Materiały źródłowe

• #1 Undifferentiated Pleomorphic Sarcoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/112229

  The exact pathogenic mechanisms of UPS remain obscure. In general, STSs are composed of heterogeneous populations of cells with mesenchymal features that can originate from simple genomic alterations or complex genomics. UPS belongs to the latter group, showcasing various potential cellular backgrounds, mutational signatures, and altered signaling pathways. […] The tumorigenesis of UPS is reportedly initiated by a subpopulation of cells called side population (SP) cells, which the Hoechst dye efflux assay can identify. According to an elegant xenograft experimental model, these cells have an increased capacity for self-renewal, growth, proliferation and can recapitulate tumor formation. […] The Hippo pathway may also be implicated in UPS tumor biology, as vestigial-like family member 3 (VGLL3) and yes1-associated transcriptional regulator (YAP1) cofactors were found to be highly amplified on a genome sequencing study.

• #2 Undifferentiated Pleomorphic Sarcoma | Sarcoma UK

  https://sarcoma.org.uk/about-sarcoma/what-is-sarcoma/types-of-sarcoma/undifferentiated-pleomorphic-sarcoma/

  Undifferentiated pleomorphic sarcoma, also known as UPS, is one of the most common types of sarcoma. […] The cause of most UPS is unknown. But, exposure to radiation has been shown to cause some UPS. […] Researchers are still trying to learn more about the causes of UPS.

• #3 Undifferentiated Pleomorphic Sarcoma: Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/22435-undifferentiated-pleomorphic-sarcoma

  Undifferentiated pleomorphic sarcoma causes […] Experts arent sure what causes undifferentiated pleomorphic sarcoma. They know it happens when healthy cells develop changes in their DNA, but they dont know what causes those changes. […] Most people who develop UPS dont have any known risk factors.

• #4 Mayo Clinic Health Library – Undifferentiated pleomorphic sarcoma | Swiss Medical Network

  https://www.swissmedical.net/en/healtcare-library/con-20344525

  It’s not clear what causes undifferentiated pleomorphic sarcoma. […] Doctors know this cancer begins when a cell develops changes in its DNA. A cell’s DNA contains the instructions that tell a cell what to do. The changes tell the cell to multiply rapidly, creating a mass of abnormal cells (tumor). The cells can invade and destroy nearby healthy tissue. In time, the cancer cells can break away and spread (metastasize) to other parts of the body, such as the lungs and bones.

• #5 Undifferentiated pleomorphic sarcoma – Vejthani Hospital | JCI Accredited International Hospital in Bangkok, Thailand.

  https://www.vejthani.com/diseases-conditions/undifferentiated-pleomorphic-sarcoma/

  The exact cause of undifferentiated pleomorphic sarcoma remains unclear. However, it is understood that the cancer originates from a cell that undergoes changes in its DNA. […] When these changes occur, the affected cells start to multiply rapidly, leading to the formation of an abnormal mass of cells, referred to as a tumor. […] These cells have the ability to invade and damage nearby healthy tissues. Over time, the cancerous cells may also detach from the original tumor and spread to other areas of the body through a process called metastasis.

• #6 Undifferentiated Pleomorphic Sarcoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/112229

  The exact pathogenic mechanisms of UPS remain obscure. In general, STSs are composed of heterogeneous populations of cells with mesenchymal features that can originate from simple genomic alterations or complex genomics. UPS belongs to the latter group, showcasing various potential cellular backgrounds, mutational signatures, and altered signaling pathways. […] The tumorigenesis of UPS is reportedly initiated by a subpopulation of cells called side population (SP) cells, which the Hoechst dye efflux assay can identify. According to an elegant xenograft experimental model, these cells have an increased capacity for self-renewal, growth, proliferation and can recapitulate tumor formation. […] The Hippo pathway may also be implicated in UPS tumor biology, as vestigial-like family member 3 (VGLL3) and yes1-associated transcriptional regulator (YAP1) cofactors were found to be highly amplified on a genome sequencing study.

• #7 Undifferentiated Pleomorphic Sarcoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/112229

  In various other reports, there are well-documented loss or deletion mutations of phosphatase and tensin homolog (PTEN) and overexpression of phosphorylated protein kinase B (pAKT) in UPS, involved in the PIK3/PTEN/AKT/mTOR pathway. […] Similarly, Dickkopf-related protein 1 (DKK1), a Wnt/B-catenin signaling pathway inhibitor, was differentially overexpressed in UPS compared to other STSs. […] Mutations in tumor protein 53 (TP53), cyclin-dependent kinase inhibitor 2A (CDKN2A), retinoblastoma-associated protein (RB1), and transcriptional regulator ATRX (ATRX) genes, as well as PR domain zinc finger protein 10 (PRDM10) and triple functional domain protein (TRIO) gene fusions have also been identified in UPS. […] Besides genetic aberrations, radiation therapy (RT) is a known risk factor for STS development. Indeed, radiation-associated STS presents in around 1% to 3% of subjects diagnosed with any sarcoma. Specifically, in a series of 1068 UPS cases, 5.1% of the patients had a prior history of radiation.

• #8 Undifferentiated Pleomorphic Sarcoma | Orthopedic Surgery | Patient Care | Montefiore Einstein

  https://montefioreeinstein.org/patient-care/services/orthopedics/specialties/orthopedic-oncology/pleomorphic-sarcoma

  Undifferentiated pleomorphic sarcoma (UPS) is a rare type of cancer that arises in soft tissue. UPS represents about 10 percent of all adult soft-tissue sarcomas, making it one of the most common subtypes. […] Several specific genetic mutations have been linked to undifferentiated pleomorphic sarcoma. These include genes such as TP53 and RB1, which are important tumor suppressors, as well as ATRX, a regulatory gene thought to manage chromatin remodeling. In some instances, genes may be amplified, while in other cases, certain genes may be deleted or lost. There is no evidence that environmental, social, dietary or other external factors contribute to its development.

