Materiały źródłowe

• #1 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. […] Undifferentiated pleomorphic sarcomas are aggressive tumors that account for 25-40% of all adult soft tissue sarcomas, making them the most common type. However, the classification system is becoming more restrictive, with many tumors being reclassified as variants of myogenic sarcomas. […] 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. The overall 5-year survival is between 25-70%.

• #2 A Detailed Examination of Retroperitoneal Undifferentiated Pleomorphic Sarcoma: A Case Report and Review of the Existing Literature

  https://www.mdpi.com/2077-0383/13/13/3684

  Undifferentiated pleomorphic sarcoma (UPS), formerly known as malignant fibrous histiocytoma (MFH), is a rare and aggressive type of soft-tissue sarcoma (STS) that represents approximately 1–2% of all solid tumors. […] Retroperitoneal UPS is an oncologically complex pathology due to its infrequent manifestation and aggressive nature, which complicates both diagnosis and management. […] The purpose of this article is to extend the existing literature by documenting new cases of retroperitoneal UPS, reviewing all known cases, and discussing broad aspects of the disease, including its epidemiology, pathogenesis, and current treatment strategies. […] The molecular pathogenesis of UPS is driven by a complex array of genetic and epigenetic changes that influence tumor behavior including proliferation, invasion, and migration.

• #3 Undifferentiated Pleomorphic Sarcoma: A Case Report

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11632629/

  Undifferentiated pleomorphic sarcoma (UPS) is an uncommon and aggressive soft tissue tumor primarily affecting older adults, often presenting as a rapidly enlarging, painless mass in the extremities or other deep-seated locations. […] The exact aetiology and pathogenesis of undifferentiated pleomorphic sarcomas remain undetermined. […] Minor trauma triggering the development of soft tissue sarcomas is rare, and the association between these events is not well understood. […] In this patient’s case, the association of trauma with the development of Undifferentiated Pleomorphic Sarcoma (UPS) remains challenging to establish definitively, though there are some theoretical and experimental underpinnings that suggest trauma might act as a promoter for sarcoma formation under certain conditions.

• #4 Undifferentiated high-grade pleomorphic sarcoma of the colon: a rare case report and literature review | BMC Gastroenterology | Full Text

  https://bmcgastroenterol.biomedcentral.com/articles/10.1186/s12876-022-02189-x

  Undifferentiated pleomorphic sarcoma (UPS), also known as malignant fibrous histiocytoma (MFH), hardly originates from the colorectum. […] Currently, the pathogenesis of UPS is not completely understood. However, it has been proposed that some predisposing factors are involved in the occurrence of UPS, including genetic abnormalities, chemoradiotherapy stimulation, chronic irritation, and lymphedema. […] UPS in the colorectum is a rare malignant tumor with a poor prognosis and unknown pathogenesis. […] UPS in the colorectum is a rare malignant tumor with a poor prognosis and unknown pathogenesis. Nearly half of the patients with UPS died of postoperative recurrences or metastasis.

• #5 A Detailed Examination of Retroperitoneal Undifferentiated Pleomorphic Sarcoma: A Case Report and Review of the Existing Literature

  https://www.mdpi.com/2077-0383/13/13/3684

  Undifferentiated pleomorphic sarcoma (UPS), formerly known as malignant fibrous histiocytoma (MFH), is a rare and aggressive type of soft-tissue sarcoma (STS) that represents approximately 1–2% of all solid tumors. […] Retroperitoneal UPS is an oncologically complex pathology due to its infrequent manifestation and aggressive nature, which complicates both diagnosis and management. […] The purpose of this article is to extend the existing literature by documenting new cases of retroperitoneal UPS, reviewing all known cases, and discussing broad aspects of the disease, including its epidemiology, pathogenesis, and current treatment strategies. […] The molecular pathogenesis of UPS is driven by a complex array of genetic and epigenetic changes that influence tumor behavior including proliferation, invasion, and migration.

• #6 Undifferentiated pleomorphic sarcoma – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/undifferentiated-pleomorphic-sarcoma/symptoms-causes/syc-20389554

  Undifferentiated pleomorphic sarcoma (UPS) is a rare type of cancer that begins mostly in the soft tissues of the body. […] 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.

• #7

  https://www.sydneysarcomaunit.com.au/undifferentiated-pleomorphic-sarcoma

  The cause of undifferentiated pleomorphic sarcoma is unknown. […] Doctors understand that cancer starts when a cell’s DNA changes. The DNA of a cell includes the instructions that tell it what to do. The changes cause the cell to grow rapidly, resulting in a swarm of abnormal cells (tumour). […] The cells have the ability to penetrate and destroy healthy tissue nearby. Cancer cells can break away and spread to other regions of the body, such as the lungs and bones, over time.

• #8 Undifferentiated Pleomorphic Sarcoma (Malignant Fibrous Histiocytoma) | Ento Key

  https://entokey.com/undifferentiated-pleomorphic-sarcoma-malignant-fibrous-histiocytoma/

  Undifferentiated pleomorphic sarcoma (UPS) describes a soft tissue tumor of primitive mesenchymal origin that involves deep skeletal muscle, fascia, and adipose tissue. […] The pathogenesis is unclear, but translocations and gene fusions common in the pathogenesis of other sarcomas have not been found to play a role in the pathogenesis of UPS. […] A well-known etiologic factor for UPS is ionizing radiation, and this has been reported following radiotherapy for breast carcinoma, malignant lymphoma, cervical carcinoma, and brain tumors. […] Interestingly, a variety of translocations and gene fusions common in the pathogenesis of other sarcomas have not been found to play a role in the pathogenesis of UPS.

• #9 Undifferentiated Sarcoma – SFA

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

  Undifferentiated pleomorphic sarcomas (UPSs) typically display extensive genomic rearrangements. Cytogenetic data from over 100 cases have revealed complex karyotypes with chromosome numbers ranging from near-haploid to hyperoctaploid. The extensive reshuffling of chromosomal material indicated by tumor karyotypes is corroborated by array-based genomic studies, showing structural rearrangements and copy-number shifts involving most if not all chromosomes, as well as by massively parallel sequencing studies. UPSs demonstrate substantial copy-number heterogeneity compared with other sarcoma subtypes and do not form distinct methylation subgroups. Whole-genome sequencing has revealed that punctuated evolutionary events such as whole-genome duplications and chromothripsis accompanied by telomere dysfunction underpin the genomic complexity of UPS. The aberrations in telomere biology are achieved either through activation of telomerase through deregulation of TERT or through the alternative lengthening of telomeres pathway. There are no tumor-specific amplicons, but some 10–15% of cases show amplification of VGLL3 or YAP1, and 10% have amplification of CCNE1. Targets of frequent deletions and disruptive rearrangements include CDKN2A and CDKN2B in 9p, PTEN in 10q, RB1 in 13q, and TP53 in 17p, each affected in 10–20% of cases. RB1 and TP53, as well as ATRX, are among the few genes that recurrently show pathogenetic variants at the nucleotide level. At the transcriptomic level, UPS cannot be distinguished from high-grade myxofibrosarcoma, and it is possible that these two entities may lie on a spectrum of differentiation. A small subset of UPSs with low mitotic counts show gene fusions involving the PRDM10 gene, with either MED12 or CITED2 as the 5′ partner. Otherwise, driver gene fusions have not been found in UPS.

• #10 Undifferentiated pleomorphic sarcoma – Wikipedia

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

  UPS tumors also show gene and chromosome abnormalities that further studies may find contribute to the development and/or progression of UPS. […] Deletion and/or inactivation or the RB1 gene that encodes (i.e. is responsible for production of) the retinoblastoma protein that functions as a tumor suppressor protein; deletions and/or mutations in the TP53 gene that encodes tumor protein P53 (a protein which regulates cell proliferation and cell death); mutations in the ATRX gene that encodes transcriptional regulator ATRX protein which contributes to regulating the expression of various genes; mutations in the KMT2C gene which encodes lysine N-methyltransferase 2C protein (the KMT2C gene is mutated in various cancer types); amplification of the IL7R gene which encodes Interleukin-7 receptor- protein (mutations in the IL7R gene are commonly found in acute lymphoblastic leukemia) and expression of a fusion gene (i.e. a hybrid gene formed from two previously independent genes as a result of a mutation) that merges TRIO with other genes and is often found in other sarcoma subtypes.

• #11 Undifferentiated pleomorphic sarcoma – Wikipedia

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

  UPS tumors also show gene and chromosome abnormalities that further studies may find contribute to the development and/or progression of UPS. […] Deletion and/or inactivation or the RB1 gene that encodes (i.e. is responsible for production of) the retinoblastoma protein that functions as a tumor suppressor protein; deletions and/or mutations in the TP53 gene that encodes tumor protein P53 (a protein which regulates cell proliferation and cell death); mutations in the ATRX gene that encodes transcriptional regulator ATRX protein which contributes to regulating the expression of various genes; mutations in the KMT2C gene which encodes lysine N-methyltransferase 2C protein (the KMT2C gene is mutated in various cancer types); amplification of the IL7R gene which encodes Interleukin-7 receptor- protein (mutations in the IL7R gene are commonly found in acute lymphoblastic leukemia) and expression of a fusion gene (i.e. a hybrid gene formed from two previously independent genes as a result of a mutation) that merges TRIO with other genes and is often found in other sarcoma subtypes.

