Sarcoma
Encyclopedia
A sarcoma is a cancer
that arises from transformed cells
in one of a number of tissues that develop from embryonic mesoderm
. Thus, sarcomas include tumors of bone
, cartilage
, fat
, muscle
, vascular
, and hematopoietic tissues. (This is in contrast to carcinoma
s, which originate from epithelial
cells and thus give rise to more common malignancies, such as breast
, colon, and lung
cancer.)
arises from bone
, chondrosarcoma
arises from cartilage
, liposarcoma
arises from fat
, and leiomyosarcoma
arises from smooth muscle
.
or chemotherapy
are used. Intermediate and high grade sarcomas are more frequently treated with a combination of surgery
, chemotherapy
and/or radiation therapy
. Since higher grade tumors are more likely to undergo metastasis
(invasion and spread to locoregional and distant sites), they are treated more aggressively. The recognition that many 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%.
Gastrointestinal stromal tumor
(GIST) is the most common form of sarcoma, with approximately 3,000-3,500 cases per year in the United States.
Sarcomas affect people of all ages. Approximately 50% of bone sarcomas and 20% of soft tissue sarcomas are diagnosed in people under the age of 35. Some sarcomas, such as leiomyosarcoma
, chondrosarcoma
, and gastrointestinal stromal tumor
(GIST), are more common in adults than in children. Most high-grade bone sarcomas, including Ewing's sarcoma and osteosarcoma
, are much more common in children and young adults.
Cancer
Cancer , known medically as a malignant neoplasm, is a large group of different diseases, all involving unregulated cell growth. In cancer, cells divide and grow uncontrollably, forming malignant tumors, and invade nearby parts of the body. The cancer may also spread to more distant parts of the...
that arises from transformed cells
Cell (biology)
The cell is the basic structural and functional unit of all known living organisms. It is the smallest unit of life that is classified as a living thing, and is often called the building block of life. The Alberts text discusses how the "cellular building blocks" move to shape developing embryos....
in one of a number of tissues that develop from embryonic mesoderm
Mesoderm
In all bilaterian animals, the mesoderm is one of the three primary germ cell layers in the very early embryo. The other two layers are the ectoderm and endoderm , with the mesoderm as the middle layer between them.The mesoderm forms mesenchyme , mesothelium, non-epithelial blood corpuscles and...
. Thus, sarcomas include tumors of bone
Bone
Bones are rigid organs that constitute part of the endoskeleton of vertebrates. They support, and protect the various organs of the body, produce red and white blood cells and store minerals. Bone tissue is a type of dense connective tissue...
, cartilage
Cartilage
Cartilage is a flexible connective tissue found in many areas in the bodies of humans and other animals, including the joints between bones, the rib cage, the ear, the nose, the elbow, the knee, the ankle, the bronchial tubes and the intervertebral discs...
, fat
Fat
Fats consist of a wide group of compounds that are generally soluble in organic solvents and generally insoluble in water. Chemically, fats are triglycerides, triesters of glycerol and any of several fatty acids. Fats may be either solid or liquid at room temperature, depending on their structure...
, muscle
Muscle
Muscle is a contractile tissue of animals and is derived from the mesodermal layer of embryonic germ cells. Muscle cells contain contractile filaments that move past each other and change the size of the cell. They are classified as skeletal, cardiac, or smooth muscles. Their function is to...
, vascular
Vascular
Vascular in zoology and medicine means "related to blood vessels", which are part of the circulatory system. An organ or tissue that is vascularized is heavily endowed with blood vessels and thus richly supplied with blood....
, and hematopoietic tissues. (This is in contrast to carcinoma
Carcinoma
Carcinoma is the medical term for the most common type of cancer occurring in humans. Put simply, a carcinoma is a cancer that begins in a tissue that lines the inner or outer surfaces of the body, and that generally arises from cells originating in the endodermal or ectodermal germ layer during...
s, which originate from epithelial
Epithelium
Epithelium is one of the four basic types of animal tissue, along with connective tissue, muscle tissue and nervous tissue. Epithelial tissues line the cavities and surfaces of structures throughout the body, and also form many glands. Functions of epithelial cells include secretion, selective...
cells and thus give rise to more common malignancies, such as breast
Breast cancer
Breast cancer is cancer originating from breast tissue, most commonly from the inner lining of milk ducts or the lobules that supply the ducts with milk. Cancers originating from ducts are known as ductal carcinomas; those originating from lobules are known as lobular carcinomas...
