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ASPS is a rare cancer that develops in soft tissue, such as muscle, fat or nerves. It typically starts in the leg, but can also appear in the arms, head or neck. In its early stages, it often causes no pain and presents as a lump, making it difficult to detect. By the time it is found, ASPS often has spread, particularly to the lungs. ASPS makes up only 0.2% to 1% of all soft tissue sarcomas. Unlike most sarcomas, ASPS may respond to immunotherapy.

AFH is a soft tissue tumor most commonly found in the arms, legs, hands, or feet. It affects children, adolescents, and young adults, with 88% of cases occurring in those 30 or younger. AFH typically forms in the dermis of the skin but can occasionally develop in other soft tissues, including the brain, lungs and abdominal organs.

Angiosarcoma forms in the lining of blood vessels and lymph vessels. It can occur anywhere in the body, but is most often found on the skin, especially on the head and neck, or in the breast. It can also appear in deeper tissues like the liver and heart, and sometimes develops in areas previously treated with radiation therapy, including the skin of the breast after previous radiation therapy for breast cancer.

ALT is a benign tumor made of fat cells, commonly found under the skin or in the muscles. They are usually small, soft lumps in areas such as the back, shoulders, or upper thighs, and occur most often in adults between 40 and 60 years old. Though typically non-cancerous, they have a very low potential to turn into cancer.

Breast sarcomas make up less than one percent of all breast cancer cases. Unlike most breast cancers, which start in the milk ducts, breast sarcomas begin in the connective tissue that supports the ducts. These tumors act differently because their cells are more like connective tissue, not duct tissue. They usually grow quickly and are often larger when first diagnosed.

starts in cartilage, most often in the pelvis, chest, arms, or legs. It can grow large and may be slow-growing in lower-grade forms. While it accounts for 25% of all primary bone cancers, it is still rare, with only about 1 in 200,000 people diagnosed each year. Chondrosarcomas are more common in adults aged 40 to 70.

Clear cell sarcoma usually occurs in the arms, legs, or feet, but can also develop in the trunk. It accounts for only 1% of all soft tissue sarcomas and typically affects adults in their 20s, 30s, or 40s. Even with treatment, clear cell sarcoma can recur or spread many years after the initial diagnosis. Clear cell sarcoma is one of the rare sarcomas that can spread to lymph nodes.

DFSP is a type of skin tumor that begins in the dermis, the middle layer of the skin. It often appears as a pimple or rough patch, growing slowly over time into a raised lump. DFSP generally affects the arms, legs and trunk. Approximately 15% can become cancerous and spread.

Desmoid tumors are non-cancerous but aggressive growths that develop in the abdomen or connective tissue, including the trunk, arms, or legs. Though non-cancerous, desmoid tumors may grow rapidly, and aggressively invade nearby tissue causing complications. Desmoid tumors may spontaneously regress, so initial periods of observation are recommended for most people with desmoid tumors.

DSRCT is an aggressive cancer that usually begins inside the abdomen. It tends to affect young men, particularly those between the ages of 10 and 30, and is most commonly found in the tissue lining the abdomen and pelvis. DSRCT spreads quickly to nearby organs like the intestine, colon, bladder and liver.

EHE forms in blood vessels, often in the liver, lungs or bones. It can also appear in other areas, including the head, neck and brain. EHE may spread to multiple organs. It is extremely rare, with fewer than 1 in 1 million people diagnosed per year.

Epithelioid sarcoma begins in soft tissues, often under the skin on the fingers, hands, arms, or legs. It usually starts as a painless lump. It primarily affects teenagers and young adults, although older people can also develop it. The cancer tends to grow slowly and may return after treatment. Epithelioid sarcoma is one of the rare sarcomas that can spread to lymph nodes.

begins in bones or surrounding soft tissue, often in the legs, retroperitoneum, or pelvis. It mainly affects children and young adults but can occur at any age. Ewing sarcoma may spread to other areas, including the lungs and bone marrow. Approximately 200 to 250 cases are diagnosed annually in the U.S.

Extraskeletal myxoid chondrosarcoma forms outside of bone, usually in the thigh or chest. It can spread to other tissues, including the lungs, and may return years after treatment. It primarily affects middle-aged adults.

Extraskeletal osteosarcoma arises in soft tissues, but under the microscope, mimics osteosarcoma, a bone cancer. It commonly occurs in adults over 40, affecting areas like the thigh, pelvis, and retroperitoneum (the back part of the abdomen).

