Materiały źródłowe

• #1 Soft Tissue Sarcoma: An Insight on Biomarkers at Molecular, Metabolic and Cellular Level

  https://www.mdpi.com/2072-6694/13/12/3044

  Soft tissue sarcomas (STSs) are a heterogeneous group of rare tumors. Although constituting only 1% of all human malignancies, STSs represent the second most common type of solid tumors in children and adolescents and comprise an important group of secondary malignancies. […] Recent scientific advancements have enabled a more precise molecular characterization of sarcoma subtypes and revealed novel therapeutic targets and prognostic/predictive biomarkers. […] As in most tumors, particularly in rare tumors, the etiology of STS is still largely unknown. There is, however, historical evidence of the association between sarcomas and various genetic syndromes as well as with radiotherapy; moreover, there are data indicating a possible role of environmental factors predisposing sarcoma development.

• #2 Benign and Malignant Soft-Tissue Tumors: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1253816-overview

  Soft-tissue tumors constitute a large and heterogeneous group of neoplasms. Traditionally, tumors have been classified according to histogenetic features. […] However, histomorphologic, immunohistochemical, and experimental data suggest that most, if not all, sarcomas arise from primitive, multipotential mesenchymal cells, which in the course of neoplastic transformation differentiate along one or more lines. […] Current achievements in the field of soft-tissue tumors are the result of advances in molecular biology, oncogenetics, imaging techniques, immunochemistry, diagnosis by fine-needle aspiration (FNA), surgical reconstruction, radiation therapy, and tissue banking. […] Future advances in molecular oncology may further improve diagnostic, prognostic, and treatment protocols for patients with soft-tissue sarcomas.

• #3 Soft tissue sarcoma – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/soft-tissue-sarcoma/symptoms-causes/syc-20377725

  Soft tissue sarcoma starts when a connective tissue cell gets changes in its DNA. A cell’s DNA holds the instructions that tell a cell what to do. The changes turn the connective tissue cells into cancer cells. The changes tell the cancer cells to grow and make more cells. Healthy cells die as part of their natural cycle, but cancer cells keep growing because they do not have instructions to stop. […] The type of cell with DNA changes is what determines the type of soft tissue sarcoma. For example, angiosarcoma begins in cells in the lining of blood vessels, while liposarcoma starts in fat cells.

• #4 Soft tissue sarcoma – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/soft-tissue-sarcoma/symptoms-causes/syc-20377725

  Soft tissue sarcoma starts when a connective tissue cell gets changes in its DNA. A cell’s DNA holds the instructions that tell a cell what to do. The changes turn the connective tissue cells into cancer cells. The changes tell the cancer cells to grow and make more cells. Healthy cells die as part of their natural cycle, but cancer cells keep growing because they do not have instructions to stop. […] The type of cell with DNA changes is what determines the type of soft tissue sarcoma. For example, angiosarcoma begins in cells in the lining of blood vessels, while liposarcoma starts in fat cells.

• #5 Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK519533/

  Soft tissue sarcomas (STS) are a group of neoplasms that can affect individuals at the extremes of age and can originate from any location throughout the human body. […] The majority of STS occurs spontaneously. However, germline mutations, radiation, and environmental exposure(s) have been causative. […] While most of these cases occur sporadically, several causative factors have been identified. […] Mutations in the NF1 gene result in multiple cutaneous neurofibromas. […] p53 functions to clear damaged cellular DNA. […] Mutant protein fails to inhibit this localization which results in unchecked cell cycling and cellular proliferation. […] A chronic lymphatic blockage is thought to stimulate the proliferation of lymphatics and vessels or lead to local immunodeficiency causing the development of malignancy.

• #6 What Causes Soft Tissue Sarcomas? | American Cancer Society

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

  Scientists don’t know exactly what causes most soft tissue sarcomas, but they have found some risk factors that can make a person more likely to develop these cancers. […] Research has shown that some of these risk factors affect the genes in cells in the soft tissues. […] Cancers can be caused by DNA mutations (defects) that turn on oncogenes or turn off tumor suppressor genes. […] DNA mutations in soft tissue sarcoma are common. But they’re usually acquired during life rather than having been inherited before birth. Acquired mutations may result from exposure to radiation or cancer-causing chemicals. […] Researchers still don’t know why most soft tissue sarcomas develop in people who have no apparent risk factors.

• #7 What Causes Soft Tissue Sarcomas? | American Cancer Society

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

  Scientists don’t know exactly what causes most soft tissue sarcomas, but they have found some risk factors that can make a person more likely to develop these cancers. […] Research has shown that some of these risk factors affect the genes in cells in the soft tissues. […] Cancers can be caused by DNA mutations (defects) that turn on oncogenes or turn off tumor suppressor genes. […] DNA mutations in soft tissue sarcoma are common. But they’re usually acquired during life rather than having been inherited before birth. Acquired mutations may result from exposure to radiation or cancer-causing chemicals. […] Researchers still don’t know why most soft tissue sarcomas develop in people who have no apparent risk factors.

• #8 The Molecular Biology of Soft Tissue Sarcomas: Current Knowledge and Future Perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9139698/

  The BAF complex is involved in chromatin remodeling and highlights the essential role of epigenetics in the pathogenesis of sarcomas. […] Molecular biology has allowed a refined classification of sarcomas with the definition of novel molecular entities and the identification of driver alterations in recent years. […] While gene fusions in sarcomas often involve transcription factors which are not easily druggable, some fusions involve tyrosine kinase receptors which are molecular drivers of oncogenesis that can be directly targeted for molecularly-driven therapy. […] While sarcomas with mutations are less frequent than fusion-driven sarcomas, they are natural candidates for classical targeted precision medicine, with the paradigmatic successful example being GISTs. […] The most frequent sarcoma with gene amplification is liposarcoma which harbors MDM2 amplification on the 12q amplicon. […] Molecular biology has a prominent role in the diagnostic and therapeutic management of numerous soft tissue sarcoma subtypes.

• #9 The Molecular Biology of Soft Tissue Sarcomas: Current Knowledge and Future Perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9139698/

  Soft tissue sarcomas are malignant tumors of mesenchymal origin, encompassing a large spectrum of entities that were historically classified according to their histological characteristics. […] Indeed, while sarcomas can be classified according to their microscopic appearance and probable mesenchymal cell-of-origin, it has become clear that many subtypes are characterized by specific genomic alterations that not only help to refine the diagnostic process but most importantly to understand their oncogenesis, which is paramount for prognosis and treatment. […] For the group of sarcomas with a driver alteration, molecular biology is logically essential for their accurate diagnosis and characterization. […] While gene fusions constitute the most frequent molecular alterations in sarcomas, some subtypes are characterized by mutations of specific genes, either oncogenesis driver genes (activating mutations), or tumor suppressor genes (inactivating mutations).

• #10

  https://www.orthobullets.com/pathology/8049/soft-tissue-sarcoma

  Soft tissue sarcomas are rare, malignant tumors comprising of a variety of subtypes distinguished by histological findings. […] Diagnosis is made by biopsy and histological findings. […] Etiology includes classic translocations such as t(X;18) leading to SYT-SSX fusion in synovial sarcoma, t(2:13) in rhabdomyosarcoma, and t(12;16)(q13:p11) in myxoid liposarcoma. […] Associated conditions include neurofibromatosis type-1, which can lead to malignant peripheral nerve sheath tumor (MPNST), and chronic lymphedema, which is associated with angiosarcoma. […] Classification of STS is based on histology, with over 50 types recognized, and histologic findings and molecular signatures are used to distinguish individual types. […] Poor prognostic factors include high-grade tumors, metastatic disease, size greater than 5 cm, tumor location below the deep fascia, and delay in diagnosis.

• #11

  https://www.orthobullets.com/pathology/8052/synovial-sarcoma

  Synovial Sarcoma is a malignant, soft tissue sarcoma caused by a t(X;18) chromosomal translocation mutation ( SS18:SSX fusion protein) most commonly found near joints but rarely within the joint. […] Mechanism: mesenchymal soft tissue sarcoma with unknown cellular origin; synovial sarcoma is a misnomer due to the tumor’s microscopic resemblance to mature synovium. […] Chromosomal translocation t(X;18) is observed in more than 90% of cases. […] Translocation forms the SYT-SSX1, 2, or 4 fusion protein. […] Fusion proteins bind to BAF complex, which displaces the tumor suppressor BAF47; new BAF complex activates Sox2, which leads to tumor formation. […] Cellular origin of synovial sarcoma is unknown.

• #12

  https://www.orthobullets.com/pathology/8052/synovial-sarcoma

  Synovial Sarcoma is a malignant, soft tissue sarcoma caused by a t(X;18) chromosomal translocation mutation ( SS18:SSX fusion protein) most commonly found near joints but rarely within the joint. […] Mechanism: mesenchymal soft tissue sarcoma with unknown cellular origin; synovial sarcoma is a misnomer due to the tumor’s microscopic resemblance to mature synovium. […] Chromosomal translocation t(X;18) is observed in more than 90% of cases. […] Translocation forms the SYT-SSX1, 2, or 4 fusion protein. […] Fusion proteins bind to BAF complex, which displaces the tumor suppressor BAF47; new BAF complex activates Sox2, which leads to tumor formation. […] Cellular origin of synovial sarcoma is unknown.

• #13 The Molecular Biology of Soft Tissue Sarcomas: Current Knowledge and Future Perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9139698/

  The BAF complex is involved in chromatin remodeling and highlights the essential role of epigenetics in the pathogenesis of sarcomas. […] Molecular biology has allowed a refined classification of sarcomas with the definition of novel molecular entities and the identification of driver alterations in recent years. […] While gene fusions in sarcomas often involve transcription factors which are not easily druggable, some fusions involve tyrosine kinase receptors which are molecular drivers of oncogenesis that can be directly targeted for molecularly-driven therapy. […] While sarcomas with mutations are less frequent than fusion-driven sarcomas, they are natural candidates for classical targeted precision medicine, with the paradigmatic successful example being GISTs. […] The most frequent sarcoma with gene amplification is liposarcoma which harbors MDM2 amplification on the 12q amplicon. […] Molecular biology has a prominent role in the diagnostic and therapeutic management of numerous soft tissue sarcoma subtypes.