• #12 Undifferentiated pleomorphic sarcoma – Wikipedia

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

  UPS tumors also show gene and chromosome abnormalities that further studies may find contribute to the development and/or progression of UPS. […] Deletion and/or inactivation or the RB1 gene that encodes (i.e. is responsible for production of) the retinoblastoma protein that functions as a tumor suppressor protein; deletions and/or mutations in the TP53 gene that encodes tumor protein P53 (a protein which regulates cell proliferation and cell death); mutations in the ATRX gene that encodes transcriptional regulator ATRX protein which contributes to regulating the expression of various genes; mutations in the KMT2C gene which encodes lysine N-methyltransferase 2C protein (the KMT2C gene is mutated in various cancer types); amplification of the IL7R gene which encodes Interleukin-7 receptor- protein (mutations in the IL7R gene are commonly found in acute lymphoblastic leukemia) and expression of a fusion gene (i.e. a hybrid gene formed from two previously independent genes as a result of a mutation) that merges TRIO with other genes and is often found in other sarcoma subtypes.

• #13 A Detailed Examination of Retroperitoneal Undifferentiated Pleomorphic Sarcoma: A Case Report and Review of the Existing Literature

  https://www.mdpi.com/2077-0383/13/13/3684

  Notably, a range of “cancer driver genes” such as tumor protein p53 (TP53), alpha-thalassemia/mental retardation syndrome X-linked (ATRX), H3 histone family member 3A (H3F3A), and zinc finger homeobox 3 (ZFHX3), among others, have been identified as critical to the onset and advancement of the disease. […] The inactivation of tumor suppressor genes is a frequent occurrence, with mutations in TP53 leading to the overexpression of p53. […] Additionally, the role of cellular senescence in UPS is highlighted by the homozygous deletion of cyclin-dependent kinase inhibitor 2A (p16INK4a), although it appears that p16 may not act as a predominant barrier to senescence in some UPS subtypes. […] From an epigenetic perspective, significant modifications include the methylation of sites around integrin subunit alpha 10 (ITGA10) and protein phosphatase 2 regulatory subunit B (PPP2R2B), which are upstream regulators of the Akt/mTOR (protein kinase B/mammalian target of rapamycin) signaling pathway.

• #14 Undifferentiated pleomorphic sarcoma – Wikipedia

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

  UPS tumors also show gene and chromosome abnormalities that further studies may find contribute to the development and/or progression of UPS. […] Deletion and/or inactivation or the RB1 gene that encodes (i.e. is responsible for production of) the retinoblastoma protein that functions as a tumor suppressor protein; deletions and/or mutations in the TP53 gene that encodes tumor protein P53 (a protein which regulates cell proliferation and cell death); mutations in the ATRX gene that encodes transcriptional regulator ATRX protein which contributes to regulating the expression of various genes; mutations in the KMT2C gene which encodes lysine N-methyltransferase 2C protein (the KMT2C gene is mutated in various cancer types); amplification of the IL7R gene which encodes Interleukin-7 receptor- protein (mutations in the IL7R gene are commonly found in acute lymphoblastic leukemia) and expression of a fusion gene (i.e. a hybrid gene formed from two previously independent genes as a result of a mutation) that merges TRIO with other genes and is often found in other sarcoma subtypes.

• #15 A Detailed Examination of Retroperitoneal Undifferentiated Pleomorphic Sarcoma: A Case Report and Review of the Existing Literature

  https://www.mdpi.com/2077-0383/13/13/3684

  Notably, a range of “cancer driver genes” such as tumor protein p53 (TP53), alpha-thalassemia/mental retardation syndrome X-linked (ATRX), H3 histone family member 3A (H3F3A), and zinc finger homeobox 3 (ZFHX3), among others, have been identified as critical to the onset and advancement of the disease. […] The inactivation of tumor suppressor genes is a frequent occurrence, with mutations in TP53 leading to the overexpression of p53. […] Additionally, the role of cellular senescence in UPS is highlighted by the homozygous deletion of cyclin-dependent kinase inhibitor 2A (p16INK4a), although it appears that p16 may not act as a predominant barrier to senescence in some UPS subtypes. […] From an epigenetic perspective, significant modifications include the methylation of sites around integrin subunit alpha 10 (ITGA10) and protein phosphatase 2 regulatory subunit B (PPP2R2B), which are upstream regulators of the Akt/mTOR (protein kinase B/mammalian target of rapamycin) signaling pathway.

• #16 Undifferentiated pleomorphic sarcoma – Wikipedia

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

  UPS tumors also show gene and chromosome abnormalities that further studies may find contribute to the development and/or progression of UPS. […] Deletion and/or inactivation or the RB1 gene that encodes (i.e. is responsible for production of) the retinoblastoma protein that functions as a tumor suppressor protein; deletions and/or mutations in the TP53 gene that encodes tumor protein P53 (a protein which regulates cell proliferation and cell death); mutations in the ATRX gene that encodes transcriptional regulator ATRX protein which contributes to regulating the expression of various genes; mutations in the KMT2C gene which encodes lysine N-methyltransferase 2C protein (the KMT2C gene is mutated in various cancer types); amplification of the IL7R gene which encodes Interleukin-7 receptor- protein (mutations in the IL7R gene are commonly found in acute lymphoblastic leukemia) and expression of a fusion gene (i.e. a hybrid gene formed from two previously independent genes as a result of a mutation) that merges TRIO with other genes and is often found in other sarcoma subtypes.

• #17 Undifferentiated pleomorphic sarcoma – Wikipedia

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

  UPS tumors also show gene and chromosome abnormalities that further studies may find contribute to the development and/or progression of UPS. […] Deletion and/or inactivation or the RB1 gene that encodes (i.e. is responsible for production of) the retinoblastoma protein that functions as a tumor suppressor protein; deletions and/or mutations in the TP53 gene that encodes tumor protein P53 (a protein which regulates cell proliferation and cell death); mutations in the ATRX gene that encodes transcriptional regulator ATRX protein which contributes to regulating the expression of various genes; mutations in the KMT2C gene which encodes lysine N-methyltransferase 2C protein (the KMT2C gene is mutated in various cancer types); amplification of the IL7R gene which encodes Interleukin-7 receptor- protein (mutations in the IL7R gene are commonly found in acute lymphoblastic leukemia) and expression of a fusion gene (i.e. a hybrid gene formed from two previously independent genes as a result of a mutation) that merges TRIO with other genes and is often found in other sarcoma subtypes.

• #18 Undifferentiated pleomorphic sarcoma – Wikipedia

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

  UPS tumors also show gene and chromosome abnormalities that further studies may find contribute to the development and/or progression of UPS. […] Deletion and/or inactivation or the RB1 gene that encodes (i.e. is responsible for production of) the retinoblastoma protein that functions as a tumor suppressor protein; deletions and/or mutations in the TP53 gene that encodes tumor protein P53 (a protein which regulates cell proliferation and cell death); mutations in the ATRX gene that encodes transcriptional regulator ATRX protein which contributes to regulating the expression of various genes; mutations in the KMT2C gene which encodes lysine N-methyltransferase 2C protein (the KMT2C gene is mutated in various cancer types); amplification of the IL7R gene which encodes Interleukin-7 receptor- protein (mutations in the IL7R gene are commonly found in acute lymphoblastic leukemia) and expression of a fusion gene (i.e. a hybrid gene formed from two previously independent genes as a result of a mutation) that merges TRIO with other genes and is often found in other sarcoma subtypes.

• #19 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.

• #20 Undifferentiated Sarcoma – SFA

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

  Undifferentiated pleomorphic sarcomas (UPSs) typically display extensive genomic rearrangements. Cytogenetic data from over 100 cases have revealed complex karyotypes with chromosome numbers ranging from near-haploid to hyperoctaploid. The extensive reshuffling of chromosomal material indicated by tumor karyotypes is corroborated by array-based genomic studies, showing structural rearrangements and copy-number shifts involving most if not all chromosomes, as well as by massively parallel sequencing studies. UPSs demonstrate substantial copy-number heterogeneity compared with other sarcoma subtypes and do not form distinct methylation subgroups. Whole-genome sequencing has revealed that punctuated evolutionary events such as whole-genome duplications and chromothripsis accompanied by telomere dysfunction underpin the genomic complexity of UPS. The aberrations in telomere biology are achieved either through activation of telomerase through deregulation of TERT or through the alternative lengthening of telomeres pathway. There are no tumor-specific amplicons, but some 10–15% of cases show amplification of VGLL3 or YAP1, and 10% have amplification of CCNE1. Targets of frequent deletions and disruptive rearrangements include CDKN2A and CDKN2B in 9p, PTEN in 10q, RB1 in 13q, and TP53 in 17p, each affected in 10–20% of cases. RB1 and TP53, as well as ATRX, are among the few genes that recurrently show pathogenetic variants at the nucleotide level. At the transcriptomic level, UPS cannot be distinguished from high-grade myxofibrosarcoma, and it is possible that these two entities may lie on a spectrum of differentiation. A small subset of UPSs with low mitotic counts show gene fusions involving the PRDM10 gene, with either MED12 or CITED2 as the 5′ partner. Otherwise, driver gene fusions have not been found in UPS.

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

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

  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. […] 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.

