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Sarcoma
 
 
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A sarcoma (from the Greek 'sarx' meaning "flesh") is a cancer
Cancer

Cancer is a class of diseases in which a group of cell display uncontrolled growth , invasion , and sometimes metastasis . These three malignant properties of cancers differentiate them from benign tumors, which are self-limited, do not invade or metastasize....
 of the connective tissue
Connective tissue

Connective tissue is a form of fibrous biological tissue.It is one of the four types of tissue in traditional classifications .Collagen is the main protein of connective tissue in animals and the most abundant protein in mammals, making up about 25% of the total protein content....
 (bone
Bone

Bones are rigid organ that form part of the endoskeleton of vertebrates. They function to move, support, and protect the various organs of the body, produce red blood cell and white blood cells and store minerals....
, cartilage
Cartilage

Cartilage is a type of dense connective tissue. It is composed of specialized cells called chondrocyte that produce a large amount of extracellular matrix composed of collagen fibers, abundant ground substance rich in proteoglycan, and elastin fibers....
, fat
Fat

Fats consist of a wide group of compounds that are generally soluble in organic solvents and largely insoluble in water. Chemistry, fats are generally ester of glycerol and fatty acids....
) resulting in mesoderm
Mesoderm

One of the three germ layers found in the embryos of animals more complex than cnidarians, making them triploblastic. Mesoderm forms in the embryo during gastrulation when some of the cells migrating inward to form the endoderm, produce an additional layer that lies between the endoderm and the ectoderm....
 proliferation.

This is in contrast to carcinomas
Carcinoma

A carcinoma is any malignant cancer that arises from Epithelium. Carcinomas invade surrounding tissues and organs and may Metastasis, or spread, to lymph nodes and other sites....
, which are of epithelial
Epithelium

In biology and medicine, epithelium is a Biological tissue composed of cell s that line the cavities and surfaces of structures throughout the body....
 origin (breast
Breast

The breast is the upper ventral region of an animal?s torso, particularly that of mammals, including human beings. The breasts of a female primate?s body contain the mammary glands, which secrete milk used to feed infants....
, colon
Colon (anatomy)

The colon is the last portion of the digestive system in most vertebrates; it extracts water and salt from feces before they are defecation from the body....
, pancreas
Pancreas

The pancreas is a gland Organ in the digestive system and endocrine system of vertebrates. It is both an endocrine gland , as well as an exocrine gland, secreting pancreatic juice containing Digestion enzymes that pass to the small intestine....
, and others). However, due to an evolving understanding of tissue origin, the term "sarcoma" is sometimes applied to tumors now known to arise from epithelial tissue. The term soft tissue sarcoma
Soft tissue sarcoma

A soft tissue sarcoma is a form of sarcoma that develops in connective tissue., though the term is sometimes applied to elements of the soft tissue that are not currently considered connective tissue....
 is used to describe tumors of soft tissue
Soft tissue

In medicine, the term soft tissue refers to Tissue that connect, support, or surround other structures and Organ s of the body.Soft tissue includes tendons, ligaments, fascia, Fibrous connective tissue, fat, and synovial membranes , and muscles, nerves and blood vessels ....
, which includes elements that are in connective tissue, but not derived from it (such as muscles and blood vessel
Blood vessel

The blood vessels are the part of the circulatory system that transport blood throughout the body. There are three major types of blood vessels: the artery, which carry the blood away from the heart, the capillary, which enable the actual exchange of water and chemicals between the blood and the tissues; and the veins, which carry blood from...
s).

omas are given a number of different names, based on the type of tissue from which they arise.






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A sarcoma (from the Greek 'sarx' meaning "flesh") is a cancer
Cancer

Cancer is a class of diseases in which a group of cell display uncontrolled growth , invasion , and sometimes metastasis . These three malignant properties of cancers differentiate them from benign tumors, which are self-limited, do not invade or metastasize....
 of the connective tissue
Connective tissue

Connective tissue is a form of fibrous biological tissue.It is one of the four types of tissue in traditional classifications .Collagen is the main protein of connective tissue in animals and the most abundant protein in mammals, making up about 25% of the total protein content....
 (bone
Bone

Bones are rigid organ that form part of the endoskeleton of vertebrates. They function to move, support, and protect the various organs of the body, produce red blood cell and white blood cells and store minerals....
, cartilage
Cartilage

Cartilage is a type of dense connective tissue. It is composed of specialized cells called chondrocyte that produce a large amount of extracellular matrix composed of collagen fibers, abundant ground substance rich in proteoglycan, and elastin fibers....
, fat
Fat

Fats consist of a wide group of compounds that are generally soluble in organic solvents and largely insoluble in water. Chemistry, fats are generally ester of glycerol and fatty acids....
) resulting in mesoderm
Mesoderm

One of the three germ layers found in the embryos of animals more complex than cnidarians, making them triploblastic. Mesoderm forms in the embryo during gastrulation when some of the cells migrating inward to form the endoderm, produce an additional layer that lies between the endoderm and the ectoderm....
 proliferation.

