Soft tissue sarcoma
Encyclopedia
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.
.
In 2006, about 9,500 new cases were diagnosed in the United States. Soft-tissue sarcomas are more commonly found in older patients (>50 years old) although in children and adolescents under age 20, certain histologies are common (rhabdomyosarcoma, synovial sarcoma).
analysis;
A pathologist examines the tissue under a microscope. If cancer is present, the pathologist can usually determine the type of cancer and its grade
. Here, 'grade' refers to a scale used to represent concisely the predicted growth rate of the tumour and its tendency to spread, and this is determined by the degree to which the cancer cells appear abnormal when examined under a microscope. Low-grade sarcomas, although cancerous, are defined as those that are less likely to metastasise
. High-grade sarcomas are defined as those more likely to spread to other parts of the body.
Soft tissue sarcomas commonly originate in the upper body, in the shoulder or upper chest. Some symptoms are uneven posture, pain in the trapezius muscle and cervical
inflexibility [difficulty in turning the head].
The most common site to which soft tissue sarcoma spreads is the lungs.
A combination of Taxotere and Gemzar could be an effective chemotherapy regimen in patients with advanced soft-tissue sarcoma.
An earlier version of this article was taken from the US
National Cancer Center
's Cancer Information Service.
Sarcoma
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...
that develops in connective tissue
Connective tissue
"Connective tissue" is a fibrous tissue. It is one of the four traditional classes of tissues . Connective Tissue is found throughout the body.In fact the whole framework of the skeleton and the different specialized connective tissues from the crown of the head to the toes determine the form of...
, though the term is sometimes applied to elements of the soft tissue
Soft tissue
In anatomy, the term soft tissue refers to tissues that connect, support, or surround other structures and organs of the body, not being bone. Soft tissue includes tendons, ligaments, fascia, skin, fibrous tissues, fat, and synovial membranes , and muscles, nerves and blood vessels .It is sometimes...
that are not currently considered connective tissue.
Risk factors
Most soft-tissue sarcomas are not associated with any known risk factors or identifiable etiology. There are some exceptions:- Studies suggest that workers who are exposed to chlorophenolChlorophenolA chlorophenol is any organochloride of phenol that contains one or more covalently bonded chlorine atoms. Chlorophenols are produced by electrophilic halogenation of phenol with chlorine. Most chlorophenols have a number of different isomers...
s in wood preservatives and phenoxy herbicidePhenoxy herbicideA phenoxy herbicide is any member of a family of chemicals related to the growth hormone indoleacetic acid . When sprayed on broad-leaf plants they induce rapid, uncontrolled growth, eventually killing them. When sprayed on crops such as wheat or corn, it selectively kills just the broad-leaf...
s may have an increased risk of developing soft-tissue sarcomas. An unusual percentage of patients with a rare blood vessel tumor, angiosarcomaAngiosarcomaAngiosarcoma is a malignant neoplasm of endothelial-type cells that line vessel walls. This may be in reference to blood or lymphatic vessels ....
of the liver, have been exposed to vinyl chlorideVinyl chlorideVinyl chloride is the organochloride with the formula H2C:CHCl. It is also called vinyl chloride monomer, VCM or chloroethene. This colorless compound is an important industrial chemical chiefly used to produce the polymer polyvinyl chloride . At ambient pressure and temperature, vinyl chloride...
in their work. This substance is used in the manufacture of certain plastics, notably PVCPolyvinyl chloridePolyvinyl chloride, commonly abbreviated PVC, is a thermoplastic polymer. It is a vinyl polymer constructed of repeating vinyl groups having one hydrogen replaced by chloride. Polyvinyl chloride is the third most widely produced plastic, after polyethylene and polypropylene. PVC is widely used in...
.
- In the early 1900s, when scientists were just discovering the potential uses of radiationRadiationIn physics, radiation is a process in which energetic particles or energetic waves travel through a medium or space. There are two distinct types of radiation; ionizing and non-ionizing...
to treat disease, little was known about safe dosage levels and precise methods of delivery. At that time, radiation was used to treat a variety of noncancerous medical problems, including enlargement of the tonsils, adenoids, and thymus gland. Later, researchers found that high doses of radiation caused soft-tissue sarcomas in some patients. Because of this risk, radiation treatment for cancer is now planned to ensure that the maximum dosage of radiation is delivered to diseased tissue while surrounding healthy tissue is protected as much as possible.
- 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...