• #22 Undifferentiated Sarcoma – SFA

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

  Undifferentiated pleomorphic sarcomas (UPSs) typically display extensive genomic rearrangements. Cytogenetic data from over 100 cases have revealed complex karyotypes with chromosome numbers ranging from near-haploid to hyperoctaploid. The extensive reshuffling of chromosomal material indicated by tumor karyotypes is corroborated by array-based genomic studies, showing structural rearrangements and copy-number shifts involving most if not all chromosomes, as well as by massively parallel sequencing studies. UPSs demonstrate substantial copy-number heterogeneity compared with other sarcoma subtypes and do not form distinct methylation subgroups. Whole-genome sequencing has revealed that punctuated evolutionary events such as whole-genome duplications and chromothripsis accompanied by telomere dysfunction underpin the genomic complexity of UPS. The aberrations in telomere biology are achieved either through activation of telomerase through deregulation of TERT or through the alternative lengthening of telomeres pathway. There are no tumor-specific amplicons, but some 10–15% of cases show amplification of VGLL3 or YAP1, and 10% have amplification of CCNE1. Targets of frequent deletions and disruptive rearrangements include CDKN2A and CDKN2B in 9p, PTEN in 10q, RB1 in 13q, and TP53 in 17p, each affected in 10–20% of cases. RB1 and TP53, as well as ATRX, are among the few genes that recurrently show pathogenetic variants at the nucleotide level. At the transcriptomic level, UPS cannot be distinguished from high-grade myxofibrosarcoma, and it is possible that these two entities may lie on a spectrum of differentiation. A small subset of UPSs with low mitotic counts show gene fusions involving the PRDM10 gene, with either MED12 or CITED2 as the 5′ partner. Otherwise, driver gene fusions have not been found in UPS.

• #23 Undifferentiated Sarcoma – SFA

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

  Undifferentiated pleomorphic sarcomas (UPSs) typically display extensive genomic rearrangements. Cytogenetic data from over 100 cases have revealed complex karyotypes with chromosome numbers ranging from near-haploid to hyperoctaploid. The extensive reshuffling of chromosomal material indicated by tumor karyotypes is corroborated by array-based genomic studies, showing structural rearrangements and copy-number shifts involving most if not all chromosomes, as well as by massively parallel sequencing studies. UPSs demonstrate substantial copy-number heterogeneity compared with other sarcoma subtypes and do not form distinct methylation subgroups. Whole-genome sequencing has revealed that punctuated evolutionary events such as whole-genome duplications and chromothripsis accompanied by telomere dysfunction underpin the genomic complexity of UPS. The aberrations in telomere biology are achieved either through activation of telomerase through deregulation of TERT or through the alternative lengthening of telomeres pathway. There are no tumor-specific amplicons, but some 10–15% of cases show amplification of VGLL3 or YAP1, and 10% have amplification of CCNE1. Targets of frequent deletions and disruptive rearrangements include CDKN2A and CDKN2B in 9p, PTEN in 10q, RB1 in 13q, and TP53 in 17p, each affected in 10–20% of cases. RB1 and TP53, as well as ATRX, are among the few genes that recurrently show pathogenetic variants at the nucleotide level. At the transcriptomic level, UPS cannot be distinguished from high-grade myxofibrosarcoma, and it is possible that these two entities may lie on a spectrum of differentiation. A small subset of UPSs with low mitotic counts show gene fusions involving the PRDM10 gene, with either MED12 or CITED2 as the 5′ partner. Otherwise, driver gene fusions have not been found in UPS.

• #24 Undifferentiated Sarcoma – SFA

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

  Undifferentiated pleomorphic sarcomas (UPSs) typically display extensive genomic rearrangements. Cytogenetic data from over 100 cases have revealed complex karyotypes with chromosome numbers ranging from near-haploid to hyperoctaploid. The extensive reshuffling of chromosomal material indicated by tumor karyotypes is corroborated by array-based genomic studies, showing structural rearrangements and copy-number shifts involving most if not all chromosomes, as well as by massively parallel sequencing studies. UPSs demonstrate substantial copy-number heterogeneity compared with other sarcoma subtypes and do not form distinct methylation subgroups. Whole-genome sequencing has revealed that punctuated evolutionary events such as whole-genome duplications and chromothripsis accompanied by telomere dysfunction underpin the genomic complexity of UPS. The aberrations in telomere biology are achieved either through activation of telomerase through deregulation of TERT or through the alternative lengthening of telomeres pathway. There are no tumor-specific amplicons, but some 10–15% of cases show amplification of VGLL3 or YAP1, and 10% have amplification of CCNE1. Targets of frequent deletions and disruptive rearrangements include CDKN2A and CDKN2B in 9p, PTEN in 10q, RB1 in 13q, and TP53 in 17p, each affected in 10–20% of cases. RB1 and TP53, as well as ATRX, are among the few genes that recurrently show pathogenetic variants at the nucleotide level. At the transcriptomic level, UPS cannot be distinguished from high-grade myxofibrosarcoma, and it is possible that these two entities may lie on a spectrum of differentiation. A small subset of UPSs with low mitotic counts show gene fusions involving the PRDM10 gene, with either MED12 or CITED2 as the 5′ partner. Otherwise, driver gene fusions have not been found in UPS.

• #25 Undifferentiated Sarcoma – SFA

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

  Undifferentiated pleomorphic sarcomas (UPSs) typically display extensive genomic rearrangements. Cytogenetic data from over 100 cases have revealed complex karyotypes with chromosome numbers ranging from near-haploid to hyperoctaploid. The extensive reshuffling of chromosomal material indicated by tumor karyotypes is corroborated by array-based genomic studies, showing structural rearrangements and copy-number shifts involving most if not all chromosomes, as well as by massively parallel sequencing studies. UPSs demonstrate substantial copy-number heterogeneity compared with other sarcoma subtypes and do not form distinct methylation subgroups. Whole-genome sequencing has revealed that punctuated evolutionary events such as whole-genome duplications and chromothripsis accompanied by telomere dysfunction underpin the genomic complexity of UPS. The aberrations in telomere biology are achieved either through activation of telomerase through deregulation of TERT or through the alternative lengthening of telomeres pathway. There are no tumor-specific amplicons, but some 10–15% of cases show amplification of VGLL3 or YAP1, and 10% have amplification of CCNE1. Targets of frequent deletions and disruptive rearrangements include CDKN2A and CDKN2B in 9p, PTEN in 10q, RB1 in 13q, and TP53 in 17p, each affected in 10–20% of cases. RB1 and TP53, as well as ATRX, are among the few genes that recurrently show pathogenetic variants at the nucleotide level. At the transcriptomic level, UPS cannot be distinguished from high-grade myxofibrosarcoma, and it is possible that these two entities may lie on a spectrum of differentiation. A small subset of UPSs with low mitotic counts show gene fusions involving the PRDM10 gene, with either MED12 or CITED2 as the 5′ partner. Otherwise, driver gene fusions have not been found in UPS.

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

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

  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. […] 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.

• #27 Undifferentiated pleomorphic sarcoma – Wikipedia

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

  UPS tumor cells are undifferentiated (i.e. do not resemble any particular cell type) and pleomorphic (i.e. highly variable in size, shape, and/or color) when examined microscopically. […] A study of 52 individuals found that their UPS tumor cells expressed on their surface membranes PD-L1 protein (i.e. programmed death-ligand 1 protein) either focally (36.5% of cases) or strongly (9.62% of cases); 48.1% of these individuals had tumor cells which also expressed IDO1 protein (i.e. indoleamine 2,3-dioxygenase protein). […] Strong PDL1 expression proved to be a poor, while expression of IDO1 proved to be a favorable, prognostic factor for disease outcomes. […] Other abnormalities found in some or isolated cases of UPS include: Amplification of the Hippo signaling pathway, an intracellular cell signaling pathway that regulates cell proliferation and cell death; this amplification is associated with the overexpression of two proteins, vestigial-like family member 3 protein, a product of the VGLL3 gene, and YAP1, i.e. yes-associated protein 1, a product of the YAP1 gene, in the Hippo signaling pathway; Abnormal activation of notch signaling pathways (this activation has been shown to promote the growth and survival of various types of cancer cells; and Overexpression of DKK1, i.e. Dickkopf-related protein 1 (elevated in the tumor cells of various cancer types).

• #28 A Detailed Examination of Retroperitoneal Undifferentiated Pleomorphic Sarcoma: A Case Report and Review of the Existing Literature

  https://www.mdpi.com/2077-0383/13/13/3684

  These epigenetic changes affect the expression of downstream signaling components such as triple functional domain protein (TRIO) and rapamycin-insensitive companion of mTOR (RICTOR), thus activating the RAC/PAK (Ras-related C3 botulinum toxin substrate/p21-activated kinase) and Akt/mTOR pathways that are crucial for UPS cell survival. […] In undifferentiated pleomorphic sarcoma, a diverse array of signaling pathways plays a crucial role in the oncogenic process, with notable involvement of the phosphoinositide 3-kinase/protein kinase B/mammalian target of rapamycin (PI3K/Akt/mTOR) and Hippo signaling pathways. […] These pathways have been frequently implicated in the disease’s pathogenesis due to their influence on cell proliferation, invasion, and migration. […] The aberrant activation of these pathways in UPS is often compounded by independent mechanisms such as receptor overexpression, altered transcription, or post-translational modifications, which underscore the complexity of UPS pathogenesis.

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

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

  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. […] 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.

• #30 A Detailed Examination of Retroperitoneal Undifferentiated Pleomorphic Sarcoma: A Case Report and Review of the Existing Literature

  https://www.mdpi.com/2077-0383/13/13/3684

  These epigenetic changes affect the expression of downstream signaling components such as triple functional domain protein (TRIO) and rapamycin-insensitive companion of mTOR (RICTOR), thus activating the RAC/PAK (Ras-related C3 botulinum toxin substrate/p21-activated kinase) and Akt/mTOR pathways that are crucial for UPS cell survival. […] In undifferentiated pleomorphic sarcoma, a diverse array of signaling pathways plays a crucial role in the oncogenic process, with notable involvement of the phosphoinositide 3-kinase/protein kinase B/mammalian target of rapamycin (PI3K/Akt/mTOR) and Hippo signaling pathways. […] These pathways have been frequently implicated in the disease’s pathogenesis due to their influence on cell proliferation, invasion, and migration. […] The aberrant activation of these pathways in UPS is often compounded by independent mechanisms such as receptor overexpression, altered transcription, or post-translational modifications, which underscore the complexity of UPS pathogenesis.