This is in contrast to carcinomas
Carcinoma

A carcinoma is any malignant cancer that arises from Epithelium. Carcinomas invade surrounding tissues and organs and may Metastasis, or spread, to lymph nodes and other sites....
, which are of epithelial
Epithelium

In biology and medicine, epithelium is a Biological tissue composed of cell s that line the cavities and surfaces of structures throughout the body....
 origin (breast
Breast

The breast is the upper ventral region of an animal?s torso, particularly that of mammals, including human beings. The breasts of a female primate?s body contain the mammary glands, which secrete milk used to feed infants....
, colon
Colon (anatomy)

The colon is the last portion of the digestive system in most vertebrates; it extracts water and salt from feces before they are defecation from the body....
, pancreas
Pancreas

The pancreas is a gland Organ in the digestive system and endocrine system of vertebrates. It is both an endocrine gland , as well as an exocrine gland, secreting pancreatic juice containing Digestion enzymes that pass to the small intestine....
, and others). However, due to an evolving understanding of tissue origin, the term "sarcoma" is sometimes applied to tumors now known to arise from epithelial tissue. The term soft tissue sarcoma
Soft tissue sarcoma

A soft tissue sarcoma is a form of sarcoma that develops in connective tissue., though the term is sometimes applied to elements of the soft tissue that are not currently considered connective tissue....
 is used to describe tumors of soft tissue
Soft tissue

In medicine, the term soft tissue refers to Tissue that connect, support, or surround other structures and Organ s of the body.Soft tissue includes tendons, ligaments, fascia, Fibrous connective tissue, fat, and synovial membranes , and muscles, nerves and blood vessels ....
, which includes elements that are in connective tissue, but not derived from it (such as muscles and blood vessel
Blood vessel

The blood vessels are the part of the circulatory system that transport blood throughout the body. There are three major types of blood vessels: the artery, which carry the blood away from the heart, the capillary, which enable the actual exchange of water and chemicals between the blood and the tissues; and the veins, which carry blood from...
s).

Classification


Tissue

Sarcomas are given a number of different names, based on the type of tissue from which they arise. For example, osteosarcoma
Osteosarcoma

Osteosarcoma is the most common type of malignant bone cancer, accounting for 35% of primary bone malignancies. There is a preference for the metaphysis region of tubular long bones....
 arises from bone
Bone

Bones are rigid organ that form part of the endoskeleton of vertebrates. They function to move, support, and protect the various organs of the body, produce red blood cell and white blood cells and store minerals....
, chondrosarcoma
Chondrosarcoma

A chondrosarcoma is a type of cancer of the bone. Chondrosarcoma is a cartilage-based tumor and is in a category of cancers called sarcomas. About 25% of primary bone cancers are chondrosarcomas....
 arises from cartilage
Cartilage

Cartilage is a type of dense connective tissue. It is composed of specialized cells called chondrocyte that produce a large amount of extracellular matrix composed of collagen fibers, abundant ground substance rich in proteoglycan, and elastin fibers....
, and leiomyosarcoma
Leiomyosarcoma

Leiomyosarcoma is a type of sarcoma which is a neoplasm of smooth muscle. Smooth muscle cells make up the involuntary muscles, which are found in most parts of the body: in uterus, stomach and intestines, walls of all blood vessels, and skin....
 arises from smooth muscle
Smooth muscle

Smooth muscle is a type of non-striated muscle, found within the tunica media layer of large and small arteries and veins, the urinary bladder, uterus, male and female reproductive tracts, gastrointestinal tract, respiratory tract, the ciliary muscle, and iris of the eye....
. Sarcomas strike people in all age ranges, but they are very rare, accounting for only 1% of all cases of cancer
Cancer

Cancer is a class of diseases in which a group of cell display uncontrolled growth , invasion , and sometimes metastasis . These three malignant properties of cancers differentiate them from benign tumors, which are self-limited, do not invade or metastasize....
. Soft tissue sarcomas, such as leiomyosarcoma
Leiomyosarcoma

Leiomyosarcoma is a type of sarcoma which is a neoplasm of smooth muscle. Smooth muscle cells make up the involuntary muscles, which are found in most parts of the body: in uterus, stomach and intestines, walls of all blood vessels, and skin....
, chondrosarcoma
Chondrosarcoma

A chondrosarcoma is a type of cancer of the bone. Chondrosarcoma is a cartilage-based tumor and is in a category of cancers called sarcomas. About 25% of primary bone cancers are chondrosarcomas....
, and gastrointestinal stromal tumor
Gastrointestinal stromal tumor

