Materiały źródłowe

• #1 Epithelioid Sarcoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/449

  Epithelioid sarcomas are malignant tumors with both mesenchymal and epithelial cell differentiation. Up to 90% of epithelioid sarcomas show loss of integrase interactor-1 (INI-1) expression. INI-1 is part of the SWI/SNF chromatin remodeling complex, which is expressed in all normal nucleated cells. This chromatin remodeling complex is essential for biological function as it alters nucleosomes to allow for DNA transcription. […] A spectrum of gene deletions and rearrangements, including translocation events involving the 22q11 locus, are responsible for tumorigenesis in INI-1 deficient epithelioid sarcomas. […] Intriguingly, an association with prior trauma at the site of the tumor has been noted in up to 27% of occurrences. However, previous trauma to the distal extremity is logically a common occurrence, making it difficult to definitively speculate on its role as an inciting event.

• #1 Epithelioid Sarcoma—From Genetics to Clinical Practice

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7463637/

  Epithelioid sarcoma is characterized by the loss of SMARCB1/INI1 (integrase interactor 1) or other proteins of the SWI/SNF complex. […] Loss of integrase interactor 1 (INI1) function is the most common alteration found in ES, occurring in nearly 90% of cases. […] SMARCB1/INI1 inactivation leads to the deregulation of targeted genes, uncontrolled cellular growth, and neoplastic transformation. […] Other mechanisms that play a crucial role in the pathogenesis of ES are the methylation events catalyzed by histone methyltransferases (HMTs). […] The trimethylated form of H3K27Me3 is connected with the repression of genes crucial for cell differentiation. […] INI1 inactivation results in overactivation of the PRC2 complex, leading to the methylation of histones, promotion of cell proliferation, and silencing the genes responsible for differentiation.

• #1 Epithelioid Sarcoma—From Genetics to Clinical Practice

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7463637/

  High expression of the vascular endothelial growth factor (VEGF) is responsible for angiogenesis and has been described in epithelioid sarcoma samples. […] The dysregulation of adhesion proteins is also described in ES. Complete loss of E-cadherin, a glycoprotein responsible for cell-cell adhesion, has been reported. […] The loss of E-cadherin can be caused by the overexpression of dysadherin, which is a glycoprotein that acts as a negative regulator of E-cadherin. […] Various other alterations have been identified in the ES, including activation of the phosphatidylinositol 3-kinase/protein kinaseB/mammalian target of rapamycin (PI3K/AKT/mTOR) signaling pathway. […] The systemic therapy of ES remains challenging and is not supported by high-quality evidence.

• #1 Epithelioid Sarcoma | Oncology | Bon Secours

  https://www.bonsecours.com/health-care-services/cancer-care-oncology/conditions/epithelioid-sarcoma

  Epithelioid sarcoma is caused when mutations (changes) occur in the soft tissue cells, and then those cells grow and multiply quickly. […] The following factors may increase your risk for developing epithelioid sarcoma: Having specific inherited genetic syndromes, including: Li-Fraumeni syndrome, Familial adenomatous polyposis, Neurofibromatosis, Tuberous sclerosis, Werner syndrome. […] Having been exposed to certain harmful chemicals, including herbicides, dioxin or arsenic. […] Having undergone radiation therapy.

• #1 Epithelioid Sarcoma: Symptoms, Treatment & Prognosis

  https://my.clevelandclinic.org/health/diseases/24331-epithelioid-sarcoma

  Epithelioid sarcoma is a rare soft tissue cancer. […] Healthcare providers arent exactly sure what causes epithelioid sarcoma. But experts know its related to an abnormality of the SMARCB1 gene (which tells your body how to make protein). This gene mutation typically occurs during life. In other words, its usually not something youre born with. […] Like most soft tissue sarcomas, ES often develops for no apparent reason. People whove received radiation therapy for other cancers in the past have a slightly raised risk for epithelioid sarcoma. […] Experts have also discovered that people who are born with certain family cancer syndromes have a slightly higher risk of developing epithelioid sarcoma. These family cancer syndromes include: Gardner syndrome, Neurofibromatosis Type 1 and Type 2, Retinoblastoma, Li-Fraumeni syndrome, Tuberous sclerosis complex, Werner syndrome, Gorlin syndrome.