, colon, and lung
Lung cancer
Lung cancer is a disease characterized by uncontrolled cell growth in tissues of the lung. If left untreated, this growth can spread beyond the lung in a process called metastasis into nearby tissue and, eventually, into other parts of the body. Most cancers that start in lung, known as primary...
cancer.)
Classification
Tissue
Sarcomas are given a number of different names based on the type of tissue from which they arise. For example, osteosarcomaOsteosarcoma
Osteosarcoma is an aggressive cancerous neoplasm arising from primitive transformed cells of mesenchymal origin that exhibit osteoblastic differentiation and produce malignant osteoid...
arises from bone
Bone
Bones are rigid organs that constitute part of the endoskeleton of vertebrates. They support, and protect the various organs of the body, produce red and white blood cells and store minerals. Bone tissue is a type of dense connective tissue...
, chondrosarcoma
Chondrosarcoma
Chondrosarcoma is a cancer composed of cells derived from transformed cells that produce cartilage. Chondrosarcoma is a member of a category of "soft tissue" malignancies known as sarcomas. About 30% of skeletal system cancers are chondrosarcomas...
arises from cartilage
Cartilage
Cartilage is a flexible connective tissue found in many areas in the bodies of humans and other animals, including the joints between bones, the rib cage, the ear, the nose, the elbow, the knee, the ankle, the bronchial tubes and the intervertebral discs...
, liposarcoma
Liposarcoma
Liposarcoma is a malignant tumor that arises in fat cells 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.Liposarcomas, like all...
arises from fat
Fat
Fats consist of a wide group of compounds that are generally soluble in organic solvents and generally insoluble in water. Chemically, fats are triglycerides, triesters of glycerol and any of several fatty acids. Fats may be either solid or liquid at room temperature, depending on their structure...
, and leiomyosarcoma
Leiomyosarcoma
Leiomyosarcoma , aka LMS, is a malignant cancer of smooth muscle....
arises from smooth muscle
Smooth muscle
Smooth muscle is an involuntary non-striated muscle. It is divided into two sub-groups; the single-unit and multiunit smooth muscle. Within single-unit smooth muscle tissues, the autonomic nervous system innervates a single cell within a sheet or bundle and the action potential is propagated by...
.
Grade
In addition to being named based on the tissue of origin, sarcomas are also assigned a grade (low, intermediate, or high) based on the presence and frequency of certain cellular and subcellular characteristics associated with malignant biological behavior. Low grade sarcomas are usually treated surgically, although sometimes radiation therapyRadiation therapy
Radiation therapy , radiation oncology, or radiotherapy , sometimes abbreviated to XRT or DXT, is the medical use of ionizing radiation, generally as part of cancer treatment to control malignant cells.Radiation therapy is commonly applied to the cancerous tumor because of its ability to control...
or chemotherapy
Chemotherapy
Chemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....
are used. Intermediate and high grade sarcomas are more frequently treated with a combination of surgery
Surgery
Surgery is an ancient 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, or to help improve bodily function or appearance.An act of performing surgery may be called a surgical...
, chemotherapy
Chemotherapy
Chemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....
and/or radiation therapy
Radiation therapy
Radiation therapy , radiation oncology, or radiotherapy , sometimes abbreviated to XRT or DXT, is the medical use of ionizing radiation, generally as part of cancer treatment to control malignant cells.Radiation therapy is commonly applied to the cancerous tumor because of its ability to control...
. Since higher grade tumors are more likely to undergo metastasis
Metastasis
Metastasis, or metastatic disease , is the spread of a disease from one organ or part to another non-adjacent organ or part. It was previously thought that only malignant tumor cells and infections have the capacity to metastasize; however, this is being reconsidered due to new research...
(invasion and spread to locoregional and distant sites), they are treated more aggressively. The recognition that many 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
(ICD-O codes are provided where available.)- Askin's Tumor (8803/3)
- Botryodies
- ChondrosarcomaChondrosarcomaChondrosarcoma is a cancer composed of cells derived from transformed cells that produce cartilage. Chondrosarcoma is a member of a category of "soft tissue" malignancies known as sarcomas. About 30% of skeletal system cancers are chondrosarcomas...
(9220/3-9240/3) - Ewing's (9260/3) - PNETPrimitive neuroectodermal tumorPrimitive neuroectodermal tumor is a neural crest tumor. It is a rare tumor, usually occurring in children and young adults under 25 years of age...