A gastrointestinal stromal tumor is a rare disease tumor of the gastrointestinal tract . They are a type of mesenchyme tumor and typically defined as tumors whose behavior is driven by genetic mutations in the CD117 gene or PDGFRA gene, and may or may not staining positively for Kit....
 (GIST), are more common in adults than in children. GIST is the most common form of sarcoma, with approximately 3000-3500 cases per year in the United States. This should be compared with breast cancer, with approximately 200,000 cases per year in North America. Bone sarcomas, such as osteosarcoma
Osteosarcoma

Osteosarcoma is the most common type of malignant bone cancer, accounting for 35% of primary bone malignancies. There is a preference for the metaphysis region of tubular long bones....
 and Ewing's sarcoma
Ewing's sarcoma

Ewing sarcoma is a malignant round-cell tumor. It is a rare disease in which cancer cells are found in the bone or in soft biological tissue. The most common areas in which it occurs are the pelvis, the femur, the humerus, and the ribs....
, are more common in children than in adults. These tumors most commonly strike adolescents and young adults between the ages of 12 and 25.

Grade

In addition to being named based on the tissue of origin, sarcomas are also assigned a grade, such as low grade or high grade. Low grade sarcomas are usually treated surgically, although sometimes radiation therapy
Radiation therapy

Radiation therapy is the medicine use of ionizing radiation as part of cancer oncology to control malignant cell s . Radiotherapy may be used for curative or Adjuvant chemotherapy cancer treatment....
 or chemotherapy
Chemotherapy

Chemotherapy, in its most general sense, refers to treatment of disease by chemicals that kill cells, specifically those of micro-organisms or cancer....
 are used. High grade sarcomas are more frequently treated with chemotherapy
Chemotherapy

Chemotherapy, in its most general sense, refers to treatment of disease by chemicals that kill cells, specifically those of micro-organisms or cancer....
. Since these tumors are more likely to undergo metastasis
Metastasis

Metastasis , or Metastatic disease, sometimes abbreviated mets, is the spread of a disease from one Organ or part to another non-adjacent organ or part....
 (spreading to distant sites), these tumors are treated more aggressively. Childhood sarcomas are almost always treated with a combination of surgery
Surgery

Surgery is a medical specialty that uses operative manual and instrumental techniques on a patient to investigate and/or treat a pathological condition such as disease or injury, to help improve bodily function or appearance, or sometimes for some other reason....
 and chemotherapy
Chemotherapy

Chemotherapy, in its most general sense, refers to treatment of disease by chemicals that kill cells, specifically those of micro-organisms or cancer....
, and radiation is frequently used as well. The recognition that childhood sarcomas are sensitive to chemotherapy has dramatically improved the survival of patients. For example, in the era before chemotherapy, long term survival for patients with localized osteosarcoma was only approximately 20%, but now has risen to 60-70%.

Types of sarcoma

(ICD-O codes are provided where available.)
  • Askin's Tumor (8803/3)
  • Chondrosarcoma
    Chondrosarcoma

    A chondrosarcoma is a type of cancer of the bone. Chondrosarcoma is a cartilage-based tumor and is in a category of cancers called sarcomas. About 25% of primary bone cancers are chondrosarcomas....
     (9220/3-9240/3)
  • Ewing's (9260/3) - PNET
    Primitive neuroectodermal tumor

    Primitive neuroectodermal tumor is a neural crest tumor. It is a rare tumor, usually occurring in children under 10 years old.PNET belongs to the Ewing family of tumors....
     (9473/3)
  • Malignant Hemangioendothelioma (9130/3)
  • Malignant Schwannoma (9560/3-9561/3)
  • Osteosarcoma
    Osteosarcoma

    Osteosarcoma is the most common type of malignant bone cancer, accounting for 35% of primary bone malignancies. There is a preference for the metaphysis region of tubular long bones....
     (9180/3-9190/3)
  • Soft tissue sarcoma
    Soft tissue sarcoma

    A soft tissue sarcoma is a form of sarcoma that develops in connective tissue., though the term is sometimes applied to elements of the soft tissue that are not currently considered connective tissue....
    s, including:
    • Alveolar soft part sarcoma
      Alveolar soft part sarcoma

      Alveolar soft part sarcoma is a very rare type of Soft tissue sarcoma - a slow growing tumor of an unknown origin that arises mainly in children and young adults....
       (9581/3)
    • Angiosarcoma
      Angiosarcoma

      Angiosarcoma is a malignant neoplasm of vessel walls. This may be in reference to blood or lymphatic vessels.The term angiosarcoma is avoided in medical practice, as it does not define the tissue origin of the tumour precisely enough....
       (9120/3)
    • Cystosarcoma Phylloides
      Phyllodes tumor