• #1 What Is Epithelioid Sarcoma?

  https://www.icliniq.com/articles/cancer/epithelioid-sarcoma

  Epithelioid sarcoma is an uncommon cancer of the soft tissues. […] The exact cause of what causes epithelioid sarcoma is unknown. However, it is believed to be related to an abnormality of the SMARCB1 gene, which instructs the body on how to make protein. This gene alteration or mutation is acquired; it occurs with time and is not something one is born with. Like other soft tissue sarcomas, epithelioid sarcoma often develops without any particular reason. People who received radiation therapy treatment for other cancers have a slightly increased risk for epithelioid sarcoma. In addition, people with a family history of certain types of cancer have a slightly higher risk of developing epithelioid sarcoma. […] Such cancers include the following: Neurofibromatosis – A genetic disorder of the nervous system. Retinoblastoma – Eye cancer. Li-Fraumeni Syndrome – A genetic disorder that increases the risk of developing cancer. Werner Syndrome – A genetic disorder causing rapid aging and an increased risk of developing cancer. Gardner Syndrome – An autosomal dominant disease with multiple polyps in the gastrointestinal tract. Tuberous Sclerosis Complex – A rare genetic disease that leads to the growth of tumors in various body parts. Gorlin Syndrome – A rare genetic disease affecting many body organs and tissues.

• #1 Sarcoma: Symptoms, Types, Causes, Treatment & Prevention PACE Hospitals – Best Hospitals in Hitech City, Hyderabad, India | Near Madhapur, Kukatpally, KPHB, Kondapur, Gachibowli, Jubilee Hills, Banjara HillsPACE Hospitals Contact Number f

  https://www.pacehospital.com/sarcoma-symptoms-types-causes-treatment-prevention

  Individuals who have undergone radiation therapy for treating previous cancer are at higher risk of developing sarcoma. […] When an individual is exposed to certain chemicals such as vinyl chloride monomer, dioxin or arsenic the risk of developing sarcoma increases. […] Individual having swelling or lymphedema in the arms or legs for a long period have an increased risk of developing sarcoma. […] Though soft tissue sarcomas can occur at any age, like most cancers the risk of cancer increases with increasing age. […] Less than 1 in 100 patients who have undergone radiotherapy for treating other cancers have a risk of developing sarcoma in the future. […] The ionizing radiation which is present in the environment increases the risk of developing sarcoma. […] Several chemicals can possibly increase the risk of some types of cancer. […] Rare genetic conditions can increase the risk of developing sarcoma.

• #1 Risks and causes | Soft tissue sarcoma | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/soft-tissue-sarcoma/risks-causes

  We don’t know what causes most soft tissue sarcomas. But some factors may increase your risk of developing it. […] Several chemicals are thought to possibly increase the risk of some types of sarcoma. […] The International Agency for Research on Cancer looks at the evidence for substances and their links with causing cancer. They say the following chemicals have limited evidence for causing soft tissue sarcoma: radioiodines, including iodine-131, polychlorophenols, 2,3,7,8-tetrachlorodibenzo-para-dioxin. […] Some types of rare genetic conditions can increase your risk of getting a sarcoma. […] Families with Li Fraumeni syndrome have a higher risk of developing: soft tissue sarcoma, other cancers. […] Children who have had retinoblastoma have an increased risk of developing a soft tissue sarcoma in the future. […] Kaposi’s sarcoma is caused by Human Herpes Virus 8 infection (HHV8). […] An infection called Epstein Barr virus (EBV) has been linked with leiomyosarcoma. […] There’s no evidence that an injury of any kind can cause a sarcoma.