• #9 What Is Undifferentiated Pleomorphic Sarcoma?

  https://www.icliniq.com/articles/cancer/undifferentiated-pleomorphic-sarcoma

  Undifferentiated pleomorphic sarcomas (UPS) have also been found to have mutations in the following: Tumor protein 53 (TP53), Cyclin-dependent kinase inhibitor 2A (CDKN2A), Retinoblastoma-associated protein (RB1), Transcriptional regulator ATRX (ATRX) genes, PR domain zinc finger protein 10 (PRDM10), Triple functional domain protein (TRIO) gene fusions.

• #10 Undifferentiated Pleomorphic Sarcoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/112229

  In various other reports, there are well-documented loss or deletion mutations of phosphatase and tensin homolog (PTEN) and overexpression of phosphorylated protein kinase B (pAKT) in UPS, involved in the PIK3/PTEN/AKT/mTOR pathway. […] Similarly, Dickkopf-related protein 1 (DKK1), a Wnt/B-catenin signaling pathway inhibitor, was differentially overexpressed in UPS compared to other STSs. […] Mutations in tumor protein 53 (TP53), cyclin-dependent kinase inhibitor 2A (CDKN2A), retinoblastoma-associated protein (RB1), and transcriptional regulator ATRX (ATRX) genes, as well as PR domain zinc finger protein 10 (PRDM10) and triple functional domain protein (TRIO) gene fusions have also been identified in UPS. […] Besides genetic aberrations, radiation therapy (RT) is a known risk factor for STS development. Indeed, radiation-associated STS presents in around 1% to 3% of subjects diagnosed with any sarcoma. Specifically, in a series of 1068 UPS cases, 5.1% of the patients had a prior history of radiation.

• #11 Undifferentiated Pleomorphic Sarcoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/112229

  In various other reports, there are well-documented loss or deletion mutations of phosphatase and tensin homolog (PTEN) and overexpression of phosphorylated protein kinase B (pAKT) in UPS, involved in the PIK3/PTEN/AKT/mTOR pathway. […] Similarly, Dickkopf-related protein 1 (DKK1), a Wnt/B-catenin signaling pathway inhibitor, was differentially overexpressed in UPS compared to other STSs. […] Mutations in tumor protein 53 (TP53), cyclin-dependent kinase inhibitor 2A (CDKN2A), retinoblastoma-associated protein (RB1), and transcriptional regulator ATRX (ATRX) genes, as well as PR domain zinc finger protein 10 (PRDM10) and triple functional domain protein (TRIO) gene fusions have also been identified in UPS. […] Besides genetic aberrations, radiation therapy (RT) is a known risk factor for STS development. Indeed, radiation-associated STS presents in around 1% to 3% of subjects diagnosed with any sarcoma. Specifically, in a series of 1068 UPS cases, 5.1% of the patients had a prior history of radiation.

• #12

  https://www.orthobullets.com/pathology/8064/undifferentiated-pleomorphic-sarcoma

  Undifferentiated Pleomorphic Sarcoma, previously known as malignant fibrous histiocytoma, is a high-grade, aggressive, malignant fibrogenic tumor. […] Etiology: Genetics: mutations: programmed cell death protein 1 (PD-1) expression in 30%.

• #13 Undifferentiated pleomorphic sarcoma of skin in unusual locations: Report of two cases | 2021, Volume 32 – Issue 1 | Joint Diseases and Related Surgery

  https://www.jointdrs.org/full-text/1229

  Undifferentiated pleomorphic sarcoma (UPS) of the skin is a rare soft tissue sarcoma subtype with a high risk of metastasis and local recurrence. Ultraviolet exposure plays a prominent role in its etiology. […] The mutation of telomerase reverse transcriptase (TERT) is present in 76% of the skin UPS cases and appears to be associated with the UV damage. […] Thus, the increased incidence in the sun-exposed or UV-damaged skin areas such as head and neck can be explained by TERT mutation. […] On the other hand, the presence of a rapidly growing aggressive lesion on the non-UV-exposed skin areas, particularly in elderly patients, it is recommended to rule out other possible malignancies before to diagnose UPS due to its rarity. […] Both of the reported cases herein were located on a limited sun-exposed region of the body (i.e., the proximal calf and the anterior aspect of the distal cruris), unlike reported in the literature (i.e., the head and neck region).

• #14 Undifferentiated Pleomorphic Sarcoma – MD Searchlight

  https://mdsearchlight.com/cancer/undifferentiated-pleomorphic-sarcoma/

  The exact causes of undifferentiated pleomorphic sarcoma (UPS), a type of cancer, are still unknown. Generally, soft tissue sarcomas (STSs), which include UPS, are made up of different types of cells that can come from simple or complex genetic changes. UPS falls into the category of complex genetic changes, with various types of cells, genetic mutations, and altered signaling pathways involved. […] The development of UPS is thought to start with certain cells called side population (SP) cells. Tests can identify these cells, and researchers believe these cells can grow, multiply, and can form tumors. […] Studies show that two signaling pathways, Hedgehog and Notch, are notably increased at both the cellular and tissue levels. […] The Hippo pathway, another signaling pathway, could also play a role in UPS. Certain genes, VGLL3 and YAP1, were found to be greatly increased in UPS.

• #15 What Is Undifferentiated Pleomorphic Sarcoma?

  https://www.icliniq.com/articles/cancer/undifferentiated-pleomorphic-sarcoma

  Undifferentiated pleomorphic sarcomas (UPS) have a specific pathogenic mechanism that is unknown. […] Undifferentiated pleomorphic sarcomas (UPS) exhibit a variety of possible cellular backgrounds, mutational markers, and altered signaling pathways. […] The carcinogenesis of undifferentiated pleomorphic sarcomas (UPS) is thought to be triggered by a subpopulation of cells known as side population (SP) cells, which the Hoechst dye efflux test may identify. […] Radiation therapy, when used to treat other forms of cancer detected in the same place, has also been associated with this illness but this is extremely rare. […] Undifferentiated pleomorphic sarcomas (UPS)-derived side population (SP) cell investigations revealed that the Hedgehog and Notch signaling pathways and their downstream transcriptional targets are highly elevated at both the subcellular and tissue levels.