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

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

  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. […] 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.

• #32 A Detailed Examination of Retroperitoneal Undifferentiated Pleomorphic Sarcoma: A Case Report and Review of the Existing Literature

  https://www.mdpi.com/2077-0383/13/13/3684

  These epigenetic changes affect the expression of downstream signaling components such as triple functional domain protein (TRIO) and rapamycin-insensitive companion of mTOR (RICTOR), thus activating the RAC/PAK (Ras-related C3 botulinum toxin substrate/p21-activated kinase) and Akt/mTOR pathways that are crucial for UPS cell survival. […] In undifferentiated pleomorphic sarcoma, a diverse array of signaling pathways plays a crucial role in the oncogenic process, with notable involvement of the phosphoinositide 3-kinase/protein kinase B/mammalian target of rapamycin (PI3K/Akt/mTOR) and Hippo signaling pathways. […] These pathways have been frequently implicated in the disease’s pathogenesis due to their influence on cell proliferation, invasion, and migration. […] The aberrant activation of these pathways in UPS is often compounded by independent mechanisms such as receptor overexpression, altered transcription, or post-translational modifications, which underscore the complexity of UPS pathogenesis.

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

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

  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. […] 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.

• #34 Undifferentiated pleomorphic sarcoma – Wikipedia

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

  UPS tumor cells are undifferentiated (i.e. do not resemble any particular cell type) and pleomorphic (i.e. highly variable in size, shape, and/or color) when examined microscopically. […] A study of 52 individuals found that their UPS tumor cells expressed on their surface membranes PD-L1 protein (i.e. programmed death-ligand 1 protein) either focally (36.5% of cases) or strongly (9.62% of cases); 48.1% of these individuals had tumor cells which also expressed IDO1 protein (i.e. indoleamine 2,3-dioxygenase protein). […] Strong PDL1 expression proved to be a poor, while expression of IDO1 proved to be a favorable, prognostic factor for disease outcomes. […] Other abnormalities found in some or isolated cases of UPS include: Amplification of the Hippo signaling pathway, an intracellular cell signaling pathway that regulates cell proliferation and cell death; this amplification is associated with the overexpression of two proteins, vestigial-like family member 3 protein, a product of the VGLL3 gene, and YAP1, i.e. yes-associated protein 1, a product of the YAP1 gene, in the Hippo signaling pathway; Abnormal activation of notch signaling pathways (this activation has been shown to promote the growth and survival of various types of cancer cells; and Overexpression of DKK1, i.e. Dickkopf-related protein 1 (elevated in the tumor cells of various cancer types).

• #35 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. UPS-derived SP cells and UPS in situ analyses showed that Hedgehog and Notch signaling pathways and downstream transcriptional targets are notoriously upregulated at both subcellular and tissue levels.

• #36 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. […] According to an experimental model, these cells have an improved potential for self-renewal, growth, and proliferation and can mimic tumor development. […] 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.

• #37 A Detailed Examination of Retroperitoneal Undifferentiated Pleomorphic Sarcoma: A Case Report and Review of the Existing Literature

  https://www.mdpi.com/2077-0383/13/13/3684

  These epigenetic changes affect the expression of downstream signaling components such as triple functional domain protein (TRIO) and rapamycin-insensitive companion of mTOR (RICTOR), thus activating the RAC/PAK (Ras-related C3 botulinum toxin substrate/p21-activated kinase) and Akt/mTOR pathways that are crucial for UPS cell survival. […] In undifferentiated pleomorphic sarcoma, a diverse array of signaling pathways plays a crucial role in the oncogenic process, with notable involvement of the phosphoinositide 3-kinase/protein kinase B/mammalian target of rapamycin (PI3K/Akt/mTOR) and Hippo signaling pathways. […] These pathways have been frequently implicated in the disease’s pathogenesis due to their influence on cell proliferation, invasion, and migration. […] The aberrant activation of these pathways in UPS is often compounded by independent mechanisms such as receptor overexpression, altered transcription, or post-translational modifications, which underscore the complexity of UPS pathogenesis.

• #38 A Detailed Examination of Retroperitoneal Undifferentiated Pleomorphic Sarcoma: A Case Report and Review of the Existing Literature

  https://www.mdpi.com/2077-0383/13/13/3684

  The tumor microenvironment plays a critical and complex role in the progression of UPS, particularly through its inflammatory components. […] Within this microenvironment, tumor-associated macrophages (TAMs) are prominent players, known for their protumoral activities. […] These macrophages produce significant levels of cytokines such as transforming growth factor-beta (TGFβ) and interleukin-6 (IL6), which activate downstream signaling pathways leading to enhanced tumor cell proliferation, migration, and invasion. […] The proportion of TAMs within the tumor has been identified as a prognostic factor in UPS, indicating their significant impact on tumor behavior and patient outcomes.

• #39 A Detailed Examination of Retroperitoneal Undifferentiated Pleomorphic Sarcoma: A Case Report and Review of the Existing Literature

  https://www.mdpi.com/2077-0383/13/13/3684

  The tumor microenvironment plays a critical and complex role in the progression of UPS, particularly through its inflammatory components. […] Within this microenvironment, tumor-associated macrophages (TAMs) are prominent players, known for their protumoral activities. […] These macrophages produce significant levels of cytokines such as transforming growth factor-beta (TGFβ) and interleukin-6 (IL6), which activate downstream signaling pathways leading to enhanced tumor cell proliferation, migration, and invasion. […] The proportion of TAMs within the tumor has been identified as a prognostic factor in UPS, indicating their significant impact on tumor behavior and patient outcomes.

• #40 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. UPS-derived SP cells and UPS in situ analyses showed that Hedgehog and Notch signaling pathways and downstream transcriptional targets are notoriously upregulated at both subcellular and tissue levels.

• #41 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. […] According to an experimental model, these cells have an improved potential for self-renewal, growth, and proliferation and can mimic tumor development. […] 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.

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

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

  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. […] 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.

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

  https://www.ochsnerjournal.org/content/early/2022/09/21/toj.22.0042

  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. […] Standard treatment for UPS is early complete surgical resection with negative resection margins and en bloc lymph node dissection. The role of chemotherapy and radiation in the treatment of UPS is debated and without strong evidence. An increasing number of reports suggest that adjuvant chemotherapy or radiation may improve prognosis in certain clinical scenarios.

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

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

  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. […] 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.

• #45 Pleomorphic Sarcoma (Malignant Fibrous Histiocytoma) of Soft Tissue Imaging: Practice Essentials, Radiography, Computed Tomography

  https://emedicine.medscape.com/article/391453-overview

  Undifferentiated pleomorphic sarcoma (UPS), previously called malignant fibrous histiocytoma (MFH), is a soft tissue sarcoma (STS) that can occur anywhere in the body, but it usually occurs in the extremities (especially the thighs) or back of the abdomen. Approximately 15% of undifferentiated pleomorphic sarcomas arise in the abdomen and pelvis. […] UPS often grows quickly and spreads to other parts of the body, including the lungs. […] Undifferentiated pleomorphic sarcoma usually occurs in older adults, and men account for two thirds of cases. UPS is the most common sarcoma to develop at sites of prior irradiation. Investigators have shown that most patients have received a radiation dose of 50 Gy or more, and the median time interval between radiation exposure and the development of a radiation-associated sarcoma is approximately 10 years. Undifferentiated pleomorphic sarcoma may also arise at sites of chronic ulceration.

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

  https://www.ochsnerjournal.org/content/early/2022/09/21/toj.22.0042

  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. […] Standard treatment for UPS is early complete surgical resection with negative resection margins and en bloc lymph node dissection. The role of chemotherapy and radiation in the treatment of UPS is debated and without strong evidence. An increasing number of reports suggest that adjuvant chemotherapy or radiation may improve prognosis in certain clinical scenarios.

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

  https://www.ochsnerjournal.org/content/early/2022/09/21/toj.22.0042

  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. […] Standard treatment for UPS is early complete surgical resection with negative resection margins and en bloc lymph node dissection. The role of chemotherapy and radiation in the treatment of UPS is debated and without strong evidence. An increasing number of reports suggest that adjuvant chemotherapy or radiation may improve prognosis in certain clinical scenarios.

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

  https://www.ochsnerjournal.org/content/early/2022/09/21/toj.22.0042

  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. […] Standard treatment for UPS is early complete surgical resection with negative resection margins and en bloc lymph node dissection. The role of chemotherapy and radiation in the treatment of UPS is debated and without strong evidence. An increasing number of reports suggest that adjuvant chemotherapy or radiation may improve prognosis in certain clinical scenarios.

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

  https://www.ochsnerjournal.org/content/early/2022/09/21/toj.22.0042

  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. […] Standard treatment for UPS is early complete surgical resection with negative resection margins and en bloc lymph node dissection. The role of chemotherapy and radiation in the treatment of UPS is debated and without strong evidence. An increasing number of reports suggest that adjuvant chemotherapy or radiation may improve prognosis in certain clinical scenarios.