• #1 Soft Tissue Sarcoma Symptoms, Causes & Treatments

  https://www.cancercenter.com/cancer-types/soft-tissue-sarcoma

  What causes soft tissue sarcoma? […] Some common risk factors for soft tissue sarcoma, also called adult soft tissue sarcoma, include: […] Hereditary conditions, including familial adenomatous polyposis (FAP or Gardners syndrome), retinoblastoma, neurofibromatosis type 1 (von Recklinghausen disease or NF1), tuberous sclerosis, Werner syndrome and Li-Fraumeni syndrome […] Chemical exposure, including to vinyl chloride and dioxin […] Previous radiation treatment […] Weakened immune system, including from the human immunodeficiency virus (HIV)

• #1 Risk Factors for Soft Tissue Sarcoma | Stanford Health Care

  https://stanfordhealthcare.org/medical-conditions/cancer/soft-tissue-sarcoma/causes.html

  There is really no way to know for sure if you’re going to get soft tissue sarcoma. Most people who get this type of cancer have no risk factors. Remember, just because you have one or more risk factors doesn’t necessarily mean you will get soft tissue sarcoma. In fact, you can have all the risk factors and still not get soft tissue sarcoma, or you can have no known risk factors and still get it. […] While the majority of people who get soft tissue sarcomas have no known risk factors, certain things can make one person more likely to get a soft tissue sarcoma than another person. […] People who have had high-dose radiation to treat other cancers, such as breast or cervical cancer, have a slightly higher risk of developing a soft tissue sarcoma. […] Chronic lymphedema (where fluid collects in the tissue and causes swelling) after radiation to lymph nodes, or surgical removal of lymph nodes is also a risk factor.

• #1 Epithelioid Sarcoma – SFA

  https://curesarcoma.org/sarcoma-subtypes/epithelioid-sarcoma/

  Epithelioid sarcoma is a rare malignant soft tissue tumor with epithelioid characteristics, occurring in two forms: classic distal and proximal large cell, with the latter often affecting deeper tissues. […] The etiology is unknown. Three studies report (probably coincidental) antecedent trauma in 20%, 27%, and 73% of patients. There is no recurrent genetic predisposition.

• #1 Epithelioid sarcoma – Hancock Health

  https://www.hancockhealth.org/fr/mayo-health-library/epithelioid-sarcoma/

  Epithelioid sarcoma is a rare type of cancer that starts as a growth of cells in the soft tissue. […] The cause of epithelioid sarcoma isn’t known. This cancer causes a growth of cells in the soft tissue. […] Epithelioid sarcoma starts when soft tissue cells get changes in their DNA. […] The risk of epithelioid sarcoma is higher in younger people. […] There is no way to prevent epithelioid sarcoma.

• #1 Epithelioid sarcoma: A rare neoplasm presenting in a sporotrichoid pattern – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/epithelioid-sarcoma-a-rare-neoplasm-presenting-in-a-sporotrichoid-pattern/

  Epithelioid sarcoma is a rare and an indolent soft-tissue sarcoma, which invariably displays epithelial differentiation. In one of the studies, inactivation of SMARCB1/INI1 suppressor gene is postulated as the probable molecular mechanism for this condition, as observed in six out of 11 cases. This is tested by immunohistochemistry studies, as noted in the present case. Epithelioid sarcoma commonly affects patients in the 20-40 years age group, with slight male preponderance of 1.8:1. It is usually seen on distal extremities, with average duration of the presentation being 29-41 months, signifying the indolent nature of the tumor. […] The presenting lesion of epithelioid sarcoma is a nodule, in most cases that grow outward and ulcerate early or a subcutaneous swelling which is usually painless. Pain, paresthesia and muscle wasting due to growth along a nerve trunk are rarely observed. Perineural and perivascular extension of tumor cells may result in a sporotrichoid distribution, which has been reported as rare presentation.

• #1 Types of Soft Tissue Sarcoma | Memorial Sloan Kettering Cancer Center

  https://www.mskcc.org/cancer-care/types/soft-tissue-sarcoma/types

  Epithelioid sarcoma: These tumors are more common in young adults. The classic form of the disease grows slowly and occurs in the feet, arms, legs, or forearms of younger men. Epithelioid tumors can also begin in the groin, and these tumors tend to be more aggressive.