• #16 Undifferentiated Pleomorphic Sarcoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/112229

  The exact pathogenic mechanisms of UPS remain obscure. In general, STSs are composed of heterogeneous populations of cells with mesenchymal features that can originate from simple genomic alterations or complex genomics. UPS belongs to the latter group, showcasing various potential cellular backgrounds, mutational signatures, and altered signaling pathways. […] The tumorigenesis of UPS is reportedly initiated by a subpopulation of cells called side population (SP) cells, which the Hoechst dye efflux assay can identify. According to an elegant xenograft experimental model, these cells have an increased capacity for self-renewal, growth, proliferation and can recapitulate tumor formation. […] The Hippo pathway may also be implicated in UPS tumor biology, as vestigial-like family member 3 (VGLL3) and yes1-associated transcriptional regulator (YAP1) cofactors were found to be highly amplified on a genome sequencing study.

• #17 Undifferentiated Pleomorphic Sarcoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/112229

  In various other reports, there are well-documented loss or deletion mutations of phosphatase and tensin homolog (PTEN) and overexpression of phosphorylated protein kinase B (pAKT) in UPS, involved in the PIK3/PTEN/AKT/mTOR pathway. […] Similarly, Dickkopf-related protein 1 (DKK1), a Wnt/B-catenin signaling pathway inhibitor, was differentially overexpressed in UPS compared to other STSs. […] Mutations in tumor protein 53 (TP53), cyclin-dependent kinase inhibitor 2A (CDKN2A), retinoblastoma-associated protein (RB1), and transcriptional regulator ATRX (ATRX) genes, as well as PR domain zinc finger protein 10 (PRDM10) and triple functional domain protein (TRIO) gene fusions have also been identified in UPS. […] Besides genetic aberrations, radiation therapy (RT) is a known risk factor for STS development. Indeed, radiation-associated STS presents in around 1% to 3% of subjects diagnosed with any sarcoma. Specifically, in a series of 1068 UPS cases, 5.1% of the patients had a prior history of radiation.

• #18 Undifferentiated Pleomorphic Sarcoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/112229

  The exact pathogenic mechanisms of UPS remain obscure. In general, STSs are composed of heterogeneous populations of cells with mesenchymal features that can originate from simple genomic alterations or complex genomics. UPS belongs to the latter group, showcasing various potential cellular backgrounds, mutational signatures, and altered signaling pathways. […] The tumorigenesis of UPS is reportedly initiated by a subpopulation of cells called side population (SP) cells, which the Hoechst dye efflux assay can identify. According to an elegant xenograft experimental model, these cells have an increased capacity for self-renewal, growth, proliferation and can recapitulate tumor formation. […] The Hippo pathway may also be implicated in UPS tumor biology, as vestigial-like family member 3 (VGLL3) and yes1-associated transcriptional regulator (YAP1) cofactors were found to be highly amplified on a genome sequencing study.

• #19 Undifferentiated Pleomorphic Sarcoma – MD Searchlight

  https://mdsearchlight.com/cancer/undifferentiated-pleomorphic-sarcoma/

  The exact causes of undifferentiated pleomorphic sarcoma (UPS), a type of cancer, are still unknown. Generally, soft tissue sarcomas (STSs), which include UPS, are made up of different types of cells that can come from simple or complex genetic changes. UPS falls into the category of complex genetic changes, with various types of cells, genetic mutations, and altered signaling pathways involved. […] The development of UPS is thought to start with certain cells called side population (SP) cells. Tests can identify these cells, and researchers believe these cells can grow, multiply, and can form tumors. […] Studies show that two signaling pathways, Hedgehog and Notch, are notably increased at both the cellular and tissue levels. […] The Hippo pathway, another signaling pathway, could also play a role in UPS. Certain genes, VGLL3 and YAP1, were found to be greatly increased in UPS.

• #20 Metastatic Undifferentiated Pleomorphic Sarcoma Causing Intraoperative Stroke

  https://www.annclinlabsci.org/content/43/2/172.full

  Malignant Fibrous Histiocytoma was historically the most commonly diagnosed soft tissue sarcoma of adults. […] Current thoughts about the origin of this tumor are being debated. […] The etiology of UPS is currently being debated, with two schools of thought predominating. The most widely accepted idea is that UPS represents a morphologic pattern that is common to a number of sarcomas as they become increasingly undifferentiated. […] A competing idea is that UPS results from the transformation of mesenchymal stem cells (MSCs), not the loss of differentiation markers from previously differentiated tumors. […] Our case supports the concept that UPS is a morphologic pattern common to various sarcomas as they become increasingly undifferentiated, as evidenced by the change in morphology upon metastasis; however, it has become clear that answering the question of how UPS comes to exist cannot be answered by morphology alone. […] Answering the questions that surround the origin of UPS will continue to involve a combination of morphologic, immunohistochemical, and molecular genetic analysis.

• #21 Metastatic Undifferentiated Pleomorphic Sarcoma Causing Intraoperative Stroke

  https://www.annclinlabsci.org/content/43/2/172.full

  Malignant Fibrous Histiocytoma was historically the most commonly diagnosed soft tissue sarcoma of adults. […] Current thoughts about the origin of this tumor are being debated. […] The etiology of UPS is currently being debated, with two schools of thought predominating. The most widely accepted idea is that UPS represents a morphologic pattern that is common to a number of sarcomas as they become increasingly undifferentiated. […] A competing idea is that UPS results from the transformation of mesenchymal stem cells (MSCs), not the loss of differentiation markers from previously differentiated tumors. […] Our case supports the concept that UPS is a morphologic pattern common to various sarcomas as they become increasingly undifferentiated, as evidenced by the change in morphology upon metastasis; however, it has become clear that answering the question of how UPS comes to exist cannot be answered by morphology alone. […] Answering the questions that surround the origin of UPS will continue to involve a combination of morphologic, immunohistochemical, and molecular genetic analysis.