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

  https://www.ochsnerjournal.org/content/early/2022/09/21/toj.22.0042

  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. […] Standard treatment for UPS is early complete surgical resection with negative resection margins and en bloc lymph node dissection. The role of chemotherapy and radiation in the treatment of UPS is debated and without strong evidence. An increasing number of reports suggest that adjuvant chemotherapy or radiation may improve prognosis in certain clinical scenarios.

• #51 Undifferentiated Pleomorphic Sarcoma: A Case Report

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11632629/

  Undifferentiated pleomorphic sarcoma (UPS) is an uncommon and aggressive soft tissue tumor primarily affecting older adults, often presenting as a rapidly enlarging, painless mass in the extremities or other deep-seated locations. […] The exact aetiology and pathogenesis of undifferentiated pleomorphic sarcomas remain undetermined. […] Minor trauma triggering the development of soft tissue sarcomas is rare, and the association between these events is not well understood. […] In this patient’s case, the association of trauma with the development of Undifferentiated Pleomorphic Sarcoma (UPS) remains challenging to establish definitively, though there are some theoretical and experimental underpinnings that suggest trauma might act as a promoter for sarcoma formation under certain conditions.

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

  https://www.ochsnerjournal.org/content/early/2022/09/21/toj.22.0042

  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. […] Standard treatment for UPS is early complete surgical resection with negative resection margins and en bloc lymph node dissection. The role of chemotherapy and radiation in the treatment of UPS is debated and without strong evidence. An increasing number of reports suggest that adjuvant chemotherapy or radiation may improve prognosis in certain clinical scenarios.

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

  https://www.ochsnerjournal.org/content/early/2022/09/21/toj.22.0042

  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. […] Standard treatment for UPS is early complete surgical resection with negative resection margins and en bloc lymph node dissection. The role of chemotherapy and radiation in the treatment of UPS is debated and without strong evidence. An increasing number of reports suggest that adjuvant chemotherapy or radiation may improve prognosis in certain clinical scenarios.

• #54 Pleomorphic Sarcoma (Malignant Fibrous Histiocytoma) of Soft Tissue Imaging: Practice Essentials, Radiography, Computed Tomography

  https://emedicine.medscape.com/article/391453-overview

  Undifferentiated pleomorphic sarcoma (UPS), previously called malignant fibrous histiocytoma (MFH), is a soft tissue sarcoma (STS) that can occur anywhere in the body, but it usually occurs in the extremities (especially the thighs) or back of the abdomen. Approximately 15% of undifferentiated pleomorphic sarcomas arise in the abdomen and pelvis. […] UPS often grows quickly and spreads to other parts of the body, including the lungs. […] Undifferentiated pleomorphic sarcoma usually occurs in older adults, and men account for two thirds of cases. UPS is the most common sarcoma to develop at sites of prior irradiation. Investigators have shown that most patients have received a radiation dose of 50 Gy or more, and the median time interval between radiation exposure and the development of a radiation-associated sarcoma is approximately 10 years. Undifferentiated pleomorphic sarcoma may also arise at sites of chronic ulceration.

• #55 Undifferentiated Pleomorphic Sarcoma: A Case Report

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11632629/

  Undifferentiated pleomorphic sarcoma (UPS) is an uncommon and aggressive soft tissue tumor primarily affecting older adults, often presenting as a rapidly enlarging, painless mass in the extremities or other deep-seated locations. […] The exact aetiology and pathogenesis of undifferentiated pleomorphic sarcomas remain undetermined. […] Minor trauma triggering the development of soft tissue sarcomas is rare, and the association between these events is not well understood. […] In this patient’s case, the association of trauma with the development of Undifferentiated Pleomorphic Sarcoma (UPS) remains challenging to establish definitively, though there are some theoretical and experimental underpinnings that suggest trauma might act as a promoter for sarcoma formation under certain conditions.

• #56 Undifferentiated pleomorphic sarcoma – Wikipedia

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

  UPS is considered a diagnosis that defies formal sub-classification after thorough histologic, immunohistochemical, and ultrastructural examinations fail to identify the type of cells involved. […] Studies strongly suggest that MFH tumors are not derived from histiocytes (cells descended from blood monocytes), but rather from mesenchymal cells. […] The majority of UPS tumors are highly aggressive, often recur after surgical removal, and often metastasize. […] More recently, UPS tumors have been treated with antibody therapy, i.e. antibodies which in the case of UPS bind to specific antigens on the surface of T-cells (a type of lymphocyte) and thereby promote the ability of these T-cells to organizes an attack on UPS tumor cells. […] UPS is a diagnosis of exclusion (a diagnosis reached by the process of elimination) because the histopathology of this disorder’s tumors is non-specific.

• #57 Undifferentiated pleomorphic sarcoma – Wikipedia

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

  UPS is considered a diagnosis that defies formal sub-classification after thorough histologic, immunohistochemical, and ultrastructural examinations fail to identify the type of cells involved. […] Studies strongly suggest that MFH tumors are not derived from histiocytes (cells descended from blood monocytes), but rather from mesenchymal cells. […] The majority of UPS tumors are highly aggressive, often recur after surgical removal, and often metastasize. […] More recently, UPS tumors have been treated with antibody therapy, i.e. antibodies which in the case of UPS bind to specific antigens on the surface of T-cells (a type of lymphocyte) and thereby promote the ability of these T-cells to organizes an attack on UPS tumor cells. […] UPS is a diagnosis of exclusion (a diagnosis reached by the process of elimination) because the histopathology of this disorder’s tumors is non-specific.

• #58 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. […] UPS is thought to be derived from a primitive mesenchymal cell capable of differentiating into histiocytes, fibroblasts, myofibroblasts and osteoclasts. The etiology of the tumor remains unknown. Prior radiation therapy is a likely risk factor in some cases.

• #59 Undifferentiated pleomorphic sarcoma of the mandible

  https://www.jkaoms.org/journal/view.html?doi=10.5125/jkaoms.2020.46.4.282

  Histogenesis of UPS remains debatable; in contrast to the original concept, tumors do not seem to have a true histiocytic differentiation and instead are more likely to harbor a fibroblastic/myofibroblastic nature. […] UPS is a diagnosis of exclusion when no other more specific entity is identified after extensive histological examination and use of ancillary diagnostic techniques. […] Immunohistochemistry is of great importance in achieving a final diagnosis. […] Recurrences are common, and distant metastases are likely to arise in the lung, liver, and bone, as observed in our patient. […] UPS is an aggressive malignant neoplasm that rarely involves the oral cavity and gnathic bones, and that demands an extensive diagnostic effort to exclude other lineage-specific neoplasms.

• #60 Undifferentiated Sarcoma – SFA

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

  Undifferentiated pleomorphic sarcomas (UPSs) typically display extensive genomic rearrangements. Cytogenetic data from over 100 cases have revealed complex karyotypes with chromosome numbers ranging from near-haploid to hyperoctaploid. The extensive reshuffling of chromosomal material indicated by tumor karyotypes is corroborated by array-based genomic studies, showing structural rearrangements and copy-number shifts involving most if not all chromosomes, as well as by massively parallel sequencing studies. UPSs demonstrate substantial copy-number heterogeneity compared with other sarcoma subtypes and do not form distinct methylation subgroups. Whole-genome sequencing has revealed that punctuated evolutionary events such as whole-genome duplications and chromothripsis accompanied by telomere dysfunction underpin the genomic complexity of UPS. The aberrations in telomere biology are achieved either through activation of telomerase through deregulation of TERT or through the alternative lengthening of telomeres pathway. There are no tumor-specific amplicons, but some 10–15% of cases show amplification of VGLL3 or YAP1, and 10% have amplification of CCNE1. Targets of frequent deletions and disruptive rearrangements include CDKN2A and CDKN2B in 9p, PTEN in 10q, RB1 in 13q, and TP53 in 17p, each affected in 10–20% of cases. RB1 and TP53, as well as ATRX, are among the few genes that recurrently show pathogenetic variants at the nucleotide level. At the transcriptomic level, UPS cannot be distinguished from high-grade myxofibrosarcoma, and it is possible that these two entities may lie on a spectrum of differentiation. A small subset of UPSs with low mitotic counts show gene fusions involving the PRDM10 gene, with either MED12 or CITED2 as the 5′ partner. Otherwise, driver gene fusions have not been found in UPS.

• #61 Smooth muscle differentiation identifies two classes of poorly differentiated pleomorphic sarcomas with distinct outcome | Modern Pathology

  https://www.nature.com/articles/modpathol2013205

  The clinical relevance of accurately diagnosing pleomorphic sarcomas has been shown, especially in cases of undifferentiated pleomorphic sarcomas with myogenic differentiation, which appear significantly more aggressive. […] The clinical relevance of a better method of diagnosing undifferentiated pleomorphic sarcomas has been demonstrated, especially in cases of undifferentiated pleomorphic sarcomas with myogenic differentiation, which seem to be significantly more aggressive. […] The smooth muscle differentiation classification method may be a useful diagnostic tool as well as a relevant prognostic tool for undifferentiated pleomorphic sarcomas. […] The smooth muscle differentiation classification method, taking into account not only positivity for smooth muscle markers but also their labeling intensity, exhibited the strongest prognostic value compared with other prognostic factors, including histological classification.

• #62 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. […] UPS is a diagnosis of exclusion. This means that the tumour shows no identifiable characteristics for the doctors to be able to diagnose it as a specific sarcoma type. […] In UPS, researchers have been learning more about treatments known as targeted therapies and immunotherapies. […] Researchers have found that some targeted therapies and immunotherapies worked well in clinical trials of UPS. But, more research and trials are needed before these treatments are approved.