• #1 Primary pleural epithelioid sarcoma of the proximal type: a diagnostic and therapeutic challenge – Ahmad – Translational Lung Cancer Research

  https://tlcr.amegroups.org/article/view/32465/html

  Epithelioid sarcoma (ES) is an uncommon soft tissue neoplasm first described in 1970. […] The proximal-type variant of this rare soft tissue neoplasm was only recently reported. […] The proximal type variant has distinct histological characteristics and aggressive clinical course as compared to the distal ES. […] Inactivation of INI1 has been reported in both distal and proximal variants and can help to make the diagnosis. […] Furthermore, the proximal variant has a possible association with malignant rhabdoid neoplasm. […] The presence of CD34 reactivity, the occurrence of tumors in deep soft tissues without any connection with the overlying epidermis or cutaneous adnexal tissue and the absence of histologic features of squamous or glandular differentiation favor the diagnosis of ES over undifferentiated carcinoma. […] The identification of the tumor suppressor gene SMARCB1 inactivation also favors the diagnosis of proximal-type ES. […] Comprehensive tumor molecular profiling should be considered to better understand the molecular drivers and guide more precise and individualized treatment decisions.

• #2 Epithelioid Sarcoma: Symptoms, Treatment & Prognosis

  https://my.clevelandclinic.org/health/diseases/24331-epithelioid-sarcoma

  Epithelioid sarcoma is a rare soft tissue cancer. […] Healthcare providers arent exactly sure what causes epithelioid sarcoma. But experts know its related to an abnormality of the SMARCB1 gene (which tells your body how to make protein). This gene mutation typically occurs during life. In other words, its usually not something youre born with. […] Like most soft tissue sarcomas, ES often develops for no apparent reason. People whove received radiation therapy for other cancers in the past have a slightly raised risk for epithelioid sarcoma. […] Experts have also discovered that people who are born with certain family cancer syndromes have a slightly higher risk of developing epithelioid sarcoma. These family cancer syndromes include: Gardner syndrome, Neurofibromatosis Type 1 and Type 2, Retinoblastoma, Li-Fraumeni syndrome, Tuberous sclerosis complex, Werner syndrome, Gorlin syndrome.

• #2 Epithelioid sarcoma – Wikipedia

  https://en.wikipedia.org/wiki/Epithelioid_sarcoma

  Epithelioid sarcoma is a rare soft tissue sarcoma arising from mesenchymal tissue and characterized by epithelioid-like features. It accounts for less than 1% of all soft tissue sarcomas. […] The most common genetic mutation (found in 80-90% of epithelioid sarcomas) is the inactivation of the SMARCB1 gene, or the loss of protein INI1 function. […] Loss of SMARCB1 function is the most common genetic mutation observed in epithelioid sarcoma, and this dysfunction is likely a major driver of disease progression. […] Epithelioid sarcoma also demonstrates lymphatic spread (in 22-48% of cases), and metastasis (in 21-63% of cases). […] These events, as well as advanced stage (progression) and grade (aggressiveness), are predictive of an overall worse outcome. […] Advanced stage and grade are associated with worse outcomes.

• #2 Epithelioid Sarcoma | Basicmedical Key

  https://basicmedicalkey.com/epithelioid-sarcoma-3/

  Epithelioid Sarcoma Cyril Fisher, MD, DSc, FRCPath Key Facts Terminology […] Etiology/Pathogenesis […] Some have abnormalities of chromosome 22q […] Some cases have abnormalities of chromosome 22q […] Rare association with neurofibromatosis type 2.

• #2 Pathology Outlines – Epithelioid sarcoma-vulva

  https://www.pathologyoutlines.com/topic/vulvaepithelioidsarcoma.html

  Epithelioid sarcoma of the vulva and deep pelvic soft tissue is a rare malignant neoplasm characterized by SMARCB1 / INI1 deletion. […] Most vulvar and pelvic epithelioid sarcomas are of the proximal type and show more aggressive clinical behavior than distal type (classical) epithelioid sarcoma. […] Histogenesis uncertain. […] Homozygous deletions of SMARCB1 / INI1 in 80 – 90% of proximal type epithelioid sarcoma. […] SMARCB1 / INI1 deletion correlates strongly with SMARCB1 / INI1 loss by IHC.