• #22 Undifferentiated Pleomorphic Sarcoma – PubMed

  https://pubmed.ncbi.nlm.nih.gov/34033374/

  The undifferentiated pleomorphic sarcoma (UPS) formerly known as malignant fibrous histiocytoma, is a high-grade aggressive soft-tissue sarcoma (STS). Mesenchymal stem cells are the most likely origin of the tumor, instead of histiocytes as previously thought. […] At present, UPS is diagnosed by excluding other well-classified STSs.

• #23 Undifferentiated pleomorphic sarcoma – Wikipedia

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

  Causes Unknown […] Studies strongly suggest that MFH tumors are not derived from histiocytes (cells descended from blood monocytes), but rather from mesenchymal cells. […] UPS had also been regarded as a more aggressive and metastasizing form of the low-grade myofibroblastic sarcomas and intermediate-grade myofibroblastic sarcomas. […] UPS tumors also show gene and chromosome abnormalities that further studies may find contribute to the development and/or progression of UPS. […] These abnormalities, which have not yet been reported to be helpful in diagnosing UPS, include the following. […] Further studies are needed to define the best treatments for UPS tumors.

• #24 Orphanet: Undifferentiated pleomorphic sarcoma

  https://www.orpha.net/en/disease/detail/2023

  An aggressive sarcoma of soft tissues or bone that can arise from any part of the body, clinically presenting as swelling, mass, pain, pathological fracture and occasional systemic features and is characterized by high local recurrence and significant metastasis. […] The etiology of the tumor remains unknown. Prior radiation therapy is a likely risk factor in some cases. […] UPS is thought to be derived from a primitive mesenchymal cell capable of differentiating into histiocytes, fibroblasts, myofibroblasts and osteoclasts.

• #25 Undifferentiated Pleomorphic Sarcoma: Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/22435-undifferentiated-pleomorphic-sarcoma

  Undifferentiated pleomorphic sarcoma causes […] Experts arent sure what causes undifferentiated pleomorphic sarcoma. They know it happens when healthy cells develop changes in their DNA, but they dont know what causes those changes. […] Most people who develop UPS dont have any known risk factors.

• #26 Undifferentiated Pleomorphic Sarcoma: Causes, Symptoms and Treatment | Apollo Hospitals

  https://www.apollohospitals.com/diseases-and-conditions/undifferentiated-pleomorphic-sarcoma-causes-symptoms-and-treatment

  The cause behind developing undifferentiated pleomorphic sarcoma is not yet known. However, there are certain factors that may increase your risk of developing this condition. These are: […] Many people who develop undifferentiated pleomorphic sarcoma have no known risk factors, and many people who have risk factors never develop cancer.

• #27 Undifferentiated Pleomorphic Sarcoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/112229

  In various other reports, there are well-documented loss or deletion mutations of phosphatase and tensin homolog (PTEN) and overexpression of phosphorylated protein kinase B (pAKT) in UPS, involved in the PIK3/PTEN/AKT/mTOR pathway. […] Similarly, Dickkopf-related protein 1 (DKK1), a Wnt/B-catenin signaling pathway inhibitor, was differentially overexpressed in UPS compared to other STSs. […] Mutations in tumor protein 53 (TP53), cyclin-dependent kinase inhibitor 2A (CDKN2A), retinoblastoma-associated protein (RB1), and transcriptional regulator ATRX (ATRX) genes, as well as PR domain zinc finger protein 10 (PRDM10) and triple functional domain protein (TRIO) gene fusions have also been identified in UPS. […] Besides genetic aberrations, radiation therapy (RT) is a known risk factor for STS development. Indeed, radiation-associated STS presents in around 1% to 3% of subjects diagnosed with any sarcoma. Specifically, in a series of 1068 UPS cases, 5.1% of the patients had a prior history of radiation.

• #28 Undifferentiated Pleomorphic Sarcoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/112229

  In various other reports, there are well-documented loss or deletion mutations of phosphatase and tensin homolog (PTEN) and overexpression of phosphorylated protein kinase B (pAKT) in UPS, involved in the PIK3/PTEN/AKT/mTOR pathway. […] Similarly, Dickkopf-related protein 1 (DKK1), a Wnt/B-catenin signaling pathway inhibitor, was differentially overexpressed in UPS compared to other STSs. […] Mutations in tumor protein 53 (TP53), cyclin-dependent kinase inhibitor 2A (CDKN2A), retinoblastoma-associated protein (RB1), and transcriptional regulator ATRX (ATRX) genes, as well as PR domain zinc finger protein 10 (PRDM10) and triple functional domain protein (TRIO) gene fusions have also been identified in UPS. […] Besides genetic aberrations, radiation therapy (RT) is a known risk factor for STS development. Indeed, radiation-associated STS presents in around 1% to 3% of subjects diagnosed with any sarcoma. Specifically, in a series of 1068 UPS cases, 5.1% of the patients had a prior history of radiation.

• #29 Undifferentiated Pleomorphic Sarcoma: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/undifferentiated-pleomorphic-sarcoma-causes-symptoms-and-treatment?srsltid=AfmBOorXGSJuAVC7BqYaDexA8qQXlb0-teGNRTXaeHOzWB2a6DV3vKr3

  While the exact cause of Undifferentiated pleomorphic sarcoma is still unknown, several scientific studies indicate that it chiefly arises when there are certain mutations in the genes of the soft tissue cells, that stimulates them to grow abnormally in size and number without dying on time like the regular ones and causing them to amass leading to the growth of tumorous structures. […] Some studies also claim that radiation therapy that is used to kill a different form of cancer might lead to soft tissue sarcoma in the same part. […] Certain causative factors that aggravate the chances of Undifferentiated pleomorphic sarcoma include: […] People being near radiation or those undergoing radiation therapy for some other types of cancer are more at risk of getting Undifferentiated pleomorphic sarcoma sarcoma.