• #63 Undifferentiated Pleomorphic Sarcoma/Myxofibrosarcoma/Other Fibrosarcomas | Springer Publishing

  https://connect.springerpub.com/content/book/978-0-8261-4853-7/part/part02/chapter/ch20

  Undifferentiated pleomorphic sarcoma (UPS) encompasses malignant neoplasms of mesenchymal origin, which demonstrate cellular pleomorphism and which lack evidence of cellular differentiation. […] The term UPS has been coined in replacement of the obsolete term malignant fibrous histiocytoma, as research evidence revealed the latter constituted a diverse group of neoplasms with various differentiation. […] For localized disease, the treatment of high-grade tumors includes a multimodality approach (perioperative chemotherapy, radiation therapy, and surgery), followed by close surveillance with scans and follow-up visits. […] For metastatic disease, patients will require systemic therapies as well as radiation therapy and surgery in selected patients. […] This chapter reviews clinical presentation, diagnostic approach, and general therapeutic approach for UPS, and discusses metastatic disease, atypical fibroxanthoma, and fibrosarcoma subtypes.

• #64 Undifferentiated pleomorphic sarcoma – Wikipedia

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

  UPS is considered a diagnosis that defies formal sub-classification after thorough histologic, immunohistochemical, and ultrastructural examinations fail to identify the type of cells involved. […] Studies strongly suggest that MFH tumors are not derived from histiocytes (cells descended from blood monocytes), but rather from mesenchymal cells. […] The majority of UPS tumors are highly aggressive, often recur after surgical removal, and often metastasize. […] More recently, UPS tumors have been treated with antibody therapy, i.e. antibodies which in the case of UPS bind to specific antigens on the surface of T-cells (a type of lymphocyte) and thereby promote the ability of these T-cells to organizes an attack on UPS tumor cells. […] UPS is a diagnosis of exclusion (a diagnosis reached by the process of elimination) because the histopathology of this disorder’s tumors is non-specific.

• #65 Undifferentiated pleomorphic sarcoma – Wikipedia

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

  UPS tumor cells are undifferentiated (i.e. do not resemble any particular cell type) and pleomorphic (i.e. highly variable in size, shape, and/or color) when examined microscopically. […] A study of 52 individuals found that their UPS tumor cells expressed on their surface membranes PD-L1 protein (i.e. programmed death-ligand 1 protein) either focally (36.5% of cases) or strongly (9.62% of cases); 48.1% of these individuals had tumor cells which also expressed IDO1 protein (i.e. indoleamine 2,3-dioxygenase protein). […] Strong PDL1 expression proved to be a poor, while expression of IDO1 proved to be a favorable, prognostic factor for disease outcomes. […] Other abnormalities found in some or isolated cases of UPS include: Amplification of the Hippo signaling pathway, an intracellular cell signaling pathway that regulates cell proliferation and cell death; this amplification is associated with the overexpression of two proteins, vestigial-like family member 3 protein, a product of the VGLL3 gene, and YAP1, i.e. yes-associated protein 1, a product of the YAP1 gene, in the Hippo signaling pathway; Abnormal activation of notch signaling pathways (this activation has been shown to promote the growth and survival of various types of cancer cells; and Overexpression of DKK1, i.e. Dickkopf-related protein 1 (elevated in the tumor cells of various cancer types).

• #66 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. […] Diagnosis is based on a combination of microscopic features and immunohistochemical staining techniques used to rule out other cell lines of origin. Lesions are typically characterized by pleomorphic, spindle-shaped cells with bizarre cytology and nuclear atypia. Importantly, UPS has no reproducible immunophenotype or pattern of protein expression that allows for further classification of the tumor, and exclusion of pleomorphic variants of other neoplastic lines is required.

• #67 An approach to pleomorphic sarcomas: can we subclassify, and does it matter? | Modern Pathology

  https://www.nature.com/articles/modpathol2013174

  The only criterion for rendering a diagnosis of pleomorphic liposarcoma is the recognition of multivacuolated pleomorphic lipoblasts. […] The only criterion for recognizing extraskeletal osteosarcoma is the production of osteoid or bone by cytologically malignant cells. […] As mentioned, several studies have suggested that pleomorphic sarcomas with myogenic differentiated are clinically more aggressive than those without myogenic differentiation. […] The process of tumor progression or dedifferentiation involves the transformation of a low-grade sarcoma to a higher-grade sarcoma, which usually (but not always) resembles a UPS. […] There is some evidence to suggest that dedifferentiated sarcomas (dedifferentiated liposarcoma in particular) are more indolent tumors than de novo pleomorphic sarcomas arising in the same location.

• #68 An approach to pleomorphic sarcomas: can we subclassify, and does it matter? | Modern Pathology

  https://www.nature.com/articles/modpathol2013174

  The only criterion for rendering a diagnosis of pleomorphic liposarcoma is the recognition of multivacuolated pleomorphic lipoblasts. […] The only criterion for recognizing extraskeletal osteosarcoma is the production of osteoid or bone by cytologically malignant cells. […] As mentioned, several studies have suggested that pleomorphic sarcomas with myogenic differentiated are clinically more aggressive than those without myogenic differentiation. […] The process of tumor progression or dedifferentiation involves the transformation of a low-grade sarcoma to a higher-grade sarcoma, which usually (but not always) resembles a UPS. […] There is some evidence to suggest that dedifferentiated sarcomas (dedifferentiated liposarcoma in particular) are more indolent tumors than de novo pleomorphic sarcomas arising in the same location.

• #69 An approach to pleomorphic sarcomas: can we subclassify, and does it matter? | Modern Pathology

  https://www.nature.com/articles/modpathol2013174

  The only criterion for rendering a diagnosis of pleomorphic liposarcoma is the recognition of multivacuolated pleomorphic lipoblasts. […] The only criterion for recognizing extraskeletal osteosarcoma is the production of osteoid or bone by cytologically malignant cells. […] As mentioned, several studies have suggested that pleomorphic sarcomas with myogenic differentiated are clinically more aggressive than those without myogenic differentiation. […] The process of tumor progression or dedifferentiation involves the transformation of a low-grade sarcoma to a higher-grade sarcoma, which usually (but not always) resembles a UPS. […] There is some evidence to suggest that dedifferentiated sarcomas (dedifferentiated liposarcoma in particular) are more indolent tumors than de novo pleomorphic sarcomas arising in the same location.

• #70 An approach to pleomorphic sarcomas: can we subclassify, and does it matter? | Modern Pathology

  https://www.nature.com/articles/modpathol2013174

  The only criterion for rendering a diagnosis of pleomorphic liposarcoma is the recognition of multivacuolated pleomorphic lipoblasts. […] The only criterion for recognizing extraskeletal osteosarcoma is the production of osteoid or bone by cytologically malignant cells. […] As mentioned, several studies have suggested that pleomorphic sarcomas with myogenic differentiated are clinically more aggressive than those without myogenic differentiation. […] The process of tumor progression or dedifferentiation involves the transformation of a low-grade sarcoma to a higher-grade sarcoma, which usually (but not always) resembles a UPS. […] There is some evidence to suggest that dedifferentiated sarcomas (dedifferentiated liposarcoma in particular) are more indolent tumors than de novo pleomorphic sarcomas arising in the same location.

• #71 An approach to pleomorphic sarcomas: can we subclassify, and does it matter? | Modern Pathology

  https://www.nature.com/articles/modpathol2013174

  The utility of MDM2 and CDK4 analysis in distinguishing dedifferentiated liposarcoma from other types of pleomorphic sarcoma is controversial, but the preponderance of evidence suggests that this analysis is useful. […] It can be exceedingly difficult to distinguish a UPS from a sarcomatoid carcinoma. […] Depending on definitional criteria, UPS still accounts for a significant proportion of sarcomas occurring in late adult life. […] UPS is characteristically a tumor of late adult life, with most cases occurring in persons between the ages of 50 and 70 years. […] The factors that correlate consistently with metastasis, survival, or both are depth, tumor size, grade, necrosis, and local recurrence, although they are not necessarily independent variables. […] In summary, pleomorphic malignant neoplasms are commonly encountered by surgical pathologists.

• #72 Smooth muscle differentiation identifies two classes of poorly differentiated pleomorphic sarcomas with distinct outcome | Modern Pathology

  https://www.nature.com/articles/modpathol2013205

  The clinical relevance of accurately diagnosing pleomorphic sarcomas has been shown, especially in cases of undifferentiated pleomorphic sarcomas with myogenic differentiation, which appear significantly more aggressive. […] The clinical relevance of a better method of diagnosing undifferentiated pleomorphic sarcomas has been demonstrated, especially in cases of undifferentiated pleomorphic sarcomas with myogenic differentiation, which seem to be significantly more aggressive. […] The smooth muscle differentiation classification method may be a useful diagnostic tool as well as a relevant prognostic tool for undifferentiated pleomorphic sarcomas. […] The smooth muscle differentiation classification method, taking into account not only positivity for smooth muscle markers but also their labeling intensity, exhibited the strongest prognostic value compared with other prognostic factors, including histological classification.

• #73

  https://journals.lww.com/jpat/fulltext/2023/27001/undifferentiated_pleomorphic_sarcoma_of_the_floor.8.aspx

  Undifferentiated pleomorphic sarcoma (UPS) previously called as malignant fibrous histiocytoma comprises a group of high-grade pleomorphic sarcomas that cannot be otherwise classified and considered as a diagnosis of exclusion. […] Undifferentiated pleomorphic sarcoma (UPS) is a soft tissue sarcoma composed of undifferentiated mesenchymal cells with fibrohistiocytic morphology without lineage specific differentiation representing a diagnosis of exclusion in the current World Health Organization. […] The UPSs represent about 5% of all soft tissue sarcomas in adults and occur more commonly in the extremities, mainly lower limbs, of patients during the 6th and 7th decades of life. […] UPS is an aggressive tumor, with a high potential of metastasis to other parts of the body. […] The prognosis of UPS is dismal.