• #14 Pathogenetic factors in soft tissue and bone sarcomas – UpToDate

  https://www.uptodate.com/contents/pathogenetic-factors-in-soft-tissue-and-bone-sarcomas

  Pathogenetic factors in soft tissue and bone sarcomas […] There is no clearly defined etiology in most cases of soft tissue sarcoma, but a number of associated or predisposing factors have been identified. These include a genetic predisposition, gene mutations, radiation therapy (RT), chemotherapy, chemical carcinogens, chronic irritation, and lymphedema. In addition, an association between viral infection and sarcoma has been shown for human herpesvirus 8 (HHV-8) in Kaposi sarcoma, and for Epstein-Barr virus (EBV) and smooth muscle tumors in immunocompromised patients. […] Some patients with bone and soft tissue sarcomas, particularly children, have a genetic predisposition to cancer. In some cases, individuals are from families with a defined inherited predisposing condition, such as Li-Fraumeni syndrome (LFS) or retinoblastoma, but many cases do not fit recognized inherited cancer syndromes. One analysis, in which 1162 patients with sarcoma, unselected for family history, underwent targeted exon sequencing of 72 genes selected for their association with cancer risk, concluded that approximately one-half of the patients had putatively pathogenic, monogenic, and polygenic variation in known and novel cancer genes. In a pooled analysis of all sarcoma probands, 240 carried multiple variants, suggesting a polygenic contribution to sarcoma risk. Only 155 (17 percent) of the 911 families with informative pedigrees fit recognizable cancer syndromes.

• #15 Pathogenetic factors in soft tissue and bone sarcomas – UpToDate

  https://www.uptodate.com/contents/pathogenetic-factors-in-soft-tissue-and-bone-sarcomas

  Pathogenetic factors in soft tissue and bone sarcomas […] There is no clearly defined etiology in most cases of soft tissue sarcoma, but a number of associated or predisposing factors have been identified. These include a genetic predisposition, gene mutations, radiation therapy (RT), chemotherapy, chemical carcinogens, chronic irritation, and lymphedema. In addition, an association between viral infection and sarcoma has been shown for human herpesvirus 8 (HHV-8) in Kaposi sarcoma, and for Epstein-Barr virus (EBV) and smooth muscle tumors in immunocompromised patients. […] Some patients with bone and soft tissue sarcomas, particularly children, have a genetic predisposition to cancer. In some cases, individuals are from families with a defined inherited predisposing condition, such as Li-Fraumeni syndrome (LFS) or retinoblastoma, but many cases do not fit recognized inherited cancer syndromes. One analysis, in which 1162 patients with sarcoma, unselected for family history, underwent targeted exon sequencing of 72 genes selected for their association with cancer risk, concluded that approximately one-half of the patients had putatively pathogenic, monogenic, and polygenic variation in known and novel cancer genes. In a pooled analysis of all sarcoma probands, 240 carried multiple variants, suggesting a polygenic contribution to sarcoma risk. Only 155 (17 percent) of the 911 families with informative pedigrees fit recognizable cancer syndromes.

• #16 Pathogenetic factors in soft tissue and bone sarcomas – UpToDate

  https://www.uptodate.com/contents/pathogenetic-factors-in-soft-tissue-and-bone-sarcomas

  Li-Fraumeni syndrome — Mutations in TP53 are the most common germline mutations that predispose to pediatric sarcomas, including osteosarcoma, undifferentiated pleomorphic sarcoma, rhabdomyosarcoma, leiomyosarcoma, and liposarcoma. As many as 7 percent of children with soft tissue sarcomas may have LFS. In a series of 151 children with soft tissue sarcomas, for example, five of the families (3.3 percent) manifested the classic LFS familial cancer syndrome, 10 (6.6 percent) had features consistent with the syndrome, and 16 (10.5 percent) had one parent with a possible hereditary cancer syndrome or with cancer before the age of 60.

• #17 Pathogenetic factors in soft tissue and bone sarcomas – UpToDate

  https://www.uptodate.com/contents/pathogenetic-factors-in-soft-tissue-and-bone-sarcomas

  Li-Fraumeni syndrome — Mutations in TP53 are the most common germline mutations that predispose to pediatric sarcomas, including osteosarcoma, undifferentiated pleomorphic sarcoma, rhabdomyosarcoma, leiomyosarcoma, and liposarcoma. As many as 7 percent of children with soft tissue sarcomas may have LFS. In a series of 151 children with soft tissue sarcomas, for example, five of the families (3.3 percent) manifested the classic LFS familial cancer syndrome, 10 (6.6 percent) had features consistent with the syndrome, and 16 (10.5 percent) had one parent with a possible hereditary cancer syndrome or with cancer before the age of 60.

• #18 Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK519533/

  Soft tissue sarcomas (STS) are a group of neoplasms that can affect individuals at the extremes of age and can originate from any location throughout the human body. […] The majority of STS occurs spontaneously. However, germline mutations, radiation, and environmental exposure(s) have been causative. […] While most of these cases occur sporadically, several causative factors have been identified. […] Mutations in the NF1 gene result in multiple cutaneous neurofibromas. […] p53 functions to clear damaged cellular DNA. […] Mutant protein fails to inhibit this localization which results in unchecked cell cycling and cellular proliferation. […] A chronic lymphatic blockage is thought to stimulate the proliferation of lymphatics and vessels or lead to local immunodeficiency causing the development of malignancy.

• #19

  https://www.orthobullets.com/pathology/8049/soft-tissue-sarcoma

  Soft tissue sarcomas are rare, malignant tumors comprising of a variety of subtypes distinguished by histological findings. […] Diagnosis is made by biopsy and histological findings. […] Etiology includes classic translocations such as t(X;18) leading to SYT-SSX fusion in synovial sarcoma, t(2:13) in rhabdomyosarcoma, and t(12;16)(q13:p11) in myxoid liposarcoma. […] Associated conditions include neurofibromatosis type-1, which can lead to malignant peripheral nerve sheath tumor (MPNST), and chronic lymphedema, which is associated with angiosarcoma. […] Classification of STS is based on histology, with over 50 types recognized, and histologic findings and molecular signatures are used to distinguish individual types. […] Poor prognostic factors include high-grade tumors, metastatic disease, size greater than 5 cm, tumor location below the deep fascia, and delay in diagnosis.

• #20 Germline predisposition to soft tissue sarcoma

  https://www.oaepublish.com/articles/2394-4722.2022.31

  In conclusion, CPS has been identified in up to 8% of RMS. […] Malignant peripheral nerve sheath tumors (MPNST) occur sporadically or are in association with CPS, notably neurofibromatosis type 1 (NF1). […] In conclusion, MPNST is most highly associated with NF1. […] There is a notable absence of Ewing sarcoma risk in CPS. […] In conclusion, Ewing sarcoma risk has not been identified within a singular CPS, but may exist in the group of DNA repair genes and their associated pathways. […] Liposarcoma has some associations with CPS, notably LFS. […] In conclusion, risks for a CPS in individuals diagnosed with liposarcoma are unknown. […] Leiomyosarcoma (LMS) has a definite association with cancer predisposition, but there remains a lack of details about germline contribution despite extensive work studying somatic variants.

• #21 Pathogenetic factors in soft tissue and bone sarcomas – UpToDate

  https://www.uptodate.com/contents/pathogenetic-factors-in-soft-tissue-and-bone-sarcomas

  Pathogenetic factors in soft tissue and bone sarcomas […] There is no clearly defined etiology in most cases of soft tissue sarcoma, but a number of associated or predisposing factors have been identified. These include a genetic predisposition, gene mutations, radiation therapy (RT), chemotherapy, chemical carcinogens, chronic irritation, and lymphedema. In addition, an association between viral infection and sarcoma has been shown for human herpesvirus 8 (HHV-8) in Kaposi sarcoma, and for Epstein-Barr virus (EBV) and smooth muscle tumors in immunocompromised patients. […] Some patients with bone and soft tissue sarcomas, particularly children, have a genetic predisposition to cancer. In some cases, individuals are from families with a defined inherited predisposing condition, such as Li-Fraumeni syndrome (LFS) or retinoblastoma, but many cases do not fit recognized inherited cancer syndromes. One analysis, in which 1162 patients with sarcoma, unselected for family history, underwent targeted exon sequencing of 72 genes selected for their association with cancer risk, concluded that approximately one-half of the patients had putatively pathogenic, monogenic, and polygenic variation in known and novel cancer genes. In a pooled analysis of all sarcoma probands, 240 carried multiple variants, suggesting a polygenic contribution to sarcoma risk. Only 155 (17 percent) of the 911 families with informative pedigrees fit recognizable cancer syndromes.