(9473/3) - Malignant Hemangioendothelioma (9130/3)
- Malignant Schwannoma (9560/3-9561/3)
- OsteosarcomaOsteosarcomaOsteosarcoma is an aggressive cancerous neoplasm arising from primitive transformed cells of mesenchymal origin that exhibit osteoblastic differentiation and produce malignant osteoid...
(9180/3-9190/3) - Soft tissue sarcomaSoft tissue sarcomaA 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.-Risk factors:...
s, including:- Alveolar soft part sarcomaAlveolar soft part sarcomaAlveolar soft part sarcoma, abbreviated ASPS, is a very rare type of soft tissue sarcoma, that grows slowly and whose cell of origin is unknown.It arises mainly in children and young adults...
(9581/3) - AngiosarcomaAngiosarcomaAngiosarcoma is a malignant neoplasm of endothelial-type cells that line vessel walls. This may be in reference to blood or lymphatic vessels ....
(9120/3) - Cystosarcoma PhyllodesPhyllodes tumorPhyllodes tumors , also cystosarcoma phyllodes, cystosarcoma phylloides and phylloides tumor, are typically large, fast growing masses that form from the periductal stromal cells of the breast...
- Dermatofibrosarcoma protuberans (DFSP) (8832/3-8833/3)
- Desmoid TumorDesmoid tumorAggressive fibromatosis is a rare condition marked by the presence of desmoid tumors, which are benign, slow-growing tumors without any metastatic potential. However, Aggressive Fibromatosis is locally aggressive. Despite their benign nature, they can damage nearby structures causing organ...
(8821/1-8822/1) - Desmoplastic small round cell tumorDesmoplastic small round cell tumorDesmoplastic 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. Other areas affected may include the lymph nodes, the lining of the abdomen, diaphragm, spleen, liver, chest wall, skull, spinal cord, large...
(8806/3) - Epithelioid SarcomaEpithelioid sarcomaEpithelioid sarcoma is a rare mesenchymal soft tissue tumour with an epithelioid pattern. It often occurs in the extremities of young adults.-Diagnosis:...
(8804/3) - Extraskeletal chondrosarcoma (9220/3)
- Extraskeletal osteosarcoma (9180/3)
- FibrosarcomaFibrosarcomaFibrosarcoma is a malignant tumor derived from fibrous connective tissue and characterized by immature proliferating fibroblasts or undifferentiated anaplastic spindle cells.Usually in males ages 30 to 40. Originates in fibrous tissues of the bone. Invades long or flat bones such as femur, tibia,...
(8810/3) - HemangiopericytomaHemangiopericytomaA hemangeopericytoma is a type of soft tissue sarcoma that originates in the pericytes in the walls of capillaries. When inside the nervous system, although not strictly a meningioma tumor, it is a meningeal tumor with an especially aggressive behavior.It was characterized in...
(9150)(Also known as "solitary fibrous tumor". Only a subset of these tumors are classified as malignant.) - HemangiosarcomaHemangiosarcomaHemangiosarcoma is a rare, rapidly growing, highly invasive variety of cancer. It is a sarcoma arising from the lining of blood vessels; that is, blood-filled channels and spaces are commonly observed microscopically...
(9120/3) (More commonly referred to as "angiosarcoma") - Kaposi's sarcomaKaposi's sarcomaKaposi's sarcoma is a tumor caused by Human herpesvirus 8 , 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. It became more widely known as one of the AIDS defining...
(9140/3) - LeiomyosarcomaLeiomyosarcomaLeiomyosarcoma , aka LMS, is a malignant cancer of smooth muscle....
(8890/3-8896/3) - LiposarcomaLiposarcomaLiposarcoma is a malignant tumor that arises in fat cells 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.Liposarcomas, like all...
(8850/3-8858/3) - LymphangiosarcomaLymphangiosarcomaLymphangiosarcoma 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.-Signs and Symptoms:...
(9170-9175) - LymphosarcomaLymphomaLymphoma is a cancer in the lymphatic cells of the immune system. Typically, lymphomas present as a solid tumor of lymphoid cells. Treatment might involve chemotherapy and in some cases radiotherapy and/or bone marrow transplantation, and can be curable depending on the histology, type, and stage...
(Not considered to be sarcomas) - Malignant fibrous histiocytomaMalignant fibrous histiocytomaPleomorphic undifferentiated sarcoma , also undifferentiated pleomorphic sarcoma and previously malignant fibrous histiocytoma , is a type of soft tissue sarcoma....