, a rare cancer of the cells that line blood vessels in the skin and mucus membranes, is caused by Human herpesvirus 8. Kaposi's sarcoma often occurs in patients with AIDSAIDSAcquired immune deficiency syndrome or acquired immunodeficiency syndrome is a disease of the human immune system caused by the human immunodeficiency virus...
(acquired immune deficiency syndrome). Kaposi's sarcoma, however, has different characteristics than typical soft-tissue sarcomas and is treated differently.
- In a very small fraction of cases, sarcoma may be related to a rare inherited genetic alteration of the p53P53p53 , is a tumor suppressor protein that in humans is encoded by the TP53 gene. p53 is crucial in multicellular organisms, where it regulates the cell cycle and, thus, functions as a tumor suppressor that is involved in preventing cancer...
gene and is known as Li-Fraumeni syndromeLi-Fraumeni syndromeLi-Fraumeni syndrome is an extremely rare autosomal dominant hereditary disorder. It is named after Frederick Pei Li and Joseph F. Fraumeni, Jr., the American physicians who first recognized and described the syndrome. Li-Fraumeni syndrome greatly increases susceptibility to cancer...
. Certain other inherited diseases are associated with an increased risk of developing soft-tissue sarcomas. For example, people with neurofibromatosis type INeurofibromatosis type INeurofibromatosis type I , formerly known as von Recklinghausen disease after the researcher who first documented the disorder, is a human genetic disorder. It is possibly the most common inherited disorder caused by a single gene...
(also called von Recklinghausen's disease, associated with alterations in the NF1 gene) are at an increased risk of developing soft-tissue sarcomas known as malignant peripheral nerve sheath tumors. Patients with inherited retinoblastoma have alterations in the RB1 gene, a tumor suppressor gene, and are likely to develop soft-tissue sarcomas as they mature into adulthood.
Frequency
Soft-tissue sarcomas are relatively uncommon cancers. They account for less than 1% of all new cancer cases each year. This may be because cells in soft tissue, in contrast to tissues that more commonly give rise to malignancies, are not continuously dividing cellsLabile cell
In cellular biology, labile cells are cells that multiply constantly throughout life. They spend little or no time in the quiescent G0 phase of the cell cycle, but regularly perform cell division.This is the case for only a minority of cells in the body...
.
In 2006, about 9,500 new cases were diagnosed in the United States. Soft-tissue sarcomas are more commonly found in older patients (>50 years old) although in children and adolescents under age 20, certain histologies are common (rhabdomyosarcoma, synovial sarcoma).
Symptoms
In their early stages, soft-tissue sarcomas usually do not cause symptoms. Because soft tissue is relatively elastic, tumors can grow rather large, pushing aside normal tissue, before they are felt or cause any problems. The first noticeable symptom is usually a painless lump or swelling. As the tumor grows, it may cause other symptoms, such as pain or soreness, as it presses against nearby nerves and muscles. If in the abdomen it can cause abdominal pains commonly mistaken for menstrual cramps, indigestion, or cause constipation.Diagnosis
The only reliable way to determine whether a soft-tissue tumour is benign or malignant is through a biopsy. There are two methods for acquisition of tumour tissue for cytopathologicalCytopathology
Cytopathology is a branch of pathology that studies and diagnoses diseases on the cellular level. The discipline was founded by Rudolf Virchow in 1858. A common application of cytopathology is the Pap smear, used as a screening tool, to detect precancerous cervical lesions and prevent cervical...
analysis;
- Needle AspirationNeedle aspiration biopsyNeedle aspiration biopsy , may refer to fine needle aspiration cytology , fine needle aspiration biopsy and fine needle aspiration , is a diagnostic procedure sometimes used to investigate superficial lumps or masses...
, via biopsy needleHypodermic needleA hypodermic needle is a hollow needle commonly used with a syringe to inject substances into the body or extract fluids from it... - surgically, via an incision made into the tumour.
A pathologist examines the tissue under a microscope. If cancer is present, the pathologist can usually determine the type of cancer and its grade
Grading (tumors)
In pathology, grading is a measure of the cell appearance in tumors and other neoplasms. Some pathology grading systems apply only to malignant neoplasms ; others apply also to benign neoplasms. The neoplastic grading is a measure of cell anaplasia in the sampled tumors arising from the...