• #22 Pathogenetic factors in soft tissue and bone sarcomas – UpToDate

  https://www.uptodate.com/contents/pathogenetic-factors-in-soft-tissue-and-bone-sarcomas

  Pathogenetic factors in soft tissue and bone sarcomas […] There is no clearly defined etiology in most cases of soft tissue sarcoma, but a number of associated or predisposing factors have been identified. These include a genetic predisposition, gene mutations, radiation therapy (RT), chemotherapy, chemical carcinogens, chronic irritation, and lymphedema. In addition, an association between viral infection and sarcoma has been shown for human herpesvirus 8 (HHV-8) in Kaposi sarcoma, and for Epstein-Barr virus (EBV) and smooth muscle tumors in immunocompromised patients. […] Some patients with bone and soft tissue sarcomas, particularly children, have a genetic predisposition to cancer. In some cases, individuals are from families with a defined inherited predisposing condition, such as Li-Fraumeni syndrome (LFS) or retinoblastoma, but many cases do not fit recognized inherited cancer syndromes. One analysis, in which 1162 patients with sarcoma, unselected for family history, underwent targeted exon sequencing of 72 genes selected for their association with cancer risk, concluded that approximately one-half of the patients had putatively pathogenic, monogenic, and polygenic variation in known and novel cancer genes. In a pooled analysis of all sarcoma probands, 240 carried multiple variants, suggesting a polygenic contribution to sarcoma risk. Only 155 (17 percent) of the 911 families with informative pedigrees fit recognizable cancer syndromes.

• #23 Soft tissue sarcoma: clinical recognition and approach to the loneliest cancer

  https://www.explorationpub.com/Journals/emd/Article/100734

  Soft tissue sarcoma (STS) is a rare malignancy with a high incidence. […] The aetiology of STS is mostly unknown but is thought to be sporadic in nature with a minor contribution from environmental influences, irradiation, viral infections, immunodeficiency and genetic susceptibility. […] The largest environmental contributor is exposure to ionizing radiation accounting for only 36% of all sarcomas. […] The clinical symptoms observed in patients with STS are nonspecific, with the most common finding being a painless, gradually enlarging mass with site-dependent symptoms of increased pressure, such as paraesthesia and distal edema. […] The best individual indicator of increased malignancy risk is an increase in tumor size. […] An accurate differential diagnosis can be obtained after a comprehensive history and physical examination, including examination of the site, size, shape, contour, consistency, tenderness and tethering.

• #24 Benign and Malignant Soft-Tissue Tumors: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1253816-overview

  The pathogenetic mechanism is the emergence of radiation-induced genetic mutations that encourage neoplastic transformation. […] Many tumor suppressor genes, oncogenes, and cytogenetic defects are now associated with various soft-tissue sarcomas. […] The lung is by far the most common site of metastasis, which occurs in up to 52% of patients with high-grade lesions. […] Although most patients do not have clinically evident metastases at the time of presentation, they may have occult micrometastases that eventually manifest clinically. […] The relation between trauma and soft-tissue tumors appears to be coincidental.

• #25 Benign and Malignant Soft-Tissue Tumors: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1253816-overview

  The pathogenetic mechanism is the emergence of radiation-induced genetic mutations that encourage neoplastic transformation. […] Many tumor suppressor genes, oncogenes, and cytogenetic defects are now associated with various soft-tissue sarcomas. […] The lung is by far the most common site of metastasis, which occurs in up to 52% of patients with high-grade lesions. […] Although most patients do not have clinically evident metastases at the time of presentation, they may have occult micrometastases that eventually manifest clinically. […] The relation between trauma and soft-tissue tumors appears to be coincidental.

• #26 Soft Tissue Sarcomas | VCA Animal Hospitals

  https://vcahospitals.com/know-your-pet/soft-tissue-sarcomas

  Soft tissue sarcomas are a broad category of tumors including those that arise from the connective, muscle, or nervous tissues in dogs and cats. These tumors are the result of abnormal production of these cell types in an uncontrolled manner. […] For most cases of soft tissue sarcomas, no direct cause has been determined for their development. […] In cats exposed to a form of the feline leukemia virus (called feline sarcoma virus), the development of sarcomas on the head and neck sometimes occurs. […] Typically, the higher the grade (these tumors are graded from I to III) the more likely that spread is possible. However, one of the biggest concerns with soft tissue sarcomas is their ability to invade the local surrounding tissues. […] Because these tumors typically produce tentacles of abnormal cells, wide margins (the amount of tissue that needs to be removed) must be obtained for the best control of the tumor.

• #27 Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK519533/

  Soft tissue sarcomas (STS) are a group of neoplasms that can affect individuals at the extremes of age and can originate from any location throughout the human body. […] The majority of STS occurs spontaneously. However, germline mutations, radiation, and environmental exposure(s) have been causative. […] While most of these cases occur sporadically, several causative factors have been identified. […] Mutations in the NF1 gene result in multiple cutaneous neurofibromas. […] p53 functions to clear damaged cellular DNA. […] Mutant protein fails to inhibit this localization which results in unchecked cell cycling and cellular proliferation. […] A chronic lymphatic blockage is thought to stimulate the proliferation of lymphatics and vessels or lead to local immunodeficiency causing the development of malignancy.

• #28

  https://www.vin.com/apputil/content/defaultadv1.aspx?pId=19840&catId=105943&id=8250202&ind=562&objTypeID=17

  Soft tissue sarcoma (STS) is a catch-all classification referring to tumors that arise from the embryonic mesoderm and, as such, can occur anywhere in the body. […] Many subcutaneous soft-tissue lesions are benign or inflammatory, but STS comprise 15% of all skin and subcutaneous tumors in dogs and 79% in cats. […] Many STS feel encapsulated and distinct from surrounding tissues. This is because they are surrounded by a pseudocapsule, which creates an easy plane of cleavage between tumor and surrounding tissues, allowing the masses to be 'shelled-out.’ […] The pseudocapsule arises, however, because expanding tumors are histologically surrounded by a poorly defined reactive zone, consisting of some or all of a vascular response (new blood vessels), mesenchymal response (to physical presence of the tumor and abnormal local tissue forces), and an inflammatory response (to necrosis/hemorrhage).

• #29 Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK519533/

  Sarcomas are connective tissue tumors, and thus the tumors may occur in bone, cartilage, fat, muscle, or vascular or hematopoietic tissues. […] Most tend to grow locally and invade the adjacent tissues. […] The most common presentation is a patient with a painless mass that requires a detailed history and physical examination upon initial evaluation. […] Neoplasms that are small, superficial, and mobile are highly suggestive of a soft tissue sarcoma. […] Indications for preoperative imaging and biopsy consider the extent of the mass on physical examination and the anticipated neurovascular involvement. […] The high risk of recurrence warrants close postoperative surveillance with a physical exam every 3 to 6 months for 2 to 3 years and, after that, every six months for the next two years and finally annually.

• #30

  https://www.vin.com/apputil/content/defaultadv1.aspx?pId=19840&catId=105943&id=8250202&ind=562&objTypeID=17

  Soft tissue sarcoma (STS) is a catch-all classification referring to tumors that arise from the embryonic mesoderm and, as such, can occur anywhere in the body. […] Many subcutaneous soft-tissue lesions are benign or inflammatory, but STS comprise 15% of all skin and subcutaneous tumors in dogs and 79% in cats. […] Many STS feel encapsulated and distinct from surrounding tissues. This is because they are surrounded by a pseudocapsule, which creates an easy plane of cleavage between tumor and surrounding tissues, allowing the masses to be 'shelled-out.’ […] The pseudocapsule arises, however, because expanding tumors are histologically surrounded by a poorly defined reactive zone, consisting of some or all of a vascular response (new blood vessels), mesenchymal response (to physical presence of the tumor and abnormal local tissue forces), and an inflammatory response (to necrosis/hemorrhage).

• #31

  https://www.vin.com/apputil/content/defaultadv1.aspx?pId=19840&catId=105943&id=8250202&ind=562&objTypeID=17

  Soft tissue sarcoma (STS) is a catch-all classification referring to tumors that arise from the embryonic mesoderm and, as such, can occur anywhere in the body. […] Many subcutaneous soft-tissue lesions are benign or inflammatory, but STS comprise 15% of all skin and subcutaneous tumors in dogs and 79% in cats. […] Many STS feel encapsulated and distinct from surrounding tissues. This is because they are surrounded by a pseudocapsule, which creates an easy plane of cleavage between tumor and surrounding tissues, allowing the masses to be 'shelled-out.’ […] The pseudocapsule arises, however, because expanding tumors are histologically surrounded by a poorly defined reactive zone, consisting of some or all of a vascular response (new blood vessels), mesenchymal response (to physical presence of the tumor and abnormal local tissue forces), and an inflammatory response (to necrosis/hemorrhage).

• #32

  https://www.vin.com/apputil/content/defaultadv1.aspx?pId=19840&catId=105943&id=8250202&ind=562&objTypeID=17

  This reactive zone creates a visible and palpable 'edge’ to the tumor, mimicking a fascial plane, but microscopically or immunohistochemically it is a three-dimensional 'halo’ of malignancy. […] Dissections within the reactive zone (i.e., shelling-out) are therefore marginal excisions which commonly result in tumor cells extending to the edges of the resected tissue, or satellite deposits of tumor cells in the reactive zone are left in the wound. […] Many STS are found when still small in size but are often not investigated, as the owner or clinician assume them to be benign. […] A study of insured dogs in the UK identified lipomas in 318 per 100,000 dogs, with STS at 142 per 100,000. […] Feline vaccine-associated sarcomas (VAS) are seen 210 months after vaccination and are on average 4 cm in diameter at diagnosis, often with a history of rapid growth.

• #33

  https://www.vin.com/apputil/content/defaultadv1.aspx?pId=19840&catId=105943&id=8250202&ind=562&objTypeID=17

  This reactive zone creates a visible and palpable 'edge’ to the tumor, mimicking a fascial plane, but microscopically or immunohistochemically it is a three-dimensional 'halo’ of malignancy. […] Dissections within the reactive zone (i.e., shelling-out) are therefore marginal excisions which commonly result in tumor cells extending to the edges of the resected tissue, or satellite deposits of tumor cells in the reactive zone are left in the wound. […] Many STS are found when still small in size but are often not investigated, as the owner or clinician assume them to be benign. […] A study of insured dogs in the UK identified lipomas in 318 per 100,000 dogs, with STS at 142 per 100,000. […] Feline vaccine-associated sarcomas (VAS) are seen 210 months after vaccination and are on average 4 cm in diameter at diagnosis, often with a history of rapid growth.

