Soft Tissue Sarcoma Subtypes . Men are much more commonly affected than women. While it does not appear particularly aggressive under the microscope, it travels very early to other sites of the body, including lung, chest cavity, liver, and bone, and occasionally to the brain. This form of sarcoma is relatively resistant to standard chemotherapy. Despite its early spread, people with this diagnosis can live for 1. Collagen diseases are a large group of systemic inflammatory diseases of autoimmune etiology. The etiopathogenesis of collagen diseases is multifactorial. There is genetic susceptibility, as many connective tissue disorders show family history, and environmental. Soft tissue sarcomas (STS) make up a large category of tumors that arise from connective tissue. This category includes tumors of fibrous tissue, fat, smooth muscle, nerves, and lymphatic vessels. The diagnosis of soft tissue sarcoma includes fibrosarcomas. Every soft tissue sarcoma patient is different. The cancer experts at Cancer Treatment Centers of America (CTCA) have extensive experience in properly staging and diagnosing the disease, and developing a treatment plan that's tailored to your specific type of soft. Angiosarcomas and Other Sarcomas of Blood Vessels. This uncommon group of sarcomas appear to arise from the lining of blood vessels (endothelial cells) or their precursors. They include less aggressive (epithelioid hemangioendothelioma or EHE) and more aggressive forms (angiosarcoma)s. Some blood- vessel sarcomas can affect the lymphatic system (which carries fluid from soft tissue to lymph nodes). The vessels of the lymphatic system are lined with cells very similar to those found in blood vessels and give rise to what is termed lymphangiosarcoma. Kaposi sarcoma is a form of blood- vessel sarcoma that is caused by a virus, HHV- 8 (also called KSHV), in patients with HIV disease, as well as a group of typically older persons who have intact immune systems. These tumors have a high risk of recurring where they start and can also travel to liver, bone, lung, and other sites. Connective-Tissue Disease Association Signs or Symptoms Laboratory Data Systemic lupus erythematosus Age, fever, photosensitivity, serositis, alopecia ANA, Anti-dsDNA, Anti-Smith, Anti-cardiolipin antibodies, Coombs positivity, leukopenia Systemic sclerosis.Even rarer tumors can arise from the sensory organs that regulate blood flow to different parts of the body. These are termed glomus tumors and can be painful. Surgery is usually the best way to treat this rare form of sarcoma. Another group of tumors once thought to arise from cells around blood vessels, hemangiopericytoma, is now termed solitary fibrous tumor/hemangiopericytoma, since the tumors all appear to be very similar in terms of their biology. Atypical Fibroxanthoma. An unusual and relatively less aggressive form of sarcoma that shows features of both fibroblasts and cells that retain fat (xanthomas). The primary treatment is surgical. Radiation is occasionally used to try and prevent tumor recurrence, and chemotherapy is largely ineffective for this diagnosis. Current Approaches to Drug Therapy Evidence from clinical trials is still needed to provide solid guidelines. There is little doubt that effective cold avoidance and stress reduction constitute the foundation of any treatment program for Raynaud’s phenomenon. In reporting on their series of 30 cases of epidermoid carcinoma of the parotid, Marks et al stated that 3 of the cases resulted from direct invasion of the skin covering the parotid and 24 originated at other skin sites that drain into the parotid area. These tumors metastasize (spread) very, very rarely. Bone and Cartilage Sarcomas of Soft Tissue. Some sarcomas that arise in soft tissue mimic those that typically arise in cartilage or bone. Many of these sarcomas have some (but not all) features in common with their bone- tumor equivalents, and each has unique characteristics that must be considered in their treatment. Clear Cell Sarcoma. This unusual hybrid tumor (formerly called melanoma of soft parts) appears to be biologically related to alveolar soft- part sarcoma. It is one of the rare tumors with features of both sarcoma and melanoma, including the ability to travel to lymph nodes (typical of melanoma) and to lung (more common for sarcomas). Surgery and radiation of the primary tumor site provide the best chance for cure. Dermatofibrosarcoma Protuberans. Another form of tumor with kinship to fibroblasts, the boring cells that hold many parts of our body together. This tumor typically arises in the skin and must be excised by an appropriate expert, as less extensive resections often end in failure. These tumors only rarely (less than 2%) travel to the lungs, even many years after removal of the initial tumor. The mild chemotherapy drug imatinib shows some usefulness for this diagnosis, and SARC members consider it a standard of care for treatment of recurrences. Desmoplastic Small Round- Cell Tumor (DSRCT)A sarcoma distantly related to Ewing sarcoma that starts and travels in the abdominal cavity long before it is recognized clinically in the majority of patients. It typically arises in young men between 1. The tumor can travel not only elsewhere in the abdominal or pelvic cavity where it starts, but it can also spread to liver, lung, or the space between the lungs (mediastinum). Though cure is infrequent because of the early spread of this form of sarcoma, patients who do the best are those who respond well to chemotherapy and have all visible signs of their tumor removed. Epithelioid Sarcoma. Another form of sarcoma that typically affects the extremities (arms and legs) and tends to travel early to other sites of the body, affecting younger people more commonly than older people. Similar to patients with alveolar soft- part sarcoma, patients can live for a long time with this metastatic disease. Unlike other sarcomas, epithelioid sarcoma can travel to lymph nodes and cause side effects in lymph nodes and other body components. Ewing Sarcoma/Primitive Neuroectodermal Tumor (PNET)This type of sarcoma typically occurs in children or young adults, although cases in people up to age 8. There are fewer than 5. United States. Without chemotherapy, the cure rate is at best 1. Surgery and radiation are also commonly used as treatment for the primary tumor, to try to achieve the highest possible cure rate. Ewing sarcomas more commonly affect bone in children and soft tissue in adults, and can be seen in any site of the body. They commonly recur in the lungs and bones.