. Here, 'grade' refers to a scale used to represent concisely the predicted growth rate of the tumour and its tendency to spread, and this is determined by the degree to which the cancer cells appear abnormal when examined under a microscope. Low-grade sarcomas, although cancerous, are defined as those that are less likely to metastasise
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...
. High-grade sarcomas are defined as those more likely to spread to other parts of the body.
Soft tissue sarcomas commonly originate in the upper body, in the shoulder or upper chest. Some symptoms are uneven posture, pain in the trapezius muscle and cervical
Neck
The neck is the part of the body, on many terrestrial or secondarily aquatic vertebrates, that distinguishes the head from the torso or trunk. The adjective signifying "of the neck" is cervical .-Boner anatomy: The cervical spine:The cervical portion of the human spine comprises seven boney...
inflexibility [difficulty in turning the head].
The most common site to which soft tissue sarcoma spreads is the lungs.
Treatment
In general, treatment for soft-tissue sarcomas depends on the stage of the cancer. The stage of the sarcoma is based on the size and grade of the tumor, and whether the cancer has spread to the lymph nodes or other parts of the body (metastasized). Treatment options for soft-tissue sarcomas include surgery, radiation therapy, and chemotherapy.- Surgery is the most common treatment for soft-tissue sarcomas. If possible, the doctor will remove the cancer and a safe margin of the healthy tissue around it. It is important to obtain a margin free of tumor to decrease the likelihood of local recurrence and give the best chance for eradication of the tumor. Depending on the size and location of the sarcoma, it may, rarely, be necessary to remove all or part of an arm or leg.
- Radiation therapyRadiation therapyRadiation 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...
may be used either before surgery to shrink tumors or after surgery to kill any cancer cells that may have been left behind. In some cases, it can be used to treat tumours that cannot be surgically removed. In multiple studies, radiation therapy has been found to improve the rate of local control, but has not had any influence on overall survival. - ChemotherapyChemotherapyChemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....
may be used with radiation therapy either before or after surgery to try to shrink the tumor or kill any remaining cancer cells. The use of chemotherapy to prevent the spread of soft-tissue sarcomas has not been proven to be effective. If the cancer has spread to other areas of the body, chemotherapy may be used to shrink tumors and reduce the pain and discomfort they cause, but is unlikely to eradicate the disease.
A combination of Taxotere and Gemzar could be an effective chemotherapy regimen in patients with advanced soft-tissue sarcoma.
Tables
| Tissue of Origin | Type of Cancer | Usual Location in the Body |
| Fibrous tissue | Fibrosarcoma Fibrosarcoma Fibrosarcoma 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,... | Arms, legs, trunk |
| Malignant fibrous hystiocytoma Malignant fibrous histiocytoma Pleomorphic undifferentiated sarcoma , also undifferentiated pleomorphic sarcoma and previously malignant fibrous histiocytoma , is a type of soft tissue sarcoma.... | Legs | |
| Dermatofibrosarcoma | Trunk | |
| Fat | 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... | Arms, legs, trunk |
| Muscle | 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. It can also be found attached to muscle tissue, wrapped around intestines, or in any anatomic location... Leiomyosarcoma Leiomyosarcoma Leiomyosarcoma , aka LMS, is a malignant cancer of smooth muscle.... | Arms, legs Uterus, digestive tract |
| Blood vessels | Hemangiosarcoma Hemangiosarcoma Hemangiosarcoma 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... | Arms, legs, trunk |
| Kaposi's sarcoma Kaposi's sarcoma Kaposi'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... | Legs, trunk | |
| Lymph vessels | 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.-Signs and Symptoms:... | Arms |
| Synovial tissue (linings of joint cavities, tendon sheaths) | Synovial sarcoma Synovial sarcoma A 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.... | Legs |
| Peripheral nerves | Malignant peripheral nerve sheath tumour/Neurofibrosarcoma | Arms, legs, trunk |
| Cartilage and bone-forming tissue | Extraskeletal chondrosarcoma | Legs |
| Extraskeletal osteosarcoma | Legs, trunk (not involving the bone) |
| Tissue of Origin | Type of Cancer | Usual Location in the Body | Most common ages |
| Muscle | |||
| Rhabdomyosarcoma | |||
| Head and neck, genitourinary tract | Infant–4 | ||
Alveolar soft part sarcoma Alveolar 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... | Arms, legs, head, and neck | Infant–19 | |
| Leiomyosarcoma | Trunk | 15–19 | |
| Fibrous tissue | Fibrosarcoma | Arms and legs | 15–19 |
| Malignant fibrous histiocytoma | Legs | 15–19 | |
| Dermatofibrosarcoma | Trunk | 15–19 | |
| Fat | Liposarcoma | Arms and Legs | 15–19 |
| Blood vessels | Infantile hemangio- | Arms, legs, trunk, head, and neck | Infant–4 |
| Synovial tissue (linings of joint cavities, tendon sheaths) | Synovial sarcoma | Legs, arms, and trunk | 15–19 |
| Peripheral nerves | Malignant peripheral nerve sheath tumors (also called neurofibrosarcomas, malignant schwannomas, and neurogenic sarcomas) | Arms, legs, and trunk | 15–19 |
| Muscular nerves | Alveolar soft part sarcoma Alveolar soft part sarcoma Alveolar 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... | Arms and legs | Infant–19 |
| Cartilage and bone-forming tissue | Extraskeletal myxoid chondrosarcoma Extraskeletal myxoid chondrosarcoma Myxoid chondrosarcoma is a type of sarcoma.It has been associated with a t EWS/CHN gene fusion.... | Legs | 10–14 |
| Extraskeletal mesenchymal | Legs | 10–14 |
An earlier version of this article was taken from the US
United States
The United States of America is a federal constitutional republic comprising fifty states and a federal district...