• #30 Undifferentiated Pleomorphic Sarcoma – SFA

  https://curesarcoma.org/sarcoma-subtypes/undifferentiated-pleomorphic-sarcoma/

  The etiology of primary UPS of bone is unknown. […] Secondary UPS, representing approximately 28% of cases, arises in association with a pre-existing bone condition or disease. […] The majority of secondary UPSs are associated with a bone infarct, Paget disease, or prior irradiation in the field of the affected bone. […] Rare examples of UPS of bone occur at the site of a metallic orthopedic prosthesis or hardware. […] Diaphyseal medullary stenosis, a rare autosomal dominant bone dysplasia, is another less common setting associated with UPS of bone. […] This disorder is caused by mutations in the gene encoding methylthioadenosine phosphorylase, MTAP.

• #31 Undifferentiated Pleomorphic Sarcoma – SFA

  https://curesarcoma.org/sarcoma-subtypes/undifferentiated-pleomorphic-sarcoma/

  The etiology of primary UPS of bone is unknown. […] Secondary UPS, representing approximately 28% of cases, arises in association with a pre-existing bone condition or disease. […] The majority of secondary UPSs are associated with a bone infarct, Paget disease, or prior irradiation in the field of the affected bone. […] Rare examples of UPS of bone occur at the site of a metallic orthopedic prosthesis or hardware. […] Diaphyseal medullary stenosis, a rare autosomal dominant bone dysplasia, is another less common setting associated with UPS of bone. […] This disorder is caused by mutations in the gene encoding methylthioadenosine phosphorylase, MTAP.

• #32 Undifferentiated pleomorphic sarcoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/undifferentiated-pleomorphic-sarcoma-1?lang=us

  Undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma (MFH), is considered the most common type of soft tissue sarcoma. It has an aggressive biological behavior and a poor prognosis. […] They are the most frequent soft tissue sarcoma to occur as a result of radiotherapy and are also seen on a background of Paget disease. Secondary transformation into malignant sarcomas (including undifferentiated pleomorphic sarcoma) has also been reported in fibrous dysplasia, giant cell tumor, enchondroma, chronic osteomyelitis, and osteonecrosis. […] Most undifferentiated pleomorphic sarcomas are high grade (3 and 4) and are aggressive in their biological behavior. They frequently metastasize (30-50% at diagnosis) and locally recur despite aggressive treatment.

• #33 Undifferentiated Pleomorph – Types of Sarcoma Cancer

  https://www.leiomyosarcoma.org/undifferentiated-pleomorph/

  Unfortunately, no one knows for sure what caused undifferentiated pleomorphic sarcoma. […] What originally causes the proliferation of cells that eventually become cancerous is a mystery. […] There are a number of risk factors that scientists have identified. They are: Genetic: This doesn’t mean that undifferentiated pleomorphic sarcoma is inherited, rather that genetic abnormalities could be causing the cancer. […] By far the most established of the risk factors is radiation therapy when used to address soft tissue sarcomas. Those who receive radiation to address their cancer are at risk of developing undifferentiated pleomorphic sarcoma. […] Another risk factor pending proof is certain chemicals. Doctors believe that exposure to things like arsenic, wood preservatives (thanks to their chlorophenols), vinyl chloride and certain herbicides may trigger the growth of undifferentiated pleomorphic sarcoma.

• #34 Undifferentiated Pleomorphic Sarcoma: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/undifferentiated-pleomorphic-sarcoma-causes-symptoms-and-treatment?srsltid=AfmBOorXGSJuAVC7BqYaDexA8qQXlb0-teGNRTXaeHOzWB2a6DV3vKr3

  Exposure to carcinogenic compounds and other harmful chemical substances daily can make a person prone to this type of cancer. […] People who have had a history of other cancerous conditions have increased incidence of Undifferentiated pleomorphic sarcoma. […] Having a depreciated immunity due to HIV or AIDS increases the risk of Undifferentiated pleomorphic sarcoma.

• #35 Risk Factors for Soft Tissue Sarcomas | American Cancer Society

  https://www.cancer.org/cancer/types/soft-tissue-sarcoma/causes-risks-prevention/risk-factors.html

  People with tuberous sclerosis have an increased risk of rhabdomyosarcoma. […] Exposure to vinyl chloride (a chemical used in making plastics) is a risk factor for developing sarcomas of the liver, but it hasn’t been proven to cause soft tissue sarcomas. Arsenic has also been linked to a type of liver sarcoma but not soft tissue sarcoma. Exposure to dioxin and to herbicides that contain phenoxyacetic acid at high doses may also be risk factors, but this isn’t known for certain.

• #36 Risk Factors for Soft Tissue Sarcomas | American Cancer Society

  https://www.cancer.org/cancer/types/soft-tissue-sarcoma/causes-risks-prevention/risk-factors.html

  Radiation exposure accounts for less than 5% of sarcomas. But patients might develop sarcomas from radiation given to treat other cancers, like breast cancer or lymphoma. The sarcoma often starts in the part of the body that was treated with radiation. The average time between the radiation treatments and the diagnosis of a sarcoma is about 10 years. […] Family cancer syndromes are disorders caused by gene defects (mutations) that people are born with (often inherited from a parent) that are linked to a high risk of getting certain cancers. Some family cancer syndromes increase a person’s risk of developing soft tissue sarcomas. […] Neurofibromatosis is also known as von Recklinghausen disease. It usually runs in families and causes many benign (not cancer) tumors that form in nerves under the skin and in other parts of the body (These are called neurofibromas.) It’s caused by a defect (mutation) in genes called NF1 and NF2. About 5% of people with neurofibromatosis will develop a sarcoma in a neurofibroma.