Fibromyxoid sarcoma is a slow-growing cancer that forms in deep soft tissues of the legs or trunk. It may spread to the lungs or chest wall. It mostly affects young to middle-aged adults. Approximately 50% will spread to other organs, though this may take years or even decades.

Fibrous histiocytoma of bone is found in bones, most often in the legs. It may cause bone destruction, leading to fractures. This cancer is linked to conditions like or past radiation treatments and usually affects older adults.

Fibrosarcoma develops in soft tissues like tendons and ligaments. It can form in bones or connective tissue. Most cases occur in the legs, arms, or trunk. Fibrosarcoma accounts for about 10% of soft tissue sarcoma diagnoses.

GIST starts in the digestive system, usually in the stomach or small intestine. GISTs may grow slowly and cause no symptoms at first but can lead to pain, nausea, and bleeding as they grow. GISTs are the most common sarcoma in adults, and they are the most common sarcoma of the gastrointestinal tract. Between 4,000 and 6,000 people are diagnosed with GISTs annually in the U.S. GISTs are one of the first sarcomas successfully treated with targeted therapy.

IMT is an often-benign tumor found in organs like the lungs, stomach and intestines. While it does not usually spread, it can invade nearby organs and cause problems. It is most commonly found in children and young adults.

Intimal sarcoma occurs in the blood vessels, often in the pulmonary artery or aorta. It is more common in middle-aged adults.

affects people with weakened immune systems, often linked to the HHV-8 virus. It causes lesions on the skin and can have various forms, including AIDS-related and classic types. Unlike other sarcomas listed here, usually Kaposi sarcoma is treated by addressing the basis of the weakened immune system.

Leiomyosarcoma starts in smooth muscle tissue, most commonly in the abdomen, retroperitoneum, arms, legs, or uterus. It can grow quickly and spread to other parts of the body. Symptoms may include pain, weight loss, and swelling. Leiomyosarcoma accounts for 10% to 20% of all sarcomas.

is made of fat cells, typically found deep in the abdomen, retroperitoneum, buttocks, or legs. Liposarcomas can be slow growing but may return or spread to other organs like the lungs or liver. About 1,700 cases are diagnosed annually in the U.S.

Glomus tumors, also called paragangliomas, are slow-growing neuroendocrine tumors that typically arise in the skull base or in the neck. While usually benign, untreated cases can damage surrounding tissue and nerves and cause serious complications. The most common types include carotid body tumors, glomus jugulare (involving the jugular bulb), glomus tympanicum (middle ear), and glomus vagale (vagus nerve).

Malignant granular cell tumor originates in Schwann cells, which support nerve cells. Most occur under the skin, particularly in the head, neck, mouth or tongue, but they can also develop in internal organs including organs in the chest or abdomen. While most granular cell tumors are benign, some are malignant and may grow aggressively. These tumors are more common in middle-aged adults and are also called Abrikossoff tumors.

Malignant melanotic nerve sheath tumors (MMNSTs) are rare cancers that develop from Schwann cells capable of producing melanin. Often found along spinal nerves, MMNSTs were reclassified as malignant in the 2020 WHO classification of soft tissue tumors.

MPNSTs arise in the lining of peripheral nerves, which connect the spinal cord to the body. Formerly called neurofibrosarcomas, they often develop in the arms, legs, or trunk. Symptoms include pain, weakness, or a growing mass.

PNETs are aggressive tumors derived from primitive nerve cells, usually developing in the central or peripheral nervous system. Common in children, they often affect areas of the brain responsible for movement, thought, and sensation. Now they are classified under embryonal tumors with multilayered rosettes (ETMRs).

Schwannomas, also known as neurilemmomas or neurinomas, are nerve sheath tumors formed by Schwann cells, which insulate nerves. While most are benign, about 5% become malignant and can spread. They occur on peripheral nerves in areas like the arms, legs, face or spine.

These noncancerous tumors grow in soft tissue around joints and are extremely rare, affecting fewer than 50 per million people annually. They may cause pain, difficulty moving or bone fractures.

Mesenchymal chondrosarcoma is an aggressive cancer that primarily develops in cartilage cells. Mesenchymal chondrosarcoma typically affects the spine, ribs, or jaw but can also occur in soft tissues. Unlike conventional chondrosarcoma, it is more common in young adults and often spreads to other parts of the body.

Myoepitheliomas originate in myoepithelial cells and can occur in soft tissue, salivary glands or skin. Depending on their location, they may behave in a benign or aggressive manner.