      Phyllodes tumors , also cystosarcoma phyllodes, cystosarcoma phylloides and phylloides tumor, are typically large, fast growing masses that form from the periductal stroma of the breast....
    • Dermatofibrosarcoma
      Dermatofibrosarcoma

      Dermatofibrosarcoma protuberans is a rare tumor. There is only about 1 case per million per year. DFSP is a fibrosarcoma, more precisely a cutaneous soft tissue sarcoma....
       (8832/3-8833/3)
    • Desmoid Tumor
      Desmoid tumor

      A desmoid tumor is a rare tumor that may or may not be part of a genetics syndrome such as familial adenomatous polyposis .Histologically they resemble grading fibrosarcomas, but they are very infiltration and tend to recur even after complete resection ....
       (8821/1-8822/1)
    • Desmoplastic small round cell tumor
      Desmoplastic small round cell tumor

      Desmoplastic small round cell tumor is classified as a soft tissue sarcoma. It is an aggressive and rare tumor that primarily occurs as masses in the abdomen....
       (8806/3)
    • Epithelioid Sarcoma
      Epithelioid sarcoma

      Epithelioid sarcoma is a rare mesenchymal soft tissue tumor with an epithelioid pattern. It often occurs in the extremities of young people. ...
       (8804/3)
    • Extraskeletal chondrosarcoma (9220/3)
    • Extraskeletal osteosarcoma (9180/3)
    • Fibrosarcoma
      Fibrosarcoma

      Fibrosarcoma is a malignant tumor derived from fibrous connective tissue and characterized by immature proliferation fibroblasts or undifferentiated anaplastic spindle cells....
       (8810/3)
    • Hemangiopericytoma
      Hemangiopericytoma

      A hemangiopericytoma is a type of soft tissue sarcoma that originates in the pericytes in the walls of capillary.It was characterized in 1942....
       (9150)
    • Hemangiosarcoma
      Hemangiosarcoma

      Hemangiosarcoma is a malignant, rare, rapidly growing, highly invasive variety of cancer. It is a blood-fed sarcoma; that is, blood vessels grow directly into the tumor and it is typically filled with blood....
       (9120/3)
    • Kaposi's sarcoma
      Kaposi's sarcoma

      Kaposi's sarcoma is a tumor caused by Kaposi's sarcoma-associated herpesvirus , also known as Kaposi's sarcoma-associated herpesvirus . It was originally described by Moritz Kaposi, a Hungarian dermatologist practicing at the University of Vienna in 1872....
       (9140/3)
    • Leiomyosarcoma
      Leiomyosarcoma

      Leiomyosarcoma is a type of sarcoma which is a neoplasm of smooth muscle. Smooth muscle cells make up the involuntary muscles, which are found in most parts of the body: in uterus, stomach and intestines, walls of all blood vessels, and skin....
       (8890/3-8896/3)
    • Liposarcoma
      Liposarcoma

      Liposarcoma is a malignant tumor that arises in Adipose tissue in deep soft tissue, such as that inside the thigh or in the retroperitoneum.They are typically large bulky tumors which tend to have multiple smaller satellites extending beyond the main confines of the tumor....
       (8850/3-8858/3)
    • Lymphangiosarcoma
      Lymphangiosarcoma

      Lymphangiosarcoma is a rare malignant tumor which occurs in long-standing cases of Primary or Secondary Lymphedema. It involves either the upper or lower lymphedemateous extremities but is most common in upper extremities....
       (9170-9175)
    • Lymphosarcoma
      Lymphoma

      Lymphoma is a type of cancer that originates in lymphocytes of the immune system. They often originate in lymph nodes, presenting as an enlargement of the node ....
    • Malignant fibrous histiocytoma
      Malignant fibrous histiocytoma

      Malignant fibrous histiocytoma is a type of histiocytoma that is the most common Sarcoma of late adult life, most commonly occurring between age 50-70....
       (8830/3)
    • Neurofibrosarcoma
      Neurofibrosarcoma

      Neurofibrosarcoma is a type of tumor combining characteristics of neurofibroma and fibrosarcoma.Neurofibrosarcoma, also known as peripheral nerve sheath tumor, malignant schwannoma, malignant nerve sheath tumor, and neurogenic sarcoma, is a type of cancer that causes malignant tumors in areas surrounding peripheral nerves....
       (9540/3)
    • Rhabdomyosarcoma
      Rhabdomyosarcoma

      A rhabdomyosarcoma is a type of cancer, specifically a sarcoma , in which the cancer cells are thought to arise from skeletal muscle progenitors....
       (8900-8920)
    • Synovial sarcoma
      Synovial sarcoma

      A synovial sarcoma is a rare form of cancer which usually occurs near to the joints of the arm or leg. It is one of the soft tissue sarcomas....
       (9040/3-9043/3)


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