• #2 Soft Tissue Sarcoma Symptoms, Causes & Treatments

  https://www.cancercenter.com/cancer-types/soft-tissue-sarcoma

  What causes soft tissue sarcoma? […] Some common risk factors for soft tissue sarcoma, also called adult soft tissue sarcoma, include: […] Hereditary conditions, including familial adenomatous polyposis (FAP or Gardners syndrome), retinoblastoma, neurofibromatosis type 1 (von Recklinghausen disease or NF1), tuberous sclerosis, Werner syndrome and Li-Fraumeni syndrome […] Chemical exposure, including to vinyl chloride and dioxin […] Previous radiation treatment […] Weakened immune system, including from the human immunodeficiency virus (HIV)

• #2 Epithelioid Sarcoma | Oncology | Bon Secours

  https://www.bonsecours.com/health-care-services/cancer-care-oncology/conditions/epithelioid-sarcoma

  Epithelioid sarcoma is caused when mutations (changes) occur in the soft tissue cells, and then those cells grow and multiply quickly. […] The following factors may increase your risk for developing epithelioid sarcoma: Having specific inherited genetic syndromes, including: Li-Fraumeni syndrome, Familial adenomatous polyposis, Neurofibromatosis, Tuberous sclerosis, Werner syndrome. […] Having been exposed to certain harmful chemicals, including herbicides, dioxin or arsenic. […] Having undergone radiation therapy.

• #2 Childhood Soft Tissue Sarcoma – NCI

  https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq

  Childhood soft tissue sarcoma is caused by certain changes to the way soft tissue cells function, especially how they grow and divide into new cells. Often, the exact cause of these changes is unknown. […] The risk of soft tissue sarcoma may be increased if your child has one of the following inherited disorders: Li-Fraumeni syndrome, RB1 gene changes, Werner syndrome, tuberous sclerosis, adenosine deaminase-deficient severe combined immunodeficiency. […] Another risk factor is past treatment with radiation therapy. […] Epithelioid sarcoma is linked to a change in a tumor suppressor gene called SMARCB1. This type of gene makes a protein that helps control cell growth.

• #2 What Is Epithelioid Sarcoma?

  https://www.icliniq.com/articles/cancer/epithelioid-sarcoma

  Epithelioid sarcoma is an uncommon cancer of the soft tissues. […] The exact cause of what causes epithelioid sarcoma is unknown. However, it is believed to be related to an abnormality of the SMARCB1 gene, which instructs the body on how to make protein. This gene alteration or mutation is acquired; it occurs with time and is not something one is born with. Like other soft tissue sarcomas, epithelioid sarcoma often develops without any particular reason. People who received radiation therapy treatment for other cancers have a slightly increased risk for epithelioid sarcoma. In addition, people with a family history of certain types of cancer have a slightly higher risk of developing epithelioid sarcoma. […] Such cancers include the following: Neurofibromatosis – A genetic disorder of the nervous system. Retinoblastoma – Eye cancer. Li-Fraumeni Syndrome – A genetic disorder that increases the risk of developing cancer. Werner Syndrome – A genetic disorder causing rapid aging and an increased risk of developing cancer. Gardner Syndrome – An autosomal dominant disease with multiple polyps in the gastrointestinal tract. Tuberous Sclerosis Complex – A rare genetic disease that leads to the growth of tumors in various body parts. Gorlin Syndrome – A rare genetic disease affecting many body organs and tissues.

