatofibrosarcoma Protuberans FAQs Houston TX – DFSP GA

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

  Dermatofibrosarcoma protuberans does involve a mutation of the genetic material between chromosomes 17 and 22, but it is not a hereditary condition. […] The causes of this rare form of skin cancer are unknown. There is some thought that dermatofibrosarcoma protuberans can begin on the skin that was badly injured from a burn or from surgery. There is not a link between sun exposure and this rare skin cancer. […] It is not clear what causes this type of skin cancer. As mentioned above, its thought the scarring that develops after a burn or surgery may increase a persons risk for developing dermatofibrosarcoma protuberans (DFSP). But its impossible for a person to prevent scarring after a burn or surgical incision. Women and African Americans are at a higher risk, but again there isnt any prevention protocol, such as applying sunscreen to ward off other skin cancers. DFSP is not caused by sun exposure.

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

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

  DFSP is associated with a random rearrangement of genetic material between two chromosomes (17 and 22). This translocation results in continuous growth of cells responsible for the cancer. […] Unlike other cancers which are causes by the sun, DFSP has shown no such association.

• #45 Dermatofibrosarcoma Protuberans | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/en-dermatofibrosarcoma-protuberans-articulo-S1578219012002740

  Dermatofibrosarcoma protuberans is the most common skin sarcoma, although its incidence is very low compared with other skin tumors. […] As for most sarcomas, DFSP does not have a well-established risk factor and its etiology is unknown. In 1951, Pack and Tabah suggested that local trauma in the region of the tumor was an etiologic factor on the basis that such an event was reported by 13% of the patients in their series. […] Several cases of DFSP have been reported in women in whom tumor growth starts or accelerates during pregnancy. […] What does seem clear is that tumor onset is not related to sun exposure as no studies report such an association.

• #46 Dermatofibrosarcoma Protuberans – SFA

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

  Dermatofibrosarcoma protuberans (DFSP) is a superficial, locally aggressive fibroblastic neoplasm, having a cellular storiform appearance and carrying a COL1A1-PDGFB or related fusion. […] Most of these tumors occur sporadically. DFSP with unique features, such as multicentricity, small size, and occurrence at early age, has been shown in children affected with adenosine deaminase–deficient severe combined immunodeficiency.

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

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

  Dermatofibrosarcoma protuberans (DFSP) is a slow growing, low- to intermediate-grade dermal soft-tissue tumor. […] DFSP has characteristic t(17; 22) (q22; q13), resulting in a COL1A1- PDGFB fusion transcripts in more than 90% of DFSPs. […] Cytogenetic and molecular studies have demonstrated that more than 90% of DFSPs are characterized by either supernumerary ring chromosomes derived from chromosomes 17 and 22 or chromosomal translocation t(17; 22) (q22; q13), resulting in the fusion of collagen type 1-alpha 1(COL1A1 at 17q22) and platelet-derived growth factor beta (PDGFB at 22q13) genes. […] These data suggest that multiple factors including oncogenes, tumor suppressor genes and immunodeficiency are involved in the development of DFSP.

• #48 Dermatofibrosarcoma Protuberans: Symptoms and Treatment

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

  Dermatofibrosarcoma protuberans (DFSP) is a rare type of cancer that begins in the middle layer of your skin called the dermis. […] The exact cause of DFSP is not well understood, but researchers believe its linked to genetic changes in the skin cells. […] One common genetic change associated with DFSP is a translocation (breaking and re-attachment of a piece of a chromosome to a different chromosome) between chromosomes 17 and 22. This change leads to the production of a protein that triggers the growth of cancer cells.

• #49 Dermatofibrosarcoma Protuberans: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/dermatofibrosarcoma-protuberans-causes-symptoms-and-treatment?srsltid=AfmBOoqwhWMPInoT_ks9wIi9ugbWPKPBSHDD1twsqLZMUEhhZxhdO1id

  Dermatofibrosarcoma protuberans (DFSP) is a rare type of soft tissue sarcoma of the skin that is characterised by the formation of malignant tumours in the connective tissue cells of the dermis (the skins middle layer). […] Just like any other form of soft tissue sarcoma, the absolute cause of DFSP is yet unknown but some clinical trials and scientific data indicate that this condition might occur due to a rearrangement or translocation of genetic material between chromosome 17 and chromosome 22. This rearrangement. This somatic mutation triggers the healthy cells to proliferate and differentiate abnormally, leading to a huge mass of irregular cells which combine to form tumours as seen in dermatofibrosarcoma protuberans. […] Certain causative factors that aggravate the chances of getting dermatofibrosarcoma protuberans include: Age: Although DFSp is usually noticed in adults between 20 – 50, even children and teens have some form of DFSP. Race: The risk of DFSP is more in people of Black origin than in other ethnicities. Skin Conditions: People having any skin injury, scars, burns or tattoos have a higher incidence of getting DFSP. Medical Treatments: People who underwent radiation therapy or any surgical incision have an aggravated risk of this type of cancer.

• #50 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. […] What causes dermatofibrosarcoma protuberans? Trauma or injury to the skin may be partly responsible in some cases. It is not known why or how the tumour occurs. […] There have been some genetic changes identified in the cells of DFSP tumours, but these are not inherited and cannot be passed on.

• #51 Dermatofibrosarcoma Protuberans FAQs Houston TX – DFSP GA

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

  Dermatofibrosarcoma protuberans does involve a mutation of the genetic material between chromosomes 17 and 22, but it is not a hereditary condition. […] The causes of this rare form of skin cancer are unknown. There is some thought that dermatofibrosarcoma protuberans can begin on the skin that was badly injured from a burn or from surgery. There is not a link between sun exposure and this rare skin cancer. […] It is not clear what causes this type of skin cancer. As mentioned above, its thought the scarring that develops after a burn or surgery may increase a persons risk for developing dermatofibrosarcoma protuberans (DFSP). But its impossible for a person to prevent scarring after a burn or surgical incision. Women and African Americans are at a higher risk, but again there isnt any prevention protocol, such as applying sunscreen to ward off other skin cancers. DFSP is not caused by sun exposure.

• #52 Dermatofibrosarcoma protuberans (DFSP) | Sarcoma UK

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

  DFSP is thought to be caused when someone has extra chromosomes, or when someone has certain chromosomes that have been broken apart and put back together in the wrong way. […] Researchers are still trying to find out why these things happen and whether they cause DFSP. […] 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. […] However, research has found that chemotherapy is not effective in treating DFSP, so its rarely used. […] 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. But, more research and trials are needed before these treatments are approved.

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