• #34 Mechanism-Driven Science Informs Novel Treatments in Soft Tissue Sarcoma

  https://www.targetedonc.com/view/mechanism-driven-science-informs-novel-treatments-in-soft-tissue-sarcoma

  Various combination strategies are being explored in soft tissue sarcomas in efforts to increase efficacy. […] Van Tine, a professor of medicine and sarcoma program director at Washington University School of Medicine in St Louis, Missouri, explained that the field is moving toward mechanism-driven science, with preclinical exploration of the biology of STS, including immune system biology and genomics, informing clinical trials. […] The hypothesis is that the targeted agents and the chemotherapy can prime the tumors, allowing the ICIs to act afterward, explained Van Tine. […] These results also suggest that a longer lead-in time with chemotherapy helps prime the tumor for immunotherapy. […] The immune micro environment is highly variable in STS, with some subtypes having strong immune presence, which offers a promise for immunotherapy in those malignancies, whereas other subtypes are classified as cold tumors.

• #35 Mechanism-Driven Science Informs Novel Treatments in Soft Tissue Sarcoma

  https://www.targetedonc.com/view/mechanism-driven-science-informs-novel-treatments-in-soft-tissue-sarcoma

  Wilky emphasized that we dont really know how to sequence these therapies for STS yet. […] The rarity and the heterogeneity contribute to the challenges faced by experts in the field, a fact that both Wilky and Van Tine highlighted. […] Evidence from preclinical studies suggests that TTI621 may enhance the response to doxorubicin in macrophage-rich tumors that express CD47, such as LMS. […] Preclinical and clinical data have shown that the combination of antiangiogenic agents and chemotherapy results in a synergistic effect. […] The primary end point was progression-free rate (PFR) at 12 weeks for cohort 1 (uterine and nonuterine LMS), which was 45.9%, with a median PFS of 6.4 months. […] This combination may help when tumors are of borderline resectability or when function preservation is a goal.

• #36 Mechanism-Driven Science Informs Novel Treatments in Soft Tissue Sarcoma

  https://www.targetedonc.com/view/mechanism-driven-science-informs-novel-treatments-in-soft-tissue-sarcoma

  Various combination strategies are being explored in soft tissue sarcomas in efforts to increase efficacy. […] Van Tine, a professor of medicine and sarcoma program director at Washington University School of Medicine in St Louis, Missouri, explained that the field is moving toward mechanism-driven science, with preclinical exploration of the biology of STS, including immune system biology and genomics, informing clinical trials. […] The hypothesis is that the targeted agents and the chemotherapy can prime the tumors, allowing the ICIs to act afterward, explained Van Tine. […] These results also suggest that a longer lead-in time with chemotherapy helps prime the tumor for immunotherapy. […] The immune micro environment is highly variable in STS, with some subtypes having strong immune presence, which offers a promise for immunotherapy in those malignancies, whereas other subtypes are classified as cold tumors.

• #37 Mechanism-Driven Science Informs Novel Treatments in Soft Tissue Sarcoma

  https://www.targetedonc.com/view/mechanism-driven-science-informs-novel-treatments-in-soft-tissue-sarcoma

  Various combination strategies are being explored in soft tissue sarcomas in efforts to increase efficacy. […] Van Tine, a professor of medicine and sarcoma program director at Washington University School of Medicine in St Louis, Missouri, explained that the field is moving toward mechanism-driven science, with preclinical exploration of the biology of STS, including immune system biology and genomics, informing clinical trials. […] The hypothesis is that the targeted agents and the chemotherapy can prime the tumors, allowing the ICIs to act afterward, explained Van Tine. […] These results also suggest that a longer lead-in time with chemotherapy helps prime the tumor for immunotherapy. […] The immune micro environment is highly variable in STS, with some subtypes having strong immune presence, which offers a promise for immunotherapy in those malignancies, whereas other subtypes are classified as cold tumors.

• #38 Medical Oncology: Canine Soft Tissue Sarcoma | Veterinary Hospital

  https://hospital.cvm.ncsu.edu/services/small-animals/cancer-oncology/oncology/canine-soft-tissue-sarcoma/

  Soft tissue sarcomas are tumors of connective tissues. Different soft tissue sarcomas are grouped together within this larger category because they have similar appearances on biopsy and similar clinical behavior in the patient. […] All soft tissue sarcomas have the potential to be locally invasive. This means they grow and invade surrounding structures. The chance for spread (metastasis) depends on the grade of the tumor. Grading is done on a biopsy sample. Regardless of grade, the most common site of spread is the lungs. The tumors can also spread to regional lymph nodes or other organs. […] The risk of regrowth after surgery and metastases depends on the tumor grade. Surgery alone can be curative for low/intermediate grade tumors. The prognosis for high grade soft tissue sarcomas is approximately one year. […] This treatment is reserved for high grade tumors that have the greatest likelihood to spread. It is generally ineffective against treating measurable tumors but is considered in cases where metastases has occurred at the time of diagnosis.

• #39 Medical Oncology: Canine Soft Tissue Sarcoma | Veterinary Hospital

  https://hospital.cvm.ncsu.edu/services/small-animals/cancer-oncology/oncology/canine-soft-tissue-sarcoma/

  Soft tissue sarcomas are tumors of connective tissues. Different soft tissue sarcomas are grouped together within this larger category because they have similar appearances on biopsy and similar clinical behavior in the patient. […] All soft tissue sarcomas have the potential to be locally invasive. This means they grow and invade surrounding structures. The chance for spread (metastasis) depends on the grade of the tumor. Grading is done on a biopsy sample. Regardless of grade, the most common site of spread is the lungs. The tumors can also spread to regional lymph nodes or other organs. […] The risk of regrowth after surgery and metastases depends on the tumor grade. Surgery alone can be curative for low/intermediate grade tumors. The prognosis for high grade soft tissue sarcomas is approximately one year. […] This treatment is reserved for high grade tumors that have the greatest likelihood to spread. It is generally ineffective against treating measurable tumors but is considered in cases where metastases has occurred at the time of diagnosis.

• #40 Benign and Malignant Soft-Tissue Tumors: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1253816-overview

  The pathogenetic mechanism is the emergence of radiation-induced genetic mutations that encourage neoplastic transformation. […] Many tumor suppressor genes, oncogenes, and cytogenetic defects are now associated with various soft-tissue sarcomas. […] The lung is by far the most common site of metastasis, which occurs in up to 52% of patients with high-grade lesions. […] Although most patients do not have clinically evident metastases at the time of presentation, they may have occult micrometastases that eventually manifest clinically. […] The relation between trauma and soft-tissue tumors appears to be coincidental.

• #41 Benign and Malignant Soft-Tissue Tumors: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1253816-overview

  The pathogenetic mechanism is the emergence of radiation-induced genetic mutations that encourage neoplastic transformation. […] Many tumor suppressor genes, oncogenes, and cytogenetic defects are now associated with various soft-tissue sarcomas. […] The lung is by far the most common site of metastasis, which occurs in up to 52% of patients with high-grade lesions. […] Although most patients do not have clinically evident metastases at the time of presentation, they may have occult micrometastases that eventually manifest clinically. […] The relation between trauma and soft-tissue tumors appears to be coincidental.

• #42 Diagnosis and management of canine soft tissue sarcomas – Veterinary Practice

  https://www.veterinary-practice.com/article/diagnosis-and-management-of-canine-soft-tissue-sarcomas

  Soft tissue sarcomas (STS) arise from the neoplastic transformation of mesenchymal cells and comprise around 15 percent of all skin and subcutaneous tumours in dogs (Theilen and Madewell, 1979). […] As well as occurring naturally, STS can develop secondary to radiation exposure, trauma, foreign bodies, implants and parasitic infection (Spirocerca lupi) (Liptak and Christensen, 2019). […] Soft tissue sarcomas are classified as either low grade (grade I), intermediate grade (grade II) or high grade (grade III) based on the degree of cellular differentiation, mitotic index and percentage necrosis (Dennis et al., 2011). […] If canine STS metastasise, it is typically via haematogenous spread to the lungs, with lymph node (LN) metastasis less common. […] Median survival times following surgery alone range between 2.5 and 5 years and are often not reached in many studies (Liptak and Christensen, 2019).

• #43

  https://link.springer.com/article/10.1007/s11547-018-0882-7

  Soft tissue sarcomas represent a heterogeneous group of rare malignancies exhibiting mesenchymal differentiation with an overall incidence of around 5/100,000/year. […] Soft tissue sarcomas are currently classified on the basis of the 2013 WHO classification of soft tissue tumors that integrate conventional morphology with immunohistochemistry and molecular genetics. […] Immunohistochemical characterization plays a key role in the diagnostic workup of soft tissue sarcomas. […] Molecular genetics is increasingly used for diagnostic purposes to distinguish specific subtypes of sarcomas, to support diagnosis in non-canonical clinical presentations and also to distinguish true sarcomas from benign mimickers. […] With many exceptions, histologic typing does not provide sufficient information for predicting the clinical course of the disease and, therefore, grading systems based on histological parameters were introduced to provide a more accurate estimation of the degree of malignancy of tumors. […] The three-tiered system devised by the French Federation of Cancer Centers Sarcoma Group (FNCLCC) systems is widely adopted; however, several limitations exist that have led to the development of prognostic nomograms that incorporate the specific histotype as one of the relevant parameters.