National Cancer Center
National Cancer Institute
The National Cancer Institute is part of the National Institutes of Health , which is one of 11 agencies that are part of the U.S. Department of Health and Human Services. The NCI coordinates the U.S...
's Cancer Information Service.
Notable patients
- Runner Terry FoxTerry FoxTerrance Stanley "Terry" Fox , was a Canadian humanitarian, athlete, and cancer research activist. In 1980, with one leg having been amputated, he embarked on a cross-Canada run to raise money and awareness for cancer research...
died from osteosarcomaOsteosarcomaOsteosarcoma is an aggressive cancerous neoplasm arising from primitive transformed cells of mesenchymal origin that exhibit osteoblastic differentiation and produce malignant osteoid...
. - Actor Robert UrichRobert UrichRobert Urich was an American actor. He played the starring roles in the television series Vega$ and Spenser: For Hire...
died from 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....
. - Actress Michelle ThomasMichelle ThomasMichelle Thomas was an American actress. She was best known for her roles as Justine Phillips on the NBC sitcom The Cosby Show, and as Myra Monkhouse on the ABC/CBS sitcom Family Matters.-Early life and career:Born in Boston, Massachusetts, Thomas grew up in Montclair, New Jersey and was an...
died from desmoplastic small-round-cell tumor, a rare abdominal soft-tissue sarcoma. - It Is WrittenIt Is WrittenIt Is Written is an internationally broadcast Christian television program founded in 1956 by George Vandeman. Its title comes from the Gospel of Matthew: "It is written, 'Man shall not live by bread alone, but by every word that proceeds out of the mouth of God.'" The programs are produced by the...
evangelist Henry Fayenbrand died from sarcoma in his leg.
Notable experts
- Retired pathologist Professor Clair du BoulayClair du BoulayProfessor Clair du Boulay FRCPath OBE is a retired British expert in pathology and medical education. She was Vice-President of the Royal College of Pathologists from 2002–2005 and became an Officer of the Order of the British Empire in 2008.-Career:...
OBE specialised in soft-tissue sarcomas at the University of SouthamptonUniversity of SouthamptonThe University of Southampton is a British public university located in the city of Southampton, England, a member of the Russell Group. The origins of the university can be dated back to the founding of the Hartley Institution in 1862 by Henry Robertson Hartley. In 1902, the Institution developed...
.
External links
- Factsheet from the National Cancer InstituteNational Cancer InstituteThe National Cancer Institute is part of the National Institutes of Health , which is one of 11 agencies that are part of the U.S. Department of Health and Human Services. The NCI coordinates the U.S...
- Information from Memorial Sloan Kettering
- The Sarcoma Learning Center
- www.soft-tissue.com - a database of review questions on soft tissue sarcomas
- Clinically reviewed soft-tissue sarcoma information for patients, from Cancer Research UKCancer Research UKCancer Research UK is a cancer research and awareness charity in the United Kingdom, formed on 4 February 2002 by the merger of The Cancer Research Campaign and the Imperial Cancer Research Fund. Its aim is to reduce the number of deaths from cancer. As the world's largest independent cancer...
.