• #37 About Soft Tissue Sarcoma | Cancer Council NSW

  https://www.cancercouncil.com.au/soft-tissue-sarcoma/about-soft-tissue-sarcoma/

  The causes of most sarcomas are not known. However, there are several risk factors: […] Some rare, inherited conditions can put people more at risk of soft tissue sarcoma. These rare, genetic conditions include von Recklinghausen disease (also known as neurofibromatosis or NF), Li–Fraumeni syndrome (also known as p53) and retinoblastoma (also known as Rb). […] Some sarcomas may be linked to being exposed to chemicals including vinyl chloride (used to make plastic) and some high-dose herbicides (weedkillers). […] Long-term lymphoedema in the body, for example in the legs or arms (swelling from a build-up of lymph fluid) has been linked with angiosarcoma.

• #38 Risk Factors for Soft Tissue Sarcomas | American Cancer Society

  https://www.cancer.org/cancer/types/soft-tissue-sarcoma/causes-risks-prevention/risk-factors.html

  Li-Fraumeni syndrome is caused by inherited defects in the TP53 gene. People affected by this syndrome have a high risk of cancer, such as breast cancer, brain tumors, leukemia, and sarcomas. Still, only 10 to 20 out of 100 people with Li-Fraumeni syndrome will develop a soft tissue sarcoma. […] Children with this gene defect also have a higher risk of developing bone or soft tissue sarcomas, especially if the retinoblastoma was treated with radiation. […] Werner syndrome is caused by defects in the RECQL2 gene. Children with this syndrome have problems like those seen in the elderly. They also have an increased risk of cancer, including soft tissue sarcomas. […] Gorlin syndrome is also called nevoid basal cell carcinoma syndrome (NBCCS). It’s caused by defects in the PTCH1 gene. People with this syndrome have a high risk of developing many basal cell skin cancers. They also have an increased risk of fibrosarcoma and rhabdomyosarcoma.

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

  https://www.netmeds.com/health-library/post/soft-tissue-sarcoma-causes-symptoms-and-treatment?srsltid=AfmBOorGwg7f7OpaaM88QwKUJ8FSKYygs_xfJFeWNX8ddGVyUe_cF-om

  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.

• #40 Malignant fibrous histiocytoma of the bone

  https://www.medicalnewstoday.com/articles/malignant-fibrous-histiocytoma-of-bone

  The exact causes and risk factors of UPS are not known. […] However, research suggests that people over the age of 50 and those who have had radiation therapy in the affected area in the past may be at a higher risk of developing UPS. […] Initially, researchers thought the cancer started in histiocytes a type of immune cell. In fact, it originates from mesenchymal cells, which are important for repairing cartilage, bone, and other types of skeletal tissue.

• #41 Undifferentiated Pleomorphic Sarcoma (UPS) – Rare Cancers Australia

  https://www.rarecancers.org.au/knowledgebase/cancer-types/undifferentiated-pleomorphic-sarcoma-ups/

  Undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma (MFH), is a common type of sarcoma, which are cancers that develop in bone or soft tissue. […] While the cause of UPS remains unknown, the following factors may increase the likelihood of developing the disease: Older age. Previous radiation therapy. […] Not everyone with these risk factors will develop the disease, and some people who have the disease may have none of these risk factors.

• #42 About Soft Tissue Sarcoma | Cancer Council NSW

  https://www.cancercouncil.com.au/soft-tissue-sarcoma/about-soft-tissue-sarcoma/

  The causes of most sarcomas are not known. However, there are several risk factors: […] Some rare, inherited conditions can put people more at risk of soft tissue sarcoma. These rare, genetic conditions include von Recklinghausen disease (also known as neurofibromatosis or NF), Li–Fraumeni syndrome (also known as p53) and retinoblastoma (also known as Rb). […] Some sarcomas may be linked to being exposed to chemicals including vinyl chloride (used to make plastic) and some high-dose herbicides (weedkillers). […] Long-term lymphoedema in the body, for example in the legs or arms (swelling from a build-up of lymph fluid) has been linked with angiosarcoma.

• #43 Pediatric Undifferentiated Pleomorphic Sarcoma of the Cecum | Ochsner Journal

  https://www.ochsnerjournal.org/content/23/1/77

  Undifferentiated pleomorphic sarcoma (UPS) is a high-grade neoplasm typically diagnosed in older adults and localized to the extremities or retroperitoneum. Because of poor response to therapy and high rates of recurrence, this neoplasm is associated with a poor prognosis. […] Risk factors for the development of UPS include genetics, radiation or chemotherapy exposure, chemical carcinogens, chronic postoperative repair, trauma, surgical incisions, and lymphedema. […] The cellular origins of UPSs are unclear, but they possibly arise from primitive mesenchymal stem cells that retain both fibroblastic and histiocytic potential and may present with markers and behaviors of both cell lines. […] The prognosis for UPS is generally poor because of regional invasiveness, distant metastases, and frequent recurrence.

• #44 Undifferentiated Pleomorphic Sarcoma: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/undifferentiated-pleomorphic-sarcoma-causes-symptoms-and-treatment?srsltid=AfmBOorXGSJuAVC7BqYaDexA8qQXlb0-teGNRTXaeHOzWB2a6DV3vKr3

  Exposure to carcinogenic compounds and other harmful chemical substances daily can make a person prone to this type of cancer. […] People who have had a history of other cancerous conditions have increased incidence of Undifferentiated pleomorphic sarcoma. […] Having a depreciated immunity due to HIV or AIDS increases the risk of Undifferentiated pleomorphic sarcoma.