• #44

  https://link.springer.com/article/10.1007/s11547-018-0882-7

  Soft tissue sarcomas represent a heterogeneous group of rare malignancies exhibiting mesenchymal differentiation with an overall incidence of around 5/100,000/year. […] Soft tissue sarcomas are currently classified on the basis of the 2013 WHO classification of soft tissue tumors that integrate conventional morphology with immunohistochemistry and molecular genetics. […] Immunohistochemical characterization plays a key role in the diagnostic workup of soft tissue sarcomas. […] Molecular genetics is increasingly used for diagnostic purposes to distinguish specific subtypes of sarcomas, to support diagnosis in non-canonical clinical presentations and also to distinguish true sarcomas from benign mimickers. […] With many exceptions, histologic typing does not provide sufficient information for predicting the clinical course of the disease and, therefore, grading systems based on histological parameters were introduced to provide a more accurate estimation of the degree of malignancy of tumors. […] The three-tiered system devised by the French Federation of Cancer Centers Sarcoma Group (FNCLCC) systems is widely adopted; however, several limitations exist that have led to the development of prognostic nomograms that incorporate the specific histotype as one of the relevant parameters.

• #45

  https://link.springer.com/article/10.1007/s11547-018-0882-7

  Soft tissue sarcomas represent a heterogeneous group of rare malignancies exhibiting mesenchymal differentiation with an overall incidence of around 5/100,000/year. […] Soft tissue sarcomas are currently classified on the basis of the 2013 WHO classification of soft tissue tumors that integrate conventional morphology with immunohistochemistry and molecular genetics. […] Immunohistochemical characterization plays a key role in the diagnostic workup of soft tissue sarcomas. […] Molecular genetics is increasingly used for diagnostic purposes to distinguish specific subtypes of sarcomas, to support diagnosis in non-canonical clinical presentations and also to distinguish true sarcomas from benign mimickers. […] With many exceptions, histologic typing does not provide sufficient information for predicting the clinical course of the disease and, therefore, grading systems based on histological parameters were introduced to provide a more accurate estimation of the degree of malignancy of tumors. […] The three-tiered system devised by the French Federation of Cancer Centers Sarcoma Group (FNCLCC) systems is widely adopted; however, several limitations exist that have led to the development of prognostic nomograms that incorporate the specific histotype as one of the relevant parameters.

• #46 The Molecular Biology of Soft Tissue Sarcomas: Current Knowledge and Future Perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9139698/

  The BAF complex is involved in chromatin remodeling and highlights the essential role of epigenetics in the pathogenesis of sarcomas. […] Molecular biology has allowed a refined classification of sarcomas with the definition of novel molecular entities and the identification of driver alterations in recent years. […] While gene fusions in sarcomas often involve transcription factors which are not easily druggable, some fusions involve tyrosine kinase receptors which are molecular drivers of oncogenesis that can be directly targeted for molecularly-driven therapy. […] While sarcomas with mutations are less frequent than fusion-driven sarcomas, they are natural candidates for classical targeted precision medicine, with the paradigmatic successful example being GISTs. […] The most frequent sarcoma with gene amplification is liposarcoma which harbors MDM2 amplification on the 12q amplicon. […] Molecular biology has a prominent role in the diagnostic and therapeutic management of numerous soft tissue sarcoma subtypes.

• #47 The Molecular Biology of Soft Tissue Sarcomas: Current Knowledge and Future Perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9139698/

  The BAF complex is involved in chromatin remodeling and highlights the essential role of epigenetics in the pathogenesis of sarcomas. […] Molecular biology has allowed a refined classification of sarcomas with the definition of novel molecular entities and the identification of driver alterations in recent years. […] While gene fusions in sarcomas often involve transcription factors which are not easily druggable, some fusions involve tyrosine kinase receptors which are molecular drivers of oncogenesis that can be directly targeted for molecularly-driven therapy. […] While sarcomas with mutations are less frequent than fusion-driven sarcomas, they are natural candidates for classical targeted precision medicine, with the paradigmatic successful example being GISTs. […] The most frequent sarcoma with gene amplification is liposarcoma which harbors MDM2 amplification on the 12q amplicon. […] Molecular biology has a prominent role in the diagnostic and therapeutic management of numerous soft tissue sarcoma subtypes.

• #48 Molecular Diagnostics in Sarcoma… | College of American Pathologists

  https://www.cap.org/member-resources/articles/molecular-diagnostics-in-sarcoma-pathology

  Although malignant tumors of bone and soft tissue (i.e., sarcomas) are rare (approximately 1% of newly diagnosed cancers), they represent an outsized diagnostic challenge. This difficulty stems in part from the impressive diversity of these neoplasms which are comprised of at least 80 distinct types. Notably, a significant number of sarcomas harbor a characteristic gene fusion (e.g., SS18 fusions in synovial sarcoma, EWSR1-FL1 and related fusions in Ewing sarcoma, FOXO1 fusions in alveolar rhabdomyosarcoma, etc.). […] To address these limitations, molecular testing (i.e., testing DNA and/or RNA) has become increasingly common in diagnostic testing pathways for sarcomas and is often required for clinical trial protocols. […] The authors conclude that, for all sarcomas with recurrent genetic aberrations (e.g., synovial sarcoma, alveolar rhabdomyosarcoma), molecular testing should be performed.

• #49 Molecular Diagnostics in Sarcoma… | College of American Pathologists

  https://www.cap.org/member-resources/articles/molecular-diagnostics-in-sarcoma-pathology

  Although malignant tumors of bone and soft tissue (i.e., sarcomas) are rare (approximately 1% of newly diagnosed cancers), they represent an outsized diagnostic challenge. This difficulty stems in part from the impressive diversity of these neoplasms which are comprised of at least 80 distinct types. Notably, a significant number of sarcomas harbor a characteristic gene fusion (e.g., SS18 fusions in synovial sarcoma, EWSR1-FL1 and related fusions in Ewing sarcoma, FOXO1 fusions in alveolar rhabdomyosarcoma, etc.). […] To address these limitations, molecular testing (i.e., testing DNA and/or RNA) has become increasingly common in diagnostic testing pathways for sarcomas and is often required for clinical trial protocols. […] The authors conclude that, for all sarcomas with recurrent genetic aberrations (e.g., synovial sarcoma, alveolar rhabdomyosarcoma), molecular testing should be performed.

• #50 Molecular Diagnostics in Sarcoma… | College of American Pathologists

  https://www.cap.org/member-resources/articles/molecular-diagnostics-in-sarcoma-pathology

  Current World Health Organization (WHO) and National Comprehensive Cancer Center (NCCN) guidelines acknowledge the utility of molecular testing in sarcoma diagnosis. Notably, the most recent WHO Classification of Tumours of Soft tissue and Bone (2020) contains a considerable number of new tumor entities defined by a genetic alteration. […] Additionally, the new WHO guidelines now list essential and desirable diagnostic criteria for tumors. For all tumors with a defining genetic alteration, molecular confirmation of the alteration falls within the desirable category. […] Current NCCN Soft Tissue Sarcoma guidelines confirm the diagnostic utility of molecular genetic testing and recommend that such testing should be carried out by a pathologist with expertise in sarcoma diagnosis and molecular diagnostic techniques.

• #51 Molecular Diagnostics in Sarcoma… | College of American Pathologists

  https://www.cap.org/member-resources/articles/molecular-diagnostics-in-sarcoma-pathology

  Current World Health Organization (WHO) and National Comprehensive Cancer Center (NCCN) guidelines acknowledge the utility of molecular testing in sarcoma diagnosis. Notably, the most recent WHO Classification of Tumours of Soft tissue and Bone (2020) contains a considerable number of new tumor entities defined by a genetic alteration. […] Additionally, the new WHO guidelines now list essential and desirable diagnostic criteria for tumors. For all tumors with a defining genetic alteration, molecular confirmation of the alteration falls within the desirable category. […] Current NCCN Soft Tissue Sarcoma guidelines confirm the diagnostic utility of molecular genetic testing and recommend that such testing should be carried out by a pathologist with expertise in sarcoma diagnosis and molecular diagnostic techniques.

• #52 The Molecular Biology of Soft Tissue Sarcomas: Current Knowledge and Future Perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9139698/

  Soft tissue sarcomas are malignant tumors of mesenchymal origin, encompassing a large spectrum of entities that were historically classified according to their histological characteristics. […] Indeed, while sarcomas can be classified according to their microscopic appearance and probable mesenchymal cell-of-origin, it has become clear that many subtypes are characterized by specific genomic alterations that not only help to refine the diagnostic process but most importantly to understand their oncogenesis, which is paramount for prognosis and treatment. […] For the group of sarcomas with a driver alteration, molecular biology is logically essential for their accurate diagnosis and characterization. […] While gene fusions constitute the most frequent molecular alterations in sarcomas, some subtypes are characterized by mutations of specific genes, either oncogenesis driver genes (activating mutations), or tumor suppressor genes (inactivating mutations).

• #53

  https://www.orthobullets.com/pathology/8052/synovial-sarcoma

  Synovial Sarcoma is a malignant, soft tissue sarcoma caused by a t(X;18) chromosomal translocation mutation ( SS18:SSX fusion protein) most commonly found near joints but rarely within the joint. […] Mechanism: mesenchymal soft tissue sarcoma with unknown cellular origin; synovial sarcoma is a misnomer due to the tumor’s microscopic resemblance to mature synovium. […] Chromosomal translocation t(X;18) is observed in more than 90% of cases. […] Translocation forms the SYT-SSX1, 2, or 4 fusion protein. […] Fusion proteins bind to BAF complex, which displaces the tumor suppressor BAF47; new BAF complex activates Sox2, which leads to tumor formation. […] Cellular origin of synovial sarcoma is unknown.