• #2 Risk Factors for Soft Tissue Sarcoma | Stanford Health Care

  https://stanfordhealthcare.org/medical-conditions/cancer/soft-tissue-sarcoma/causes.html

  There is really no way to know for sure if you’re going to get soft tissue sarcoma. Most people who get this type of cancer have no risk factors. Remember, just because you have one or more risk factors doesn’t necessarily mean you will get soft tissue sarcoma. In fact, you can have all the risk factors and still not get soft tissue sarcoma, or you can have no known risk factors and still get it. […] While the majority of people who get soft tissue sarcomas have no known risk factors, certain things can make one person more likely to get a soft tissue sarcoma than another person. […] People who have had high-dose radiation to treat other cancers, such as breast or cervical cancer, have a slightly higher risk of developing a soft tissue sarcoma. […] Chronic lymphedema (where fluid collects in the tissue and causes swelling) after radiation to lymph nodes, or surgical removal of lymph nodes is also a risk factor.

• #2 Risks and causes | Soft tissue sarcoma | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/soft-tissue-sarcoma/risks-causes

  We don’t know what causes most soft tissue sarcomas. But some factors may increase your risk of developing it. […] Several chemicals are thought to possibly increase the risk of some types of sarcoma. […] The International Agency for Research on Cancer looks at the evidence for substances and their links with causing cancer. They say the following chemicals have limited evidence for causing soft tissue sarcoma: radioiodines, including iodine-131, polychlorophenols, 2,3,7,8-tetrachlorodibenzo-para-dioxin. […] Some types of rare genetic conditions can increase your risk of getting a sarcoma. […] Families with Li Fraumeni syndrome have a higher risk of developing: soft tissue sarcoma, other cancers. […] Children who have had retinoblastoma have an increased risk of developing a soft tissue sarcoma in the future. […] Kaposi’s sarcoma is caused by Human Herpes Virus 8 infection (HHV8). […] An infection called Epstein Barr virus (EBV) has been linked with leiomyosarcoma. […] There’s no evidence that an injury of any kind can cause a sarcoma.

• #2 Soft Tissue Sarcoma: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/soft-tissue-sarcoma-causes-symptoms-and-treatment?srsltid=AfmBOopx5TEgxDWui-dsvj0zWtLq9OLXRjQHuYjuNjnDosfs01qHHpXT

  Inherited Diseases: Genetic syndromes like Hereditary retinoblastoma, Li-Fraumeni syndrome, Familial adenomatous polyposis, Neurofibromatosis, Tuberous sclerosis and Werner syndrome may increase the risk of soft tissue sarcoma […] Weak Immunity: Having a compromised immunity due to HIV or AIDS makes a person prone to soft tissue sarcoma […] Other Health Conditions: People who are suffering from lymphedema in their arms or legs for a long time are more susceptible to soft tissue sarcoma.

• #2 Pathology Outlines – Epithelioid sarcoma

  https://www.pathologyoutlines.com/topic/softtissueepithelioidsarcoma.html

  An association with prior trauma at the site of the tumor has been noted in up to 27% of occurrences. […] Epithelioid sarcoma is a malignant mesenchymal neoplasm that exhibits epithelioid cytomorphology and a predominantly epithelial phenotype.

• #2 Epithelioid sarcoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/epithelioid-sarcoma?embed_domain=external.radpair.com%27%5B0%5D%27%5B0%5Dfavicon.icofavicon.icofavicon.ico&lang=us

  Epithelioid sarcomas are malignant usually slow-growing mesenchymal tumors of unknown and multidirectional differentiation. […] The etiology of epithelioid sarcomas is unknown. Some studies have been reporting a previous trauma which at this point is considered a probable coincidence. […] Epithelioid sarcomas are associated with mutations of the SMARCB1 or INI1 gene resulting in a loss of SMARCB1 nuclear protein expression.

• #2 Epithelioid Sarcoma | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/e/epithelioid-sarcoma

  Epithelioid sarcoma is a type of soft tissue sarcoma that typically appears in the extremities (especially in the arms and hands). It also can develop in the main part of the body. It can affect both children and adults but is most common in young adulthood. […] Experts do not know what causes epithelioid sarcoma, but the disease is linked with an abnormality in the SMARCB1 gene (sometimes called INI-1). Doctors can order testing of the tumor to identify this abnormality when confirming a diagnosis of epithelioid sarcoma.