Myxofibrosarcoma usually arises in the legs or arms of older adults. Myxofibrosarcomas can invade nearby tissue, track along nerves, blood vessels, or other tissue planes for long distances, and they have a high risk of recurrence after treatment. While slow growing in some cases, high-grade tumors are more aggressive and may metastasize to the lungs or lymph nodes (though less often than other sarcomas arising in the arms or legs).

This locally aggressive tumor typically affects the hands and feet. Often mistaken for a benign growth, it develops slowly and commonly recurs after treatment. Prognosis depends on early detection and successful treatment.

See Malignant Peripheral Nerve Sheath Tumor.

begins in the bones, often during adolescence. It most commonly affects long bones, such as those in the arms and legs, particularly near the knee or other areas of rapid growth during a teenager’s growth spurt. Most cases are high-grade, meaning the cancer grows quickly and can spread to other parts of the body. Osteosarcoma is more common in young people under 25, with an average diagnosis age of 15. It occurs slightly more often in boys after puberty and is slightly more common in African Americans.

PEComa typically grows in the soft tissues of organs like the stomach, intestines, lungs, and reproductive or urinary systems or in the tissue adjacent to them. Most PEComas are noncancerous and often occur in children with tuberous sclerosis.

Radiation-associated sarcomas are rare cancers that develop as a long-term consequence of radiation therapy. These secondary malignancies may appear years after treatment. Radiation sarcomas tend to be aggressive. Common types of sarcomas (seen after prior radiation for other cancers) include angiosarcoma and osteosarcoma.

Retroperitoneal sarcoma (RPS) forms in the retroperitoneum, the space between the abdominal wall and the peritoneum, which holds the kidneys, pancreas, and parts of major blood vessels.

Rhabdomyosarcoma starts in soft tissues, often in muscles. It commonly affects children and teenagers, developing in areas like the head, neck, arms, legs, or abdomen. Treatment options usually depend on the tumor’s location and whether it has spread. Each year, about 400–500 people in the U.S. are diagnosed with this cancer.

Solitary fibrous tumors (SFTs) arise from connective tissue and can occur almost anywhere in the body, though they most often form around the lungs or in the abdomen, pelvis, or retroperitoneum. Many are indolent (slow growing), but malignant cases can occur. Symptoms depend on the tumor’s size and location. SFTs differ from pleural mesothelioma, as they are unrelated to asbestos exposure.

Spindle cell sarcoma is a non-specific designation for sarcomas that do not fit into the other categories, based on how they look under the microscope or what the genetics of the tumor reveal. The name is derived from the appearance of these sarcoma cells under the microscope, based on the long, narrow shape of its cancer cells.

Synovial sarcoma develops in soft tissues, such as muscles or ligaments, and often resembles the cells found in synovial joints like the elbows, hips, and shoulders (though not derived from synovium). This cancer often grows slowly and might not cause symptoms until the tumor becomes large enough to form a visible or noticeable lump. It may cause pain or numbness if it presses on nearby nerves. Synovial sarcoma can develop in areas like the abdomen, arms, legs, feet, and lungs, as well as joints such as the ankles, wrists, and shoulders. It primarily affects young adults and is diagnosed in about 1,000 people annually in the U.S.

Undifferentiated pleomorphic sarcoma, previously called malignant fibrous histiocytoma, is an aggressive type of sarcoma escaping further classification that typically starts in soft tissues like fat, muscles, or fibrous tissues such as tendons and ligaments. It most often develops in the arms, legs, or behind the abdominal organs (retroperitoneum). The cancer cells are called “undifferentiated” because they do not resemble the tissue where they form, and “pleomorphic” because they appear in various shapes and sizes under a microscope. UPS often spreads to other parts of the body, such as the lungs or lymph nodes, and has a high risk of returning to the same location after treatment. Soft tissue sarcomas like UPS are very rare, with around 13,000 cases diagnosed annually in the U.S., representing less than 1% of adult cancers.

Unclassified sarcomas are a group of rare cancers that do not match the characteristics of other sarcoma types when viewed under a microscope. These cancers often form in muscles attached to bones that assist with movement. Unclassified sarcomas tend to grow rapidly and may spread to other parts of the body. In some cases, they can develop in areas previously exposed to radiation therapy.

Uterine sarcomas may develop in the muscle and connective tissues of the uterus. Symptoms may include unusual bleeding, pain, and feeling full. Unlike endometrial cancer, which starts in the uterine lining, uterine sarcoma forms in the uterine muscle. The most common type is leiomyosarcoma.

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