(Extraskeletal) Myxoid Chondrosarcoma. An unusual form of chondrosarcoma that shows a wide variety of features under the microscope and typically arises in people from 2. It grows relatively slowly but has a high risk of recurrence elsewhere in the body, such as the lung. It is largely insensitive to standard chemotherapy drugs. Extraskeletal Osteosarcoma. This sarcoma (also called extraskeletal osteogenic sarcoma) arises in soft tissue but looks just like its counterpart in bone. Like conventional osteogenic sarcomas, it typically arises in older adults, not in children. It does not respond very well to the chemotherapy drugs used in osteogenic sarcoma of bone and is more commonly treated like other soft- tissue sarcomas. Extrarenal Rhabdoid Tumor. A very aggressive form of sarcoma that nearly always arises in childhood. It affects the kidneys and other structures in the abdomen and has a high risk of early spread to liver, lung, and other sites. Fibrosarcoma. Fibrosarcoma arises from fibroblasts or their precursors and forms a group of tumors that are difficult to diagnose correctly, given their relative scarcity. In decades past, fibrosarcoma was a common name given to sarcomas, but now a true fibrosarcoma is only infrequently diagnosed. These tumors most frequently affect the extremity and trunk, and can metastasize to the lungs, like other sarcomas. Inflammatory myofibroblastic tumor is a similar tumor to inflammatory forms of fibrosarcoma, in which the tumor cells have both features of connective tissue cells (fibroblasts) and muscle. Gastrointestinal Stromal Tumor (GIST)GIST is one of the most common types of sarcoma. It appears to arise from the interstitial cells of Cajal (or its precursors), which are the pacemaker cells of the intestines. In other words, they are responsible for the . There may be as many as 4,0. GIST patients per year in the United States. Approximately two- thirds arise in the wall of the stomach, 2. The common places that GIST recur are in the abdominal cavity or in the liver. GIST tumor cells nearly always show evidence of the KIT molecule or PDGFRA molecule being activated. This is the means by which the three FDA- approved drugs for metastatic GIST, imatinib (Gleevec. They are initially removable with surgery, but some have a high risk of recurrence. Very rarely, GCT of tendon sheath can become more aggressive and travel to other organs, such as the lung. Leiomyosarcoma. This is a tumor of smooth muscle (or its precursors), and can arise anywhere in the body. This is one of the most common types of sarcoma, and about 3,0. United States are affected. Common initial sites for this tumor are the uterus, small intestine or stomach, or the wall of a blood vessel in the abdomen, extremity, or skin. These sarcomas have a highly variable behavior, but when they metastasize they characteristically travel to the lungs and less commonly to the liver, bone, and soft tissues. Liposarcoma. A sarcoma that arises from fat cells or their precursors. It is also a fairly common sarcoma, with approximately 2,5. United States. There are three families of liposarcoma: well- differentiated and/or dedifferentiated (~5. Each has its own specific biology and risk of recurrence or spread. Myxoid and/or Round- Cell Liposarcoma. The second- most- common family of liposarcomas. Both forms carry the same translocations (abnormal joining) between two chromosomes, numbers 1. These tumors typically arise in the leg, with an unusually high risk of recurring in other soft tissue sites or in bones such as the spine and pelvis. Myxoid and/or round- cell liposarcoma appears to be sensitive to eceteinascidin, (Yondelis. This type of sarcoma is considered relatively chemotherapy- sensitive. Pleomorphic Liposarcoma. This is the least common form of liposarcoma, and it also tends to affect an extremity. It is often more aggressive than other liposarcomas and can spread to other sites of the body such as lung and soft tissue. It has much in common with undifferentiated pleomorphic sarcomas (UPS). Well- Differentiated and/or Dedifferentiated Liposarcoma. This sarcoma typically arises in the abdominal cavity or in an extremity. It appears as a large painless mass. Primary therapy is surgical, although the recurrence risk in the abdomen is very high, at least 7. The less aggressive form of this tumor is termed . The more aggressive version of this sarcoma is called . They can arise in people who have a familial disposition to forming benign versions of these tumors called neurofibromas or schwannomas. This condition is called neurofibromatosis type I and carries with it the risk of developing MPNSTs and other tumors, such as relatively less aggressive tumors that affect the brain or nerve sheaths of nerve endings as they leave the brain, but within the skull (acoustic neuromas).
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November 2017
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