• #54

  https://www.orthobullets.com/pathology/8049/soft-tissue-sarcoma

  Soft tissue sarcomas are rare, malignant tumors comprising of a variety of subtypes distinguished by histological findings. […] Diagnosis is made by biopsy and histological findings. […] Etiology includes classic translocations such as t(X;18) leading to SYT-SSX fusion in synovial sarcoma, t(2:13) in rhabdomyosarcoma, and t(12;16)(q13:p11) in myxoid liposarcoma. […] Associated conditions include neurofibromatosis type-1, which can lead to malignant peripheral nerve sheath tumor (MPNST), and chronic lymphedema, which is associated with angiosarcoma. […] Classification of STS is based on histology, with over 50 types recognized, and histologic findings and molecular signatures are used to distinguish individual types. […] Poor prognostic factors include high-grade tumors, metastatic disease, size greater than 5 cm, tumor location below the deep fascia, and delay in diagnosis.

• #55

  https://www.orthobullets.com/pathology/8049/soft-tissue-sarcoma

  Soft tissue sarcomas are rare, malignant tumors comprising of a variety of subtypes distinguished by histological findings. […] Diagnosis is made by biopsy and histological findings. […] Etiology includes classic translocations such as t(X;18) leading to SYT-SSX fusion in synovial sarcoma, t(2:13) in rhabdomyosarcoma, and t(12;16)(q13:p11) in myxoid liposarcoma. […] Associated conditions include neurofibromatosis type-1, which can lead to malignant peripheral nerve sheath tumor (MPNST), and chronic lymphedema, which is associated with angiosarcoma. […] Classification of STS is based on histology, with over 50 types recognized, and histologic findings and molecular signatures are used to distinguish individual types. […] Poor prognostic factors include high-grade tumors, metastatic disease, size greater than 5 cm, tumor location below the deep fascia, and delay in diagnosis.

• #56 The Molecular Biology of Soft Tissue Sarcomas: Current Knowledge and Future Perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9139698/

  The BAF complex is involved in chromatin remodeling and highlights the essential role of epigenetics in the pathogenesis of sarcomas. […] Molecular biology has allowed a refined classification of sarcomas with the definition of novel molecular entities and the identification of driver alterations in recent years. […] While gene fusions in sarcomas often involve transcription factors which are not easily druggable, some fusions involve tyrosine kinase receptors which are molecular drivers of oncogenesis that can be directly targeted for molecularly-driven therapy. […] While sarcomas with mutations are less frequent than fusion-driven sarcomas, they are natural candidates for classical targeted precision medicine, with the paradigmatic successful example being GISTs. […] The most frequent sarcoma with gene amplification is liposarcoma which harbors MDM2 amplification on the 12q amplicon. […] Molecular biology has a prominent role in the diagnostic and therapeutic management of numerous soft tissue sarcoma subtypes.

• #57 Sarcoma – Wikipedia

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

  Most soft-tissue sarcomas arise from what doctors call „sporadic” (or random) genetic mutations within an affected person’s cells. […] Nevertheless, there are certain risk factors associated with an increased risk of developing soft-tissue sarcoma. Previous exposure to ionizing radiation is one such risk factor. […] The precise molecular changes that result in sarcoma are not always known, but certain types of sarcomas are associated with particular genetic mutations. […] Most cases of Ewing sarcoma are associated with a chromosomal translocation in which part of chromosome 11 fuses with part of chromosome 22. […] Dermatofibrosarcoma protuberans often is associated with a chromosomal translocation in which the COL1A1 gene becomes fused to the PDGFRB gene. […] Many liposarcomas are associated with amplification of part of chromosome 12, which results in extra copies of known cancer-promoting genes („oncogenes”) such as the CDK4 gene, the MDM2 gene and the HMGA2 gene.

• #58 Sarcoma – Wikipedia

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

  Most soft-tissue sarcomas arise from what doctors call „sporadic” (or random) genetic mutations within an affected person’s cells. […] Nevertheless, there are certain risk factors associated with an increased risk of developing soft-tissue sarcoma. Previous exposure to ionizing radiation is one such risk factor. […] The precise molecular changes that result in sarcoma are not always known, but certain types of sarcomas are associated with particular genetic mutations. […] Most cases of Ewing sarcoma are associated with a chromosomal translocation in which part of chromosome 11 fuses with part of chromosome 22. […] Dermatofibrosarcoma protuberans often is associated with a chromosomal translocation in which the COL1A1 gene becomes fused to the PDGFRB gene. […] Many liposarcomas are associated with amplification of part of chromosome 12, which results in extra copies of known cancer-promoting genes („oncogenes”) such as the CDK4 gene, the MDM2 gene and the HMGA2 gene.

• #59 Sarcoma – Wikipedia

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

  Most soft-tissue sarcomas arise from what doctors call „sporadic” (or random) genetic mutations within an affected person’s cells. […] Nevertheless, there are certain risk factors associated with an increased risk of developing soft-tissue sarcoma. Previous exposure to ionizing radiation is one such risk factor. […] The precise molecular changes that result in sarcoma are not always known, but certain types of sarcomas are associated with particular genetic mutations. […] Most cases of Ewing sarcoma are associated with a chromosomal translocation in which part of chromosome 11 fuses with part of chromosome 22. […] Dermatofibrosarcoma protuberans often is associated with a chromosomal translocation in which the COL1A1 gene becomes fused to the PDGFRB gene. […] Many liposarcomas are associated with amplification of part of chromosome 12, which results in extra copies of known cancer-promoting genes („oncogenes”) such as the CDK4 gene, the MDM2 gene and the HMGA2 gene.

• #60

  https://journals.lww.com/ijpm/fulltext/2021/64030/recent_updates_in_the_diagnosis_of_soft_tissue.3.aspx

  Furthermore, two distinct tumors have been described, such as CIC-rearranged sarcoma, characterized by fusion genes, such as CIC-DUX4 and CIC-NUTM1, and sarcoma with BCOR-genetic alterations, such as BCOR-CCNB3, BCOR-MAML3-positive sarcomas. […] While BCOR-CCNB3 positive sarcomas mostly occur in the bones (commonly tibia) of young patients and histopathologically show the presence of short spindly cells with twisted nuclei, interspersed blood vessels, and myxoid change; CIC-rearranged tumors, including CIC-DUX4 positive sarcomas more commonly occur in the soft tissues of older patients and histopathologically display tumor cells with epithelioid morphology, prominent nucleoli, myxoid change, and a variable amount of myxoid change. […] The importance of identifying this subset of tumors is in view of their resistance to tyrosine kinase inhibitors, including imatinib.

• #61

  https://link.springer.com/article/10.1007/s11547-018-0882-7

  Soft tissue sarcomas represent a heterogeneous group of rare malignancies exhibiting mesenchymal differentiation with an overall incidence of around 5/100,000/year. […] Soft tissue sarcomas are currently classified on the basis of the 2013 WHO classification of soft tissue tumors that integrate conventional morphology with immunohistochemistry and molecular genetics. […] Immunohistochemical characterization plays a key role in the diagnostic workup of soft tissue sarcomas. […] Molecular genetics is increasingly used for diagnostic purposes to distinguish specific subtypes of sarcomas, to support diagnosis in non-canonical clinical presentations and also to distinguish true sarcomas from benign mimickers. […] With many exceptions, histologic typing does not provide sufficient information for predicting the clinical course of the disease and, therefore, grading systems based on histological parameters were introduced to provide a more accurate estimation of the degree of malignancy of tumors. […] The three-tiered system devised by the French Federation of Cancer Centers Sarcoma Group (FNCLCC) systems is widely adopted; however, several limitations exist that have led to the development of prognostic nomograms that incorporate the specific histotype as one of the relevant parameters.

• #62

  https://link.springer.com/article/10.1007/s11547-018-0882-7

  Soft tissue sarcomas represent a heterogeneous group of rare malignancies exhibiting mesenchymal differentiation with an overall incidence of around 5/100,000/year. […] Soft tissue sarcomas are currently classified on the basis of the 2013 WHO classification of soft tissue tumors that integrate conventional morphology with immunohistochemistry and molecular genetics. […] Immunohistochemical characterization plays a key role in the diagnostic workup of soft tissue sarcomas. […] Molecular genetics is increasingly used for diagnostic purposes to distinguish specific subtypes of sarcomas, to support diagnosis in non-canonical clinical presentations and also to distinguish true sarcomas from benign mimickers. […] With many exceptions, histologic typing does not provide sufficient information for predicting the clinical course of the disease and, therefore, grading systems based on histological parameters were introduced to provide a more accurate estimation of the degree of malignancy of tumors. […] The three-tiered system devised by the French Federation of Cancer Centers Sarcoma Group (FNCLCC) systems is widely adopted; however, several limitations exist that have led to the development of prognostic nomograms that incorporate the specific histotype as one of the relevant parameters.

• #63

  https://www.vin.com/apputil/content/defaultadv1.aspx?pId=19840&catId=105943&id=8250202&ind=562&objTypeID=17

  STS are graded into low (I), intermediate (II), and high (III) grade tumors, taking into account histologic features such as mitotic rate, extent of necrosis, cellular differentiation, and cellular pleomorphism. […] Grading of STS in dogs is considered more important for prognosis than the cell of origin, because grade significantly affects likelihood of metastasis. […] Immunohistochemical staining has demonstrated that increased AgNOR count in tissue specimens is a significant prognostic indicator for decreased survival time. […] The abundance of skin on the neck and flanks of most patients means wide resections of the upper limb or flank are possible and complex reconstructive techniques are often only indicated in awkward anatomical locations, for example around the perineum. […] Following excision, all cut edges of the tissue (skin margin and deep margin) should be stained with India Ink, left to dry, and then placed into 10% formalin to fix, at a ratio at least 1 part mass: 10 parts formalin. […] Without presurgical biopsies, inadvertent shell-out procedures on masses assumed to be benign that subsequently turn out to be malignant, represent an unfortunately common but easily avoidable clinical scenario.