• #2 Epithelioid Sarcoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/449

  Epithelioid sarcomas are malignant tumors with both mesenchymal and epithelial cell differentiation. Up to 90% of epithelioid sarcomas show loss of integrase interactor-1 (INI-1) expression. INI-1 is part of the SWI/SNF chromatin remodeling complex, which is expressed in all normal nucleated cells. This chromatin remodeling complex is essential for biological function as it alters nucleosomes to allow for DNA transcription. […] A spectrum of gene deletions and rearrangements, including translocation events involving the 22q11 locus, are responsible for tumorigenesis in INI-1 deficient epithelioid sarcomas. […] Intriguingly, an association with prior trauma at the site of the tumor has been noted in up to 27% of occurrences. However, previous trauma to the distal extremity is logically a common occurrence, making it difficult to definitively speculate on its role as an inciting event.

• #2 Sarcoma: Symptoms, Types, Causes, Treatment & Prevention PACE Hospitals – Best Hospitals in Hitech City, Hyderabad, India | Near Madhapur, Kukatpally, KPHB, Kondapur, Gachibowli, Jubilee Hills, Banjara HillsPACE Hospitals Contact Number f

  https://www.pacehospital.com/sarcoma-symptoms-types-causes-treatment-prevention

  There is no exact known cause of sarcoma but there are a few known factors such as bone diseases, exposure to certain chemicals, some genetic syndromes, lymphedema, and previous medical history of radiation or chemotherapy treatment which can increase the risk of developing sarcoma. […] There is no exact known cause of cancer but there are several factors that can increase an individual’s risk of developing sarcoma. […] Individuals with a family history of inherited disorders such as Von Recklinghausen’s disease (neurofibromatosis), Gardner syndrome (rare, inherited disorder that causes abnormal growths in the colon and rectum), Werner syndrome (rare genetic disorder causing premature aging), tuberous sclerosis (non-cancerous tumors growing in the brain and various parts of the body), nevoid basal cell carcinoma syndrome (a rare genetic disorder that increases the risk of developing skin cancer), Li-Fraumeni syndrome (an inherited condition which increases the risk of developing several types of cancer) or retinoblastoma are at higher risk of developing sarcoma.

• #3 An Overview of Epithelioid Sarcoma

  https://www.pharmacytimes.com/view/an-overview-of-epithelioid-sarcoma

  Epithelioid sarcoma (ES) is marked, in more than 90% of cases, by a genetic mutation referred to as inactivation, deletion, or loss of the SWI/SNF-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1 gene (SMARCB1 or INI-1). Loss of this tumor-suppressor gene can result in oncogenic transformation and thus the development of ES. […] Mechanistically, tazemetostat is a potent and selective inhibitor of histone methyltransferase EZH2 (enhancer of zeste homologue 2). EZH2 becomes overexpressed in certain cancers, such as ES, that are characterized by loss of function of the INI-1 gene. Overexpression of EZH2 results in oncogenic transformation.

• #3 What Is Epithelioid Sarcoma?

  https://www.icliniq.com/articles/cancer/epithelioid-sarcoma

  Epithelioid sarcoma is an uncommon cancer of the soft tissues. […] The exact cause of what causes epithelioid sarcoma is unknown. However, it is believed to be related to an abnormality of the SMARCB1 gene, which instructs the body on how to make protein. This gene alteration or mutation is acquired; it occurs with time and is not something one is born with. Like other soft tissue sarcomas, epithelioid sarcoma often develops without any particular reason. People who received radiation therapy treatment for other cancers have a slightly increased risk for epithelioid sarcoma. In addition, people with a family history of certain types of cancer have a slightly higher risk of developing epithelioid sarcoma. […] Such cancers include the following: Neurofibromatosis – A genetic disorder of the nervous system. Retinoblastoma – Eye cancer. Li-Fraumeni Syndrome – A genetic disorder that increases the risk of developing cancer. Werner Syndrome – A genetic disorder causing rapid aging and an increased risk of developing cancer. Gardner Syndrome – An autosomal dominant disease with multiple polyps in the gastrointestinal tract. Tuberous Sclerosis Complex – A rare genetic disease that leads to the growth of tumors in various body parts. Gorlin Syndrome – A rare genetic disease affecting many body organs and tissues.