• #45 Inflammatory Undifferentiated Pleomorphic Sarcoma Mimicking Bacteremia in an Elderly Patient: A Case Report

  https://www.mdpi.com/1648-9144/57/2/175

  Undifferentiated pleomorphic sarcoma (UPS) is major type of soft tissue sarcomas. […] Recently, MFH and UPS associated with inflammation have been reported; however, the details of their pathogenesis remain unknown. […] Approximately 22–65% of MFH cases are CRP positive and show inflammatory infiltrates without infection, as previously described. […] Recently, according to the WHO classification guidelines for STS, the MFH classification was eliminated in 2002 and was replaced with UPS. […] The current case could be further classified as immune-high (D and E), and/or highly vascularized (C) groups. […] Rapid growth and early metastasis have been observed in IUPS, and the involvement of G-CSF, IL-6, IL-7, IL-8, SCF, TGB, and G-CSF have also been suggested. […] In this case, intratumoral infiltration of megakaryocytes and lymphocytes was observed. Thus, it was suggested that inflammatory cytokines might be acting on the tumor microenvironment.

• #46 Undifferentiated pleomorphic sarcoma of skin in unusual locations: Report of two cases | 2021, Volume 32 – Issue 1 | Joint Diseases and Related Surgery

  https://www.jointdrs.org/full-text/1229

  Undifferentiated pleomorphic sarcoma (UPS) of the skin is a rare soft tissue sarcoma subtype with a high risk of metastasis and local recurrence. Ultraviolet exposure plays a prominent role in its etiology. […] The mutation of telomerase reverse transcriptase (TERT) is present in 76% of the skin UPS cases and appears to be associated with the UV damage. […] Thus, the increased incidence in the sun-exposed or UV-damaged skin areas such as head and neck can be explained by TERT mutation. […] On the other hand, the presence of a rapidly growing aggressive lesion on the non-UV-exposed skin areas, particularly in elderly patients, it is recommended to rule out other possible malignancies before to diagnose UPS due to its rarity. […] Both of the reported cases herein were located on a limited sun-exposed region of the body (i.e., the proximal calf and the anterior aspect of the distal cruris), unlike reported in the literature (i.e., the head and neck region).

• #47 Pleomorphic Dermal Sarcoma

  https://dermnetnz.org/topics/pleomorphic-dermal-sarcoma

  PDS has been reported to be caused by ultraviolet (UV) radiation based on its clinical features and genetic studies which have found highly-mutated tumours with recurrent mutations in FAT1, NOTCH1/2, CDKN2A, TP53, and the TERT promoter genes. […] Patients often have a history of previous skin cancers related to sun damage.

• #48 Cardiac undifferentiated pleomorphic sarcoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/cardiac-undifferentiated-pleomorphic-sarcoma?lang=us

  Cardiac undifferentiated pleomorphic sarcomas are highly malignant mesenchymal tumors of the heart. […] Cardiac undifferentiated pleomorphic sarcomas are rare primary malignant tumors of the heart that account for a substantial number of cardiac sarcomas. […] The etiology of cardiac undifferentiated pleomorphic sarcomas is unknown.

• #49 Mouse genetic background influences whether Hras G12V expression plus Cdkn2a knockdown causes angiosarcoma or undifferentiated pleomorphic sarcoma | Oncotarget

  https://www.oncotarget.com/article/24831/text/

  HrasG12V expression plus knockdown of Cdkn2a or Trp53 causes angiosarcomas in SCID/beige mice. […] The genetic alterations responsible for the development of UPS are also incompletely understood. […] Mouse studies have confirmed that the cooperation of oncogenic Kras and Trp53 or Cdkn2a deficiency resulted in the development of undifferentiated pleomorphic sarcomas in different tissues. […] HrasG12V expression plus knockdown of Cdkn2a causes angiosarcomas and undifferentiated pleomorphic sarcomas in 129Sv mice. […] Given the absence of morphologic features of any cellular lineage, the absence of clear evidence for positivity of tumour cells for a panel of lineage markers and absence of strong and diffuse pan-CYTOKERATIN staining we therefore favour the diagnosis of these tumours as high-grade UPS Not Otherwise Specified in keeping with WHO diagnostic guidelines.

• #50 Mouse genetic background influences whether Hras G12V expression plus Cdkn2a knockdown causes angiosarcoma or undifferentiated pleomorphic sarcoma | Oncotarget

  https://www.oncotarget.com/article/24831/text/

  Importantly, these analyses show that 129/Sv mice develop two different types of tumours, angiosarcomas and UPS, in response to the same oncogenic stimulus. […] Our data demonstrate that the same combination of genetic drivers can cause different types of tumours based not only on the site of viral delivery, likely due to infection of different cell types, but also based on the genetic background of the mouse strain. […] The cell of origin of UPS remains unknown and it is not clear whether UPS represent a group of de-differentiated sarcomas which share a common morphology but originated from different cell types or if all UPS tumours arise from a common cell of origin. […] These new experimental models will facilitate future pre-clinical studies for establishing new therapeutic interventions for these aggressive malignancies.

• #51 Frontiers | Undifferentiated Pleomorphic Sarcoma and the Importance of Considering the Oncogenic and Immune-Suppressant Role of the Human T-Cell Lymphotropic Virus Type 1: A Case Report

  https://www.frontiersin.org/journals/oncology/articles/10.3389/fonc.2017.00091/full

  Human T-cell lymphotropic virus type 1 (HTLV-1) is a human retrovirus, classified as group 1 human carcinogens by The International Agency for Research on Cancer, that causes an aggressive malignancy known as adult T-cell lymphoma/leukemia and a progressive chronic inflammatory neurological disease named HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). […] HTLV-1 causes accumulation of genetic mutations in the host genome that could contribute to cellular transformation, one of the oncogenic features of HTLV-1. […] Considering undifferentiated pleomorphic sarcoma (UPS), two missense mutations in the KRAS proto-oncogene and in PIK3CA have been recently identified by gene mutation screening. […] Human T-cell lymphotropic virus type 1 is a human retrovirus capable of causing cancer, and it has been recently classified as group 1 human carcinogens by The IARC.