(8830/3)(This is an obsolete term that is no longer recognized by the World Health Organization. Many of these tumors would currently be classified as "undifferentiated pleomorphic sarcoma".) - Malignant peripheral nerve sheath tumor (MPNST)
- Neurofibrosarcoma (9540/3)
- RhabdomyosarcomaRhabdomyosarcomaA rhabdomyosarcoma is a type of cancer, specifically a sarcoma , in which the cancer cells are thought to arise from skeletal muscle progenitors. It can also be found attached to muscle tissue, wrapped around intestines, or in any anatomic location...
(8900-8920) - Synovial sarcomaSynovial sarcomaA synovial sarcoma is a rare form of cancer which usually occurs near to the joints of the arm, neck or leg. It is one of the soft tissue sarcomas....
(9040/3-9043/3) - Undifferentiated pleomorphic sarcoma (previously referred to as Malignant fibrous histiocytomaMalignant fibrous histiocytomaPleomorphic undifferentiated sarcoma , also undifferentiated pleomorphic sarcoma and previously malignant fibrous histiocytoma , is a type of soft tissue sarcoma....
)
- Alveolar soft part sarcoma
Treatment
Surgery is important in the treatment of most sarcomas. Limb sparing surgery, as opposed to amputation, can now be used to save the limbs of patients in at least 90% of extremity tumor cases. Additional treatments, including chemotherapy and radiation therapy, may be administered before and/or after surgery. Chemotherapy significantly improves the prognosis for many sarcoma patients, especially those with bone sarcomas. Treatment can be a long and arduous process, lasting about a year for many patients, but the process is necessary in order to cure the disease.- LiposarcomaLiposarcomaLiposarcoma is a malignant tumor that arises in fat cells 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.Liposarcomas, like all...
treatment consists of surgical resection, with chemotherapy not being used outside of the experimental setting. Adjuvant radiotherapy may also be used after surgical excision for liposarcoma. - Rhabdomyosarcoma is treated with surgerySurgerySurgery is an ancient 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, or to help improve bodily function or appearance.An act of performing surgery may be called a surgical...
, radiotherapy, and/or chemotherapyChemotherapyChemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....
. The majority of rhabdomyosarcoma patients have between a 50-85% survival rate. - Osteosarcoma is treated with surgical resection of as much of the cancer as possible, often along with neoadjuvant chemotherapyNeoadjuvant chemotherapyNeoadjuvant therapy is the administration of therapeutic agents before a main treatment. One example is neoadjuvant hormone therapy prior to radical radiotherapy for adenocarcinoma of the prostate...
. Radiotherapy is a second alternative although not as successful.
Epidemiology
Sarcomas are quite rare with only 15,000 new cases per year in the United States. Sarcomas therefore represent about one percent of the 1.5 million new cancer diagnoses in that country each year, and are well below the US's orphan disease threshold of 200,000 cases per year.Gastrointestinal stromal tumor
Gastrointestinal stromal tumor
A gastrointestinal stromal tumor is one of the most common mesenchymal tumors of the gastrointestinal tract...
(GIST) is the most common form of sarcoma, with approximately 3,000-3,500 cases per year in the United States.
Sarcomas affect people of all ages. Approximately 50% of bone sarcomas and 20% of soft tissue sarcomas are diagnosed in people under the age of 35. Some sarcomas, such as leiomyosarcoma
Leiomyosarcoma
Leiomyosarcoma , aka LMS, is a malignant cancer of smooth muscle....
, chondrosarcoma
Chondrosarcoma
Chondrosarcoma is a cancer composed of cells derived from transformed cells that produce cartilage. Chondrosarcoma is a member of a category of "soft tissue" malignancies known as sarcomas. About 30% of skeletal system cancers are chondrosarcomas...
, and gastrointestinal stromal tumor
Gastrointestinal stromal tumor
A gastrointestinal stromal tumor is one of the most common mesenchymal tumors of the gastrointestinal tract...
(GIST), are more common in adults than in children. Most high-grade bone sarcomas, including Ewing's sarcoma and osteosarcoma
Osteosarcoma
Osteosarcoma is an aggressive cancerous neoplasm arising from primitive transformed cells of mesenchymal origin that exhibit osteoblastic differentiation and produce malignant osteoid...
, are much more common in children and young adults.