• #74

  https://journals.lww.com/jpat/fulltext/2023/27001/undifferentiated_pleomorphic_sarcoma_of_the_floor.8.aspx

  The vast majority of UPS are high-grade lesions having a local recurrence rate ranging from 19-31%, a metastatic rate of 31-35%, and a 5-year survival of 65-70%. […] Once diagnosed, multimodality therapeutic approach combining surgery, external beam radiation therapy chemotherapy drugs like doxorubicin tailored according to the patient is the only option left.

• #75 An approach to pleomorphic sarcomas: can we subclassify, and does it matter? | Modern Pathology

  https://www.nature.com/articles/modpathol2013174

  The utility of MDM2 and CDK4 analysis in distinguishing dedifferentiated liposarcoma from other types of pleomorphic sarcoma is controversial, but the preponderance of evidence suggests that this analysis is useful. […] It can be exceedingly difficult to distinguish a UPS from a sarcomatoid carcinoma. […] Depending on definitional criteria, UPS still accounts for a significant proportion of sarcomas occurring in late adult life. […] UPS is characteristically a tumor of late adult life, with most cases occurring in persons between the ages of 50 and 70 years. […] The factors that correlate consistently with metastasis, survival, or both are depth, tumor size, grade, necrosis, and local recurrence, although they are not necessarily independent variables. […] In summary, pleomorphic malignant neoplasms are commonly encountered by surgical pathologists.

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

  https://radiopaedia.org/articles/undifferentiated-pleomorphic-sarcoma-1?embed_domain=hackmd.io%25252F%252540yipuafecsl2jsu8smr5njq%25252Fbnjhjgjghjghjgh&lang=gb

  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 behaviour and a poor prognosis. […] Undifferentiated pleomorphic sarcomas are aggressive tumours that account for 25-40% of all adult soft tissue sarcomas, making them the most common type. However, the classification system is becoming more restrictive, with many tumours being reclassified as variants of myogenic sarcomas. […] Most undifferentiated pleomorphic sarcomas are high grade (3 and 4) and are aggressive in their biological behaviour. They frequently metastasise (30-50% at diagnosis) and locally recur despite aggressive treatment. The overall 5-year survival is between 25-70%. […] Prognostic factors include: tumour size: smaller being better; location: superficial is better; distal is better; histological grade; histological subtype: the myxoid subtype has a better prognosis compared to the storiform-pleomorphic subtype.

• #77 Undifferentiated pleomorphic sarcoma – Wikipedia

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

  UPS tumor cells are undifferentiated (i.e. do not resemble any particular cell type) and pleomorphic (i.e. highly variable in size, shape, and/or color) when examined microscopically. […] A study of 52 individuals found that their UPS tumor cells expressed on their surface membranes PD-L1 protein (i.e. programmed death-ligand 1 protein) either focally (36.5% of cases) or strongly (9.62% of cases); 48.1% of these individuals had tumor cells which also expressed IDO1 protein (i.e. indoleamine 2,3-dioxygenase protein). […] Strong PDL1 expression proved to be a poor, while expression of IDO1 proved to be a favorable, prognostic factor for disease outcomes. […] Other abnormalities found in some or isolated cases of UPS include: Amplification of the Hippo signaling pathway, an intracellular cell signaling pathway that regulates cell proliferation and cell death; this amplification is associated with the overexpression of two proteins, vestigial-like family member 3 protein, a product of the VGLL3 gene, and YAP1, i.e. yes-associated protein 1, a product of the YAP1 gene, in the Hippo signaling pathway; Abnormal activation of notch signaling pathways (this activation has been shown to promote the growth and survival of various types of cancer cells; and Overexpression of DKK1, i.e. Dickkopf-related protein 1 (elevated in the tumor cells of various cancer types).

• #78 Undifferentiated Pleomorphic Sarcoma: A Case Report

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11632629/

  Undifferentiated pleomorphic sarcoma (UPS) is an uncommon and aggressive soft tissue tumor primarily affecting older adults, often presenting as a rapidly enlarging, painless mass in the extremities or other deep-seated locations. […] The exact aetiology and pathogenesis of undifferentiated pleomorphic sarcomas remain undetermined. […] Minor trauma triggering the development of soft tissue sarcomas is rare, and the association between these events is not well understood. […] In this patient’s case, the association of trauma with the development of Undifferentiated Pleomorphic Sarcoma (UPS) remains challenging to establish definitively, though there are some theoretical and experimental underpinnings that suggest trauma might act as a promoter for sarcoma formation under certain conditions.

• #79 UNDIFFERENTIATED PLEOMORPHIC SARCOMA – MALIGNANT FIBROUS HISTIOCYTOMA

  https://www.seyitaligumustas.com/en/undifferentiated-pleomorphic-sarcoma-malignant-fibrous-histiocytoma

  Undifferentiated pleomorphic sarcoma is the most common malignant soft tissue tumor. The exact cause and tissue of origin are unknown. […] The main treatment for undifferentiated pleomorphic sarcoma is clean surgical removal of the tumor. The tumor that is not removed cleanly with wide margins has a nearly one hundred percent recurrence rate and is prone to metastasis. […] Radiation therapy is used before or after surgery to facilitate surgery and reduce the possibility of recurrence. […] Chemotherapy is not routinely used for undifferentiated pleomorphic sarcoma. Chemotherapy is used especially in the presence of metastases (often to the lungs) and in large and deep-seated tumors. […] The five-year survival rate for patients with undifferentiated pleomorphic sarcoma is 60-65%. Life expectancy is lower for deep-seated and large tumors, especially in the presence of metastases. […] Patients diagnosed with undifferentiated pleomorphic sarcoma should be followed for many years at regular intervals for recurrence and metastasis, which means spread to other parts of the body.

• #80 UNDIFFERENTIATED PLEOMORPHIC SARCOMA – MALIGNANT FIBROUS HISTIOCYTOMA

  https://www.seyitaligumustas.com/en/undifferentiated-pleomorphic-sarcoma-malignant-fibrous-histiocytoma

  Undifferentiated pleomorphic sarcoma is the most common malignant soft tissue tumor. The exact cause and tissue of origin are unknown. […] The main treatment for undifferentiated pleomorphic sarcoma is clean surgical removal of the tumor. The tumor that is not removed cleanly with wide margins has a nearly one hundred percent recurrence rate and is prone to metastasis. […] Radiation therapy is used before or after surgery to facilitate surgery and reduce the possibility of recurrence. […] Chemotherapy is not routinely used for undifferentiated pleomorphic sarcoma. Chemotherapy is used especially in the presence of metastases (often to the lungs) and in large and deep-seated tumors. […] The five-year survival rate for patients with undifferentiated pleomorphic sarcoma is 60-65%. Life expectancy is lower for deep-seated and large tumors, especially in the presence of metastases. […] Patients diagnosed with undifferentiated pleomorphic sarcoma should be followed for many years at regular intervals for recurrence and metastasis, which means spread to other parts of the body.

• #81 Undifferentiated Pleomorphic Sarcoma: A Case Report

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11632629/

  Animal studies suggest a link between trauma, inflammation, and sarcoma formation, particularly in genetically predisposed tissues. […] For this patient, trauma may have promoted inflammation and cellular changes, potentially accelerating sarcoma formation in a genetically susceptible area, though it’s unclear if trauma caused or revealed a pre-existing lesion. […] The primary presentation of UPS is often non-specific, with the majority of patients noticing a painless, enlarging mass. […] Definitive treatment of UPS involves en bloc surgical excision, with an emphasis on achieving clear microscopic margins to reduce the risk of local recurrence. […] Due to the non-specific nature of UPS, making a definitive pathological diagnosis requires multiple immunohistochemical stains, as it is largely a diagnosis of exclusion. […] The role of adjuvant chemotherapy in the treatment of resectable soft tissue sarcomas remains controversial. […] UPS carries a significant risk of recurrence, even following complete resection.

• #82 UNDIFFERENTIATED PLEOMORPHIC SARCOMA – MALIGNANT FIBROUS HISTIOCYTOMA

  https://www.seyitaligumustas.com/en/undifferentiated-pleomorphic-sarcoma-malignant-fibrous-histiocytoma

  Undifferentiated pleomorphic sarcoma is the most common malignant soft tissue tumor. The exact cause and tissue of origin are unknown. […] The main treatment for undifferentiated pleomorphic sarcoma is clean surgical removal of the tumor. The tumor that is not removed cleanly with wide margins has a nearly one hundred percent recurrence rate and is prone to metastasis. […] Radiation therapy is used before or after surgery to facilitate surgery and reduce the possibility of recurrence. […] Chemotherapy is not routinely used for undifferentiated pleomorphic sarcoma. Chemotherapy is used especially in the presence of metastases (often to the lungs) and in large and deep-seated tumors. […] The five-year survival rate for patients with undifferentiated pleomorphic sarcoma is 60-65%. Life expectancy is lower for deep-seated and large tumors, especially in the presence of metastases. […] Patients diagnosed with undifferentiated pleomorphic sarcoma should be followed for many years at regular intervals for recurrence and metastasis, which means spread to other parts of the body.