• #64

  https://www.vin.com/apputil/content/defaultadv1.aspx?pId=19840&catId=105943&id=8250202&ind=562&objTypeID=17

  STS are graded into low (I), intermediate (II), and high (III) grade tumors, taking into account histologic features such as mitotic rate, extent of necrosis, cellular differentiation, and cellular pleomorphism. […] Grading of STS in dogs is considered more important for prognosis than the cell of origin, because grade significantly affects likelihood of metastasis. […] Immunohistochemical staining has demonstrated that increased AgNOR count in tissue specimens is a significant prognostic indicator for decreased survival time. […] The abundance of skin on the neck and flanks of most patients means wide resections of the upper limb or flank are possible and complex reconstructive techniques are often only indicated in awkward anatomical locations, for example around the perineum. […] Following excision, all cut edges of the tissue (skin margin and deep margin) should be stained with India Ink, left to dry, and then placed into 10% formalin to fix, at a ratio at least 1 part mass: 10 parts formalin. […] Without presurgical biopsies, inadvertent shell-out procedures on masses assumed to be benign that subsequently turn out to be malignant, represent an unfortunately common but easily avoidable clinical scenario.

• #65

  https://www.vin.com/apputil/content/defaultadv1.aspx?pId=19840&catId=105943&id=8250202&ind=562&objTypeID=17

  STS are graded into low (I), intermediate (II), and high (III) grade tumors, taking into account histologic features such as mitotic rate, extent of necrosis, cellular differentiation, and cellular pleomorphism. […] Grading of STS in dogs is considered more important for prognosis than the cell of origin, because grade significantly affects likelihood of metastasis. […] Immunohistochemical staining has demonstrated that increased AgNOR count in tissue specimens is a significant prognostic indicator for decreased survival time. […] The abundance of skin on the neck and flanks of most patients means wide resections of the upper limb or flank are possible and complex reconstructive techniques are often only indicated in awkward anatomical locations, for example around the perineum. […] Following excision, all cut edges of the tissue (skin margin and deep margin) should be stained with India Ink, left to dry, and then placed into 10% formalin to fix, at a ratio at least 1 part mass: 10 parts formalin. […] Without presurgical biopsies, inadvertent shell-out procedures on masses assumed to be benign that subsequently turn out to be malignant, represent an unfortunately common but easily avoidable clinical scenario.

• #66 Mechanism-Driven Science Informs Novel Treatments in Soft Tissue Sarcoma

  https://www.targetedonc.com/view/mechanism-driven-science-informs-novel-treatments-in-soft-tissue-sarcoma

  Various combination strategies are being explored in soft tissue sarcomas in efforts to increase efficacy. […] Van Tine, a professor of medicine and sarcoma program director at Washington University School of Medicine in St Louis, Missouri, explained that the field is moving toward mechanism-driven science, with preclinical exploration of the biology of STS, including immune system biology and genomics, informing clinical trials. […] The hypothesis is that the targeted agents and the chemotherapy can prime the tumors, allowing the ICIs to act afterward, explained Van Tine. […] These results also suggest that a longer lead-in time with chemotherapy helps prime the tumor for immunotherapy. […] The immune micro environment is highly variable in STS, with some subtypes having strong immune presence, which offers a promise for immunotherapy in those malignancies, whereas other subtypes are classified as cold tumors.

• #67 MicroRNAs in the Pathogenesis, Prognostication and Prediction of Treatment Resistance in Soft Tissue Sarcomas

  https://www.mdpi.com/2072-6694/15/3/577

  MicroRNAs (miRNAs) are short non-coding RNAs that target mRNAs and control gene expression and may exert both oncogenic and tumour suppressor functions in cancers. The deregulation of miRNAs in soft tissue sarcomas may be exploited in the development of miRNA-based strategies for the prognostication of disease outcomes, identification of treatment resistance and new-generation therapeutics. […] Soft tissue sarcomas are highly aggressive malignant neoplasms of mesenchymal origin, accounting for less than 1% of adult cancers, but comprising over 20% of paediatric solid tumours. […] MicroRNAs (miRNAs) are important regulators of various cancer-related processes, such as differentiation, proliferation, metastasis and apoptosis, and are therefore attractive targets for miRNA-based therapies.

• #68 MicroRNAs in the Pathogenesis, Prognostication and Prediction of Treatment Resistance in Soft Tissue Sarcomas

  https://www.mdpi.com/2072-6694/15/3/577

  MicroRNAs (miRNAs) are short non-coding RNAs that target mRNAs and control gene expression and may exert both oncogenic and tumour suppressor functions in cancers. The deregulation of miRNAs in soft tissue sarcomas may be exploited in the development of miRNA-based strategies for the prognostication of disease outcomes, identification of treatment resistance and new-generation therapeutics. […] Soft tissue sarcomas are highly aggressive malignant neoplasms of mesenchymal origin, accounting for less than 1% of adult cancers, but comprising over 20% of paediatric solid tumours. […] MicroRNAs (miRNAs) are important regulators of various cancer-related processes, such as differentiation, proliferation, metastasis and apoptosis, and are therefore attractive targets for miRNA-based therapies.

• #69 MicroRNAs in the Pathogenesis, Prognostication and Prediction of Treatment Resistance in Soft Tissue Sarcomas

  https://www.mdpi.com/2072-6694/15/3/577

  The role of miRNAs in the diagnosis of soft tissue sarcomas has been proposed and discussed elsewhere; however, the use of miRNA as biomarkers for predicting patient outcomes and therapeutic resistance in soft tissue sarcomas is less defined. In this review, we will first summarise the known roles of specific miRNAs in the pathogenesis of sarcomas, then discuss their potential use in prognosticating outcomes and prediction of therapeutic resistance. […] miRNAs mediate soft tissue sarcoma progression by influencing various pathogenic processes, thereby acting as oncogenes or tumour suppressors. […] The regulation of liposarcoma by miRNAs occurs through various mechanisms. […] miR-206 plays an important role in the regulation of RMS, with multiple studies demonstrating downregulation of miR-206 in RMS tissues and cell lines as compared to human myotubes and skeletal muscle.

• #70 MicroRNAs in the Pathogenesis, Prognostication and Prediction of Treatment Resistance in Soft Tissue Sarcomas

  https://www.mdpi.com/2072-6694/15/3/577

  The role of miRNAs in the diagnosis of soft tissue sarcomas has been proposed and discussed elsewhere; however, the use of miRNA as biomarkers for predicting patient outcomes and therapeutic resistance in soft tissue sarcomas is less defined. In this review, we will first summarise the known roles of specific miRNAs in the pathogenesis of sarcomas, then discuss their potential use in prognosticating outcomes and prediction of therapeutic resistance. […] miRNAs mediate soft tissue sarcoma progression by influencing various pathogenic processes, thereby acting as oncogenes or tumour suppressors. […] The regulation of liposarcoma by miRNAs occurs through various mechanisms. […] miR-206 plays an important role in the regulation of RMS, with multiple studies demonstrating downregulation of miR-206 in RMS tissues and cell lines as compared to human myotubes and skeletal muscle.

• #71 MicroRNAs in the Pathogenesis, Prognostication and Prediction of Treatment Resistance in Soft Tissue Sarcomas

  https://www.mdpi.com/2072-6694/15/3/577

  The role of miRNAs in the diagnosis of soft tissue sarcomas has been proposed and discussed elsewhere; however, the use of miRNA as biomarkers for predicting patient outcomes and therapeutic resistance in soft tissue sarcomas is less defined. In this review, we will first summarise the known roles of specific miRNAs in the pathogenesis of sarcomas, then discuss their potential use in prognosticating outcomes and prediction of therapeutic resistance. […] miRNAs mediate soft tissue sarcoma progression by influencing various pathogenic processes, thereby acting as oncogenes or tumour suppressors. […] The regulation of liposarcoma by miRNAs occurs through various mechanisms. […] miR-206 plays an important role in the regulation of RMS, with multiple studies demonstrating downregulation of miR-206 in RMS tissues and cell lines as compared to human myotubes and skeletal muscle.

• #72 MicroRNAs in the Pathogenesis, Prognostication and Prediction of Treatment Resistance in Soft Tissue Sarcomas

  https://www.mdpi.com/2072-6694/15/3/577

  The role of miRNAs in the diagnosis of soft tissue sarcomas has been proposed and discussed elsewhere; however, the use of miRNA as biomarkers for predicting patient outcomes and therapeutic resistance in soft tissue sarcomas is less defined. In this review, we will first summarise the known roles of specific miRNAs in the pathogenesis of sarcomas, then discuss their potential use in prognosticating outcomes and prediction of therapeutic resistance. […] miRNAs mediate soft tissue sarcoma progression by influencing various pathogenic processes, thereby acting as oncogenes or tumour suppressors. […] The regulation of liposarcoma by miRNAs occurs through various mechanisms. […] miR-206 plays an important role in the regulation of RMS, with multiple studies demonstrating downregulation of miR-206 in RMS tissues and cell lines as compared to human myotubes and skeletal muscle.

• #73 MicroRNAs in the Pathogenesis, Prognostication and Prediction of Treatment Resistance in Soft Tissue Sarcomas

  https://www.mdpi.com/2072-6694/15/3/577

  In recent studies, miRNAs which have been identified to play a role in skeletal muscle proliferation and differentiation have been investigated for their roles in RMS. […] miR-204 was found to be downregulated in both NF1 and non-NF1 MPNST tissues and cell lines. […] Inhibition of PDGFRβ attenuates the differentiation and proliferation of LPS cells, while knockdown of SMAD4 promotes adipogenic differentiation. […] The exciting field of miRNA research has seen miRNA-based technology entering pre-clinical and clinical settings as diagnostic and therapeutic tools for various diseases in recent years.