• #52 Frontiers | Undifferentiated Pleomorphic Sarcoma and the Importance of Considering the Oncogenic and Immune-Suppressant Role of the Human T-Cell Lymphotropic Virus Type 1: A Case Report

  https://www.frontiersin.org/journals/oncology/articles/10.3389/fonc.2017.00091/full

  Human T-cell lymphotropic virus type 1 (HTLV-1) is a human retrovirus, classified as group 1 human carcinogens by The International Agency for Research on Cancer, that causes an aggressive malignancy known as adult T-cell lymphoma/leukemia and a progressive chronic inflammatory neurological disease named HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). […] HTLV-1 causes accumulation of genetic mutations in the host genome that could contribute to cellular transformation, one of the oncogenic features of HTLV-1. […] Considering undifferentiated pleomorphic sarcoma (UPS), two missense mutations in the KRAS proto-oncogene and in PIK3CA have been recently identified by gene mutation screening. […] Human T-cell lymphotropic virus type 1 is a human retrovirus capable of causing cancer, and it has been recently classified as group 1 human carcinogens by The IARC.

• #53 Frontiers | Undifferentiated Pleomorphic Sarcoma and the Importance of Considering the Oncogenic and Immune-Suppressant Role of the Human T-Cell Lymphotropic Virus Type 1: A Case Report

  https://www.frontiersin.org/journals/oncology/articles/10.3389/fonc.2017.00091/full

  In this case, the patient had no laboratory results or symptoms related to an immunocompromised status even though it should not be discarded due to HTLV-1 infection which could contribute to UPS oncogenesis by both, direct and indirect mechanisms. […] Given the high grade of UPS and the progressive invalidating myelopathy caused by HTLV-1, the treatment should be carefully evaluated to positively impact on the patient’s life expectancy.

• #54 Analysis of prognostic factors of undifferentiated pleomorphic sarcoma and construction and validation of a prediction nomogram based on SEER database | European Journal of Medical Research | Full Text

  https://eurjmedres.biomedcentral.com/articles/10.1186/s40001-022-00810-z

  The prognostic factors with statistical differences in univariate Cox regression analysis were further analyzed by multivariate Cox regression analysis, and the results determined the independent influencing factors of age, tumor site, tumor size, tumor depth, N stage, M stage, tumor grade, surgery and radiotherapy. […] Age, tumor site, tumor size, tumor depth, N stage, M stage, Grade, surgery and radiotherapy are independent prognostic factors for patients with undifferentiated pleomorphic sarcoma. […] Among them, surgery and radiotherapy can improve the prognosis of patients to a certain extent. […] No effect of sex, race and chemotherapy on prognosis was found.

• #55 Undifferentiated pleomorphic sarcoma of skin in unusual locations: Report of two cases | 2021, Volume 32 – Issue 1 | Joint Diseases and Related Surgery

  https://www.jointdrs.org/full-text/1229

  A positive surgical margin is considered to be the most important prognostic factor due to the inverse relation with disease-free survival. […] Additionally, some other factors can cause difficulty to achieve a reliable, long-term follow-up for these patients such as being elderly, presence of systemic comorbidities related to mortality, and presence of immunosuppression as an etiological factor due to the short life expectancy. […] In conclusion, malignancy potential of a rapidly growing skin lesion should be taken into the consideration, and the treatment should be planned after meticulous radiological and histological examination.

• #56 Analysis of prognostic factors of undifferentiated pleomorphic sarcoma and construction and validation of a prediction nomogram based on SEER database | European Journal of Medical Research | Full Text

  https://eurjmedres.biomedcentral.com/articles/10.1186/s40001-022-00810-z

  The prognostic factors with statistical differences in univariate Cox regression analysis were further analyzed by multivariate Cox regression analysis, and the results determined the independent influencing factors of age, tumor site, tumor size, tumor depth, N stage, M stage, tumor grade, surgery and radiotherapy. […] Age, tumor site, tumor size, tumor depth, N stage, M stage, Grade, surgery and radiotherapy are independent prognostic factors for patients with undifferentiated pleomorphic sarcoma. […] Among them, surgery and radiotherapy can improve the prognosis of patients to a certain extent. […] No effect of sex, race and chemotherapy on prognosis was found.

• #57 Mayo Clinic Health Library – Undifferentiated pleomorphic sarcoma | Swiss Medical Network

  https://www.swissmedical.net/en/healtcare-library/con-20344525

  It’s not clear what causes undifferentiated pleomorphic sarcoma. […] Doctors know this cancer begins when a cell develops changes in its DNA. A cell’s DNA contains the instructions that tell a cell what to do. The changes tell the cell to multiply rapidly, creating a mass of abnormal cells (tumor). The cells can invade and destroy nearby healthy tissue. In time, the cancer cells can break away and spread (metastasize) to other parts of the body, such as the lungs and bones.

• #58 Undifferentiated Pleomorphic Sarcoma: Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/22435-undifferentiated-pleomorphic-sarcoma

  Undifferentiated pleomorphic sarcoma causes […] Experts arent sure what causes undifferentiated pleomorphic sarcoma. They know it happens when healthy cells develop changes in their DNA, but they dont know what causes those changes. […] Most people who develop UPS dont have any known risk factors.

• #59 Targeting Tumor Initiating Cell in Undifferentiated Pleomorphic Sarcoma – Benjamin Alman

  https://grantome.com/grant/NIH/R01-CA183811-03

  Undifferentiated pleomorphic sarcoma (UPS) is a soft tissue sarcoma, with one of the worst prognosis. […] Our hypothesis is that the TIC fraction in UPS is responsible for maintaining UPS self-renewal and tumor growth, and that therapeutically targeting the TIC fraction can be developed into an effective UPS treatment. […] Undifferentiated pleomorphic sarcoma (previously called malignant fibrous histiocytoma) is the soft tissue sarcoma with one of the worst prognosis. 50% of afflicted individuals will have a recurrence or develop metastatic disease within two years using conventional treatment approaches, and most of these patients will succumb to their disease. As such, novel approaches to therapy are urgently needed. […] Here we identified an innovative approach to treatment based on eradiating a small population of tumor cells that maintain tumor growth over time.

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