• #83 Moving the Needle in Undifferentiated Pleomorphic Sarcoma: Overview of Paradigm

  https://www.onclive.com/view/moving-the-needle-in-undifferentiated-pleomorphic-sarcoma-overview-of-paradigm

  Undifferentiated pleomorphic sarcoma, or UPS, is a type of high-grade malignant soft tissue tumor thats characterized by significant cellular pleomorphism and the absence of a specific line of differentiation. Historically known as malignant fibrous histiocytoma, it is now classified as undifferentiated pleomorphic sarcoma. UPS typically presents as a rapidly growing mass, often located in the extremities or retroperitoneum. It is known for its aggressive behavior with a high propensity for local recurrence and distant metastases. The diagnosis of UPS is one of exclusion, made after ruling out other specific types of sarcomas through histologic assessment, immunohistochemistry, and molecular genetic studies. […] Recently, immunotherapy, radiation therapy, and surgery are emerging as a growing and promising combination for the treatment of UPS of the extremities. Pembrolizumab [Keytruda], an antiPD-1 immune checkpoint inhibitor, has shown efficacy in various malignancies, including soft tissue sarcomas. The Stand Up to Cancer Sarcoma Alliance for Research through Collaboration [SARC] 032 trial demonstrated that the addition of pembrolizumab to preoperative radiotherapy and surgery significantly improved disease-free survival in patients with stage III UPS of the extremities compared with radiation and surgery alone.

• #84 Moving the Needle in Undifferentiated Pleomorphic Sarcoma: Overview of Paradigm

  https://www.onclive.com/view/moving-the-needle-in-undifferentiated-pleomorphic-sarcoma-overview-of-paradigm

  Undifferentiated pleomorphic sarcoma, or UPS, is a type of high-grade malignant soft tissue tumor thats characterized by significant cellular pleomorphism and the absence of a specific line of differentiation. Historically known as malignant fibrous histiocytoma, it is now classified as undifferentiated pleomorphic sarcoma. UPS typically presents as a rapidly growing mass, often located in the extremities or retroperitoneum. It is known for its aggressive behavior with a high propensity for local recurrence and distant metastases. The diagnosis of UPS is one of exclusion, made after ruling out other specific types of sarcomas through histologic assessment, immunohistochemistry, and molecular genetic studies. […] Recently, immunotherapy, radiation therapy, and surgery are emerging as a growing and promising combination for the treatment of UPS of the extremities. Pembrolizumab [Keytruda], an antiPD-1 immune checkpoint inhibitor, has shown efficacy in various malignancies, including soft tissue sarcomas. The Stand Up to Cancer Sarcoma Alliance for Research through Collaboration [SARC] 032 trial demonstrated that the addition of pembrolizumab to preoperative radiotherapy and surgery significantly improved disease-free survival in patients with stage III UPS of the extremities compared with radiation and surgery alone.

• #85 UNDIFFERENTIATED PLEOMORPHIC SARCOMA – MALIGNANT FIBROUS HISTIOCYTOMA

  https://www.seyitaligumustas.com/en/undifferentiated-pleomorphic-sarcoma-malignant-fibrous-histiocytoma

  Undifferentiated pleomorphic sarcoma is the most common malignant soft tissue tumor. The exact cause and tissue of origin are unknown. […] The main treatment for undifferentiated pleomorphic sarcoma is clean surgical removal of the tumor. The tumor that is not removed cleanly with wide margins has a nearly one hundred percent recurrence rate and is prone to metastasis. […] Radiation therapy is used before or after surgery to facilitate surgery and reduce the possibility of recurrence. […] Chemotherapy is not routinely used for undifferentiated pleomorphic sarcoma. Chemotherapy is used especially in the presence of metastases (often to the lungs) and in large and deep-seated tumors. […] The five-year survival rate for patients with undifferentiated pleomorphic sarcoma is 60-65%. Life expectancy is lower for deep-seated and large tumors, especially in the presence of metastases. […] Patients diagnosed with undifferentiated pleomorphic sarcoma should be followed for many years at regular intervals for recurrence and metastasis, which means spread to other parts of the body.

• #86 UNDIFFERENTIATED PLEOMORPHIC SARCOMA – MALIGNANT FIBROUS HISTIOCYTOMA

  https://www.seyitaligumustas.com/en/undifferentiated-pleomorphic-sarcoma-malignant-fibrous-histiocytoma

  Undifferentiated pleomorphic sarcoma is the most common malignant soft tissue tumor. The exact cause and tissue of origin are unknown. […] The main treatment for undifferentiated pleomorphic sarcoma is clean surgical removal of the tumor. The tumor that is not removed cleanly with wide margins has a nearly one hundred percent recurrence rate and is prone to metastasis. […] Radiation therapy is used before or after surgery to facilitate surgery and reduce the possibility of recurrence. […] Chemotherapy is not routinely used for undifferentiated pleomorphic sarcoma. Chemotherapy is used especially in the presence of metastases (often to the lungs) and in large and deep-seated tumors. […] The five-year survival rate for patients with undifferentiated pleomorphic sarcoma is 60-65%. Life expectancy is lower for deep-seated and large tumors, especially in the presence of metastases. […] Patients diagnosed with undifferentiated pleomorphic sarcoma should be followed for many years at regular intervals for recurrence and metastasis, which means spread to other parts of the body.

• #87 Numerical and Structural Chromosomal Anomalies in Undifferentiated Pleomorphic Sarcoma | Anticancer Research

  https://ar.iiarjournals.org/content/34/12/7119

  We investigated numerical and structural chromosome anomalies in undifferentiated pleomorphic sarcoma and examined five special chromosomal breaks, five potential gene amplifications as well as performed numerical interphase karyotyping in UPS and the UPS/MFH cell line (U2197). Chromosomal breaks/translocations in the genes CHOP, SYT, EWS and FKHR are characteristic for myxoid liposarcoma, synovial sarcomas, Ewing’s sarcoma/PNET and alveolar rhabdomyosarcoma, respectively. Our study demonstrates that UPS exhibit no chromosomal breaks of the CHOP, SYT, EWS, FUS and FKHR genes detectable by standard FISH probes. Absence of CHOP rearrangements has been independently reported in MFH. We propose that although the above-mentioned five different breaks/translocations may be relevant in the pathogenesis of other sarcoma entities, they play no critical role in the pathogenesis of UPS. To date, 41 gene fusions have been described in 17 different sarcoma types, but no specific chromosomal translocation has been reported in UPS. The present study reinforces this observation.

• #88 Undifferentiated Pleomorphic Sarcoma (Malignant Fibrous Histiocytoma) | Ento Key

  https://entokey.com/undifferentiated-pleomorphic-sarcoma-malignant-fibrous-histiocytoma/

  Undifferentiated pleomorphic sarcoma (UPS) describes a soft tissue tumor of primitive mesenchymal origin that involves deep skeletal muscle, fascia, and adipose tissue. […] The pathogenesis is unclear, but translocations and gene fusions common in the pathogenesis of other sarcomas have not been found to play a role in the pathogenesis of UPS. […] A well-known etiologic factor for UPS is ionizing radiation, and this has been reported following radiotherapy for breast carcinoma, malignant lymphoma, cervical carcinoma, and brain tumors. […] Interestingly, a variety of translocations and gene fusions common in the pathogenesis of other sarcomas have not been found to play a role in the pathogenesis of UPS.

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

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

  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. […] 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.

• #90 A Detailed Examination of Retroperitoneal Undifferentiated Pleomorphic Sarcoma: A Case Report and Review of the Existing Literature

  https://www.mdpi.com/2077-0383/13/13/3684

  Notably, a range of “cancer driver genes” such as tumor protein p53 (TP53), alpha-thalassemia/mental retardation syndrome X-linked (ATRX), H3 histone family member 3A (H3F3A), and zinc finger homeobox 3 (ZFHX3), among others, have been identified as critical to the onset and advancement of the disease. […] The inactivation of tumor suppressor genes is a frequent occurrence, with mutations in TP53 leading to the overexpression of p53. […] Additionally, the role of cellular senescence in UPS is highlighted by the homozygous deletion of cyclin-dependent kinase inhibitor 2A (p16INK4a), although it appears that p16 may not act as a predominant barrier to senescence in some UPS subtypes. […] From an epigenetic perspective, significant modifications include the methylation of sites around integrin subunit alpha 10 (ITGA10) and protein phosphatase 2 regulatory subunit B (PPP2R2B), which are upstream regulators of the Akt/mTOR (protein kinase B/mammalian target of rapamycin) signaling pathway.

• #91 Primary Culture of Undifferentiated Pleomorphic Sarcoma: Molecular Characterization and Response to Anticancer Agents

  https://www.mdpi.com/1422-0067/18/12/2662

  Undifferentiated pleomorphic sarcoma (UPS) is an aggressive mesenchymal neoplasm with no specific line of differentiation. […] Our understanding of the molecular biology of UPS is limited, as other STS, by the small number of cell lines available. […] Gene expression analysis of the tumor specimen confirmed the mesenchymal origin of this disease. In particular, vimentin and mmp2, both mesenchymal-related genes, were upregulated, whereas the expression of e-cadherin, an epithelial gene, was lower than that of matched healthy tissue. […] Furthermore, upregulation of genes involved in drug resistance, especially to anthracyclines, was observed. […] The significant anticancer activity of eribulin in the primary culture with respect to the first-line ifosfamide–epirubicin combination, prompted us to analyze the mechanism of action through which eribulin exerts its antitumor activity in UPS.

• #92 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. […] According to an experimental model, these cells have an improved potential for self-renewal, growth, and proliferation and can mimic tumor development. […] 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.

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