• #74 Mechanism-Driven Science Informs Novel Treatments in Soft Tissue Sarcoma

  https://www.targetedonc.com/view/mechanism-driven-science-informs-novel-treatments-in-soft-tissue-sarcoma

  Wilky emphasized that we dont really know how to sequence these therapies for STS yet. […] The rarity and the heterogeneity contribute to the challenges faced by experts in the field, a fact that both Wilky and Van Tine highlighted. […] Evidence from preclinical studies suggests that TTI621 may enhance the response to doxorubicin in macrophage-rich tumors that express CD47, such as LMS. […] Preclinical and clinical data have shown that the combination of antiangiogenic agents and chemotherapy results in a synergistic effect. […] The primary end point was progression-free rate (PFR) at 12 weeks for cohort 1 (uterine and nonuterine LMS), which was 45.9%, with a median PFS of 6.4 months. […] This combination may help when tumors are of borderline resectability or when function preservation is a goal.

• #75 MicroRNAs in the Pathogenesis, Prognostication and Prediction of Treatment Resistance in Soft Tissue Sarcomas

  https://www.mdpi.com/2072-6694/15/3/577

  In recent studies, miRNAs which have been identified to play a role in skeletal muscle proliferation and differentiation have been investigated for their roles in RMS. […] miR-204 was found to be downregulated in both NF1 and non-NF1 MPNST tissues and cell lines. […] Inhibition of PDGFRβ attenuates the differentiation and proliferation of LPS cells, while knockdown of SMAD4 promotes adipogenic differentiation. […] The exciting field of miRNA research has seen miRNA-based technology entering pre-clinical and clinical settings as diagnostic and therapeutic tools for various diseases in recent years.

• #76 MicroRNAs in the Pathogenesis, Prognostication and Prediction of Treatment Resistance in Soft Tissue Sarcomas

  https://www.mdpi.com/2072-6694/15/3/577

  In recent studies, miRNAs which have been identified to play a role in skeletal muscle proliferation and differentiation have been investigated for their roles in RMS. […] miR-204 was found to be downregulated in both NF1 and non-NF1 MPNST tissues and cell lines. […] Inhibition of PDGFRβ attenuates the differentiation and proliferation of LPS cells, while knockdown of SMAD4 promotes adipogenic differentiation. […] The exciting field of miRNA research has seen miRNA-based technology entering pre-clinical and clinical settings as diagnostic and therapeutic tools for various diseases in recent years.

• #77 Soft tissue sarcoma: clinical recognition and approach to the loneliest cancer

  https://www.explorationpub.com/Journals/emd/Article/100734

  The goal of treatment for STS of the limbs is function-preserving. […] Advances in STS and prospectives include the emergence of immunotherapy as a novel approach that has revolutionized and rejuvenated the field of tumor immunology. […] Overall, understanding of the loneliest cancer has greatly increased over the years but with very little progress.

• #78 Mechanism-Driven Science Informs Novel Treatments in Soft Tissue Sarcoma

  https://www.targetedonc.com/view/mechanism-driven-science-informs-novel-treatments-in-soft-tissue-sarcoma

  Various combination strategies are being explored in soft tissue sarcomas in efforts to increase efficacy. […] Van Tine, a professor of medicine and sarcoma program director at Washington University School of Medicine in St Louis, Missouri, explained that the field is moving toward mechanism-driven science, with preclinical exploration of the biology of STS, including immune system biology and genomics, informing clinical trials. […] The hypothesis is that the targeted agents and the chemotherapy can prime the tumors, allowing the ICIs to act afterward, explained Van Tine. […] These results also suggest that a longer lead-in time with chemotherapy helps prime the tumor for immunotherapy. […] The immune micro environment is highly variable in STS, with some subtypes having strong immune presence, which offers a promise for immunotherapy in those malignancies, whereas other subtypes are classified as cold tumors.

• #79 Molecular Diagnostics in Sarcoma… | College of American Pathologists

  https://www.cap.org/member-resources/articles/molecular-diagnostics-in-sarcoma-pathology

  While uncovering diagnostic alterations is a large focus of molecular testing in sarcomas, revealing therapeutically targetable alterations is another important application. […] As sequencing costs continue to decrease, NGS-based approaches to identify diagnostic alterations and targeted therapy options in sarcomas are likely to become more common across the world. […] Therefore, it is expected that pathologists will increasingly be called for their expertise in the relevant molecular methods, both traditional and newer, for the sarcomas that they encounter in their clinical practice.

• #80 Molecular Diagnostics in Sarcoma… | College of American Pathologists

  https://www.cap.org/member-resources/articles/molecular-diagnostics-in-sarcoma-pathology

  While uncovering diagnostic alterations is a large focus of molecular testing in sarcomas, revealing therapeutically targetable alterations is another important application. […] As sequencing costs continue to decrease, NGS-based approaches to identify diagnostic alterations and targeted therapy options in sarcomas are likely to become more common across the world. […] Therefore, it is expected that pathologists will increasingly be called for their expertise in the relevant molecular methods, both traditional and newer, for the sarcomas that they encounter in their clinical practice.

• #81 Benign and Malignant Soft-Tissue Tumors: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1253816-overview

  Soft-tissue tumors constitute a large and heterogeneous group of neoplasms. Traditionally, tumors have been classified according to histogenetic features. […] However, histomorphologic, immunohistochemical, and experimental data suggest that most, if not all, sarcomas arise from primitive, multipotential mesenchymal cells, which in the course of neoplastic transformation differentiate along one or more lines. […] Current achievements in the field of soft-tissue tumors are the result of advances in molecular biology, oncogenetics, imaging techniques, immunochemistry, diagnosis by fine-needle aspiration (FNA), surgical reconstruction, radiation therapy, and tissue banking. […] Future advances in molecular oncology may further improve diagnostic, prognostic, and treatment protocols for patients with soft-tissue sarcomas.

• #82 Soft Tissue Sarcoma Symptoms, Facts, Diagnosis, and Treatment | MD Anderson Cancer Center

  https://www.mdanderson.org/cancer-types/soft-tissue-sarcoma.html

  New ways to combine therapies, including surgery, chemotherapy or radiation therapy, to be more successful […] Advanced chemotherapy agents and novel delivery methods that have less impact on the body […] Limb-sparing surgery that can help preserve arms and legs in many cases […] Medicines to fight cancer on a molecular level […] Ways to deliver more intense chemotherapy.

• #83 UK guidelines for the management of soft tissue sarcomas | Clinical Sarcoma Research | Full Text

  https://clinicalsarcomaresearch.biomedcentral.com/articles/10.1186/s13569-016-0060-4

  The most commonly used staging system for soft-tissue sarcoma, produced by the American Joint Committee on Cancer, includes information on both the grade and stage of the tumour. […] The treatment of advanced disease may involve a combination of various strategies, often used in a stepwise fashion, particularly for those patients with a prolonged disease course. […] The development of optimal treatment protocols is hampered by the rarity and heterogeneity of sarcoma. […] A national algorithm produced by the NHS England Sarcoma Clinical Reference Group (CRG) has been proposed to guide the systemic treatment of sarcoma in England; a draft is under review. […] Current and future trials are focusing on targeting new therapies more specifically, utilising genomic profiling, a better understanding of tumourogenesis, and the mechanisms of drug activity. […] It is important therefore, where possible to develop multicentre clinical trials and recruit patients into them. Increasingly it is clear that rather than treating sarcoma as one condition, systemic treatment should be tailored to the histology or genetics of the individual subtype.

• #84 UK guidelines for the management of soft tissue sarcomas | Clinical Sarcoma Research | Full Text

  https://clinicalsarcomaresearch.biomedcentral.com/articles/10.1186/s13569-016-0060-4

  The most commonly used staging system for soft-tissue sarcoma, produced by the American Joint Committee on Cancer, includes information on both the grade and stage of the tumour. […] The treatment of advanced disease may involve a combination of various strategies, often used in a stepwise fashion, particularly for those patients with a prolonged disease course. […] The development of optimal treatment protocols is hampered by the rarity and heterogeneity of sarcoma. […] A national algorithm produced by the NHS England Sarcoma Clinical Reference Group (CRG) has been proposed to guide the systemic treatment of sarcoma in England; a draft is under review. […] Current and future trials are focusing on targeting new therapies more specifically, utilising genomic profiling, a better understanding of tumourogenesis, and the mechanisms of drug activity. […] It is important therefore, where possible to develop multicentre clinical trials and recruit patients into them. Increasingly it is clear that rather than treating sarcoma as one condition, systemic treatment should be tailored to the histology or genetics of the individual subtype.

• #85 UK guidelines for the management of soft tissue sarcomas | Clinical Sarcoma Research | Full Text

  https://clinicalsarcomaresearch.biomedcentral.com/articles/10.1186/s13569-016-0060-4

  The most commonly used staging system for soft-tissue sarcoma, produced by the American Joint Committee on Cancer, includes information on both the grade and stage of the tumour. […] The treatment of advanced disease may involve a combination of various strategies, often used in a stepwise fashion, particularly for those patients with a prolonged disease course. […] The development of optimal treatment protocols is hampered by the rarity and heterogeneity of sarcoma. […] A national algorithm produced by the NHS England Sarcoma Clinical Reference Group (CRG) has been proposed to guide the systemic treatment of sarcoma in England; a draft is under review. […] Current and future trials are focusing on targeting new therapies more specifically, utilising genomic profiling, a better understanding of tumourogenesis, and the mechanisms of drug activity. […] It is important therefore, where possible to develop multicentre clinical trials and recruit patients into them. Increasingly it is clear that rather than treating sarcoma as one condition, systemic treatment should be tailored to the histology or genetics of the individual subtype.

• #86 Mechanism-Driven Science Informs Novel Treatments in Soft Tissue Sarcoma

  https://www.targetedonc.com/view/mechanism-driven-science-informs-novel-treatments-in-soft-tissue-sarcoma

  We can do that in STS, and we can do it pretty quickly and get good data. […] The field is starting to divide up our hotter tumors from our clearly colder tumors, said Van Tine, adding that there is a need for future clinical trials to figure out which patients should be matched with which agents.

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