• #3 Sarcoma: Symptoms, Types, Causes, Treatment & Prevention PACE Hospitals – Best Hospitals in Hitech City, Hyderabad, India | Near Madhapur, Kukatpally, KPHB, Kondapur, Gachibowli, Jubilee Hills, Banjara HillsPACE Hospitals Contact Number f

  https://www.pacehospital.com/sarcoma-symptoms-types-causes-treatment-prevention

  Individuals who have undergone radiation therapy for treating previous cancer are at higher risk of developing sarcoma. […] When an individual is exposed to certain chemicals such as vinyl chloride monomer, dioxin or arsenic the risk of developing sarcoma increases. […] Individual having swelling or lymphedema in the arms or legs for a long period have an increased risk of developing sarcoma. […] Though soft tissue sarcomas can occur at any age, like most cancers the risk of cancer increases with increasing age. […] Less than 1 in 100 patients who have undergone radiotherapy for treating other cancers have a risk of developing sarcoma in the future. […] The ionizing radiation which is present in the environment increases the risk of developing sarcoma. […] Several chemicals can possibly increase the risk of some types of cancer. […] Rare genetic conditions can increase the risk of developing sarcoma.

• #3 Epithelioid Sarcoma – SFA

  https://curesarcoma.org/sarcoma-subtypes/epithelioid-sarcoma/

  Epithelioid sarcoma is a rare malignant soft tissue tumor with epithelioid characteristics, occurring in two forms: classic distal and proximal large cell, with the latter often affecting deeper tissues. […] The etiology is unknown. Three studies report (probably coincidental) antecedent trauma in 20%, 27%, and 73% of patients. There is no recurrent genetic predisposition.

• #3 Soft tissue sarcomas | NHS inform

  https://www.nhsinform.scot/illnesses-and-conditions/cancer/cancer-types-in-teenagers-and-young-adults/soft-tissue-sarcomas-teenagers-and-young-adults/

  Its unknown what causes sarcomas in teenagers and young adults. […] People often think a knock or injury might have caused a sarcoma, but theres no evidence for this. […] Remember that nothing youve done has caused the cancer. […] Some rare conditions that run in families (known as genetic conditions) can increase the chance (risk) of getting a soft tissue sarcoma. […] People who had radiotherapy for another type of cancer when they were a child may have an increased risk of developing a sarcoma in later life this would be from their teens onwards. […] People often think a knock or injury might have caused a sarcoma, but theres no evidence for this.

• #3 Types of Soft Tissue Sarcoma | Memorial Sloan Kettering Cancer Center

  https://www.mskcc.org/cancer-care/types/soft-tissue-sarcoma/types

  Epithelioid sarcoma: These tumors are more common in young adults. The classic form of the disease grows slowly and occurs in the feet, arms, legs, or forearms of younger men. Epithelioid tumors can also begin in the groin, and these tumors tend to be more aggressive.

• #3 Selected topics in the pathology of epithelioid soft tissue tumors | Modern Pathology

  https://www.nature.com/articles/modpathol2013175

  Epithelioid morphology, mimicking carcinoma, is a key or defining feature of several soft tissue tumors and may be seen in a wide variety of other tumors. […] The role of loss of the SMARCB1 tumor-suppressor gene in the pathogenesis of these epithelioid soft tissue tumors will be discussed. […] Over 90% of ESs of all types show the loss of expression of the tumor-suppressor gene product SMARCB1/INI1/BAF47 (SMARCB1). […] The loss of SMARCB1 expression is also seen in 90% of ESs of all types. […] The precise relationship between SMARCB1 abnormalities and epithelioid morphology remains to be fully elucidated; however, the presence of such aberrations in ES, MERT, and EMPNST suggests that this is more than a coincidence.

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