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About Soft Tissue Sarcoma

Overview and Types

If you've been diagnosed with soft tissue sarcoma or are worried about it, you likely
have a lot of questions. Learning some basics is a good place to start.

● What Is a Soft Tissue Sarcoma?

Research and Statistics

See the latest estimates for new cases of soft tissue sarcoma and deaths in the US and
what research is currently being done.

● Key Statistics for Soft Tissue Sarcomas

● What's New in Soft Tissue Sarcoma Research?

What Is a Soft Tissue Sarcoma?

Cancer starts when cells start to grow out of control. Cells in nearly any part of the body
can become cancer and can spread to other areas. To learn more about how cancers
start and spread, see What Is Cancer?1

There are many types of soft tissue tumors, and not all of them are cancerous. Many
benign tumors are found in soft tissues. The word benign means they're not cancer.
These tumors can't spread to other parts of the body. Some soft tissue tumors behave

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in ways between a cancer and a non-cancer. These are called intermediate soft
tissue tumors.

When the word sarcoma is part of the name of a disease, it means the tumor is
malignant (cancer).A sarcoma is a type of cancer that starts in tissues like bone or
muscle. Bone and soft tissue sarcomas are the main types of sarcoma. Soft tissue
sarcomas can develop in soft tissues like fat, muscle, nerves, fibrous tissues, blood
vessels, or deep skin tissues. They can be found in any part of the body. Most of them
start in the arms or legs. They can also be found in the trunk, head and neck area,
internal organs, and the area in back of the abdominal (belly) cavity (known as the
retroperitoneum). Sarcomas are not common tumors.

Sarcomas that most often start in bones, such as osteosarcomas2, and sarcomas that
are most often seen in children, such as the Ewing Family of Tumors3 and
Rhabdomyosarcoma4, are not covered here.

Types of soft tissue sarcomas

There are more than 50 different types of soft tissue sarcomas. Some are quite rare,
and not all are listed here:

● Adult fibrosarcoma usually affects fibrous tissue in the legs, arms, or trunk. It's
most common in people between the ages of 20 and 60, but can occur in people of
any age, even in infants.
● Alveolar soft-part sarcoma is a rare cancer that mostly affects young adults.
These tumors most commonly start in legs.
● Angiosarcoma can start in blood vessels (hemangiosarcomas) or in lymph
vessels (lymphangiosarcomas). These tumors sometimes start in a part of the
body that has been treated with radiation. Angiosarcomas are sometimes seen in
the breast after radiation therapy and in limbs with lymphedema5.
● Clear cell sarcoma is a rare cancer that often starts in tendons of the arms or legs.
Under the microscope, it has some features of malignant melanoma6, a type of
cancer that starts in pigment-producing skin cells. How cancers with these features
start in parts of the body other than the skin is not known.
● Desmoplastic small round cell tumor is a rare sarcoma of teens and young
adults. It's found most often in the abdomen (belly).
● Epithelioid sarcoma most often starts in tissues under the skin of the hands,
forearms, feet, or lower legs. Teens and young adults are often affected.
● Fibromyxoid sarcoma, low-grade is a slow-growing cancer that most often starts

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as a painless growth in the trunk or arms and legs (particularly the thigh). It is more
common in young to middle aged adults. It is sometimes called an Evans’ tumor.
● Gastrointestinal stromal tumor (GIST) is a type of sarcoma that starts in the
digestive tract. See Gastrointestinal Stromal Tumor (GIST)7 for more details.
● Kaposi sarcoma is a type of sarcoma that starts in the cells lining lymph or blood
vessels. See Kaposi Sarcoma8.
● Leiomyosarcoma is a type of cancer that starts in smooth muscle tissue. These
tumors often start in the abdomen, but they can also start in other parts of the body,
such as the arms or legs, or in the uterus (see Uterine Sarcoma9).
● Liposarcomas are malignant tumors of fat tissue. They can start anywhere in the
body, but they most often start in the thigh, behind the knee, and inside the back of
the abdomen (belly). They occur mostly in adults between 50 and 65 years old.
● Malignant mesenchymomais a rare type of sarcoma that shows features of
fibrosarcoma and features of at least 2 other types of sarcoma.
● Malignant peripheral nerve sheath tumors include neurofibrosarcomas,
malignant schwannomas, and neurogenic sarcomas. These are sarcomas that
start in the cells that surround a nerve.
● Myxofibrosarcomas, low-grade are most often found in the arms and legs of
elderly patients. They are most common in or just under the skin and there might be
more than one tumor.
● Rhabdomyosarcoma is the most common type of soft tissue sarcoma seen in
children. See Rhabdomyosarcoma10.
● Synovial sarcomais a malignant tumor of the tissue around joints. The most
common locations are the hip, knee, ankle, and shoulder. This tumor is more
common in children and young adults, but it can occur in older people.
● Undifferentiated pleomorphic sarcoma (UPS) was once called malignant
fibrous histiocytoma (MFH). It's most often found in the arms or legs. Less often,
it can start inside at the back of the abdomen (the retroperitoneum). This sarcoma
is most common in older adults. It mostly tends to grow into other tissues around
the place it started, but it can spread to distant parts of the body.

Intermediate soft tissue tumors

These may grow and invade nearby tissues and organs, but they tend to not spread to
other parts of the body.

● Dermatofibrosarcoma protuberans is a slow-growing cancer of the fibrous tissue

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beneath the skin, usually in the trunk or limbs. It grows into nearby tissues but
rarely spreads to distant sites.
● Fibromatosisis the name given to fibrous tissue tumor with features in between
fibrosarcoma and benign tumors such as fibromas and superficial fibromatosis.
They tend to grow slowly but, often, steadily. They are also called desmoid
tumors, as well as the more scientific name musculoaponeurotic fibromatosisor
just aggressive fibromatosis. They rarely, if ever, spread to distant sites, but they do
cause problems by growing into nearby tissues. They can sometimes be fatal.
Some doctors consider them a type of low-grade fibrosarcoma; but others believe
they are a unique type of fibrous tissue tumors. Certain hormones, like estrogen,
make some desmoid tumors grow. Anti-estrogen drugs are sometimes useful in
treating desmoids that cannot be completely removed by surgery.
● Hemangioendothelioma is a blood vessel tumor that is considered a low-grade
cancer (meaning it grows slowly and is slow to spread). It does grow into nearby
tissues and sometimes can spread to distant parts of the body. It may start in soft
tissues or in internal organs, such as the liver or lungs.
● Infantile fibrosarcoma is the most common soft tissue sarcoma in children under
one year of age. It tends to be slow-growing and is less likely to spread to other
organs than adult fibrosarcomas.
● Solitary fibrous tumors are most often not cancer (benign) but can be cancer
(malignant). Some start in the thigh, underarm, and pelvis. They can also start in
the tissue surrounding the lung (called the pleura). Many tumors that were once
called hemangiopericytomas are now considered solitary fibrous tumors.

Benign soft tissue tumors

Many benign tumors, or tumors that are not cancer, can start in soft tissues. These
include:

● Elastofibromas: benign tumors of fibrous tissue

● Fibromas: benign tumors of fibrous tissue
● Fibrous histiocytomas: benign tumors of fibrous tissue
● Glomus tumors: benign tumors that occur near blood vessels
● Granular cell tumors: usually benign tumors in adults that often start in the tongue
but can be found almost anywhere in the body
● Hemangiomas: benign tumors of blood vessels
● Hibernomas: benign tumors of fat tissue

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● Lipomas: very common benign tumors of fat tissue

● Leiomyomas: benign tumors of smooth muscle that can be found anywhere in the
body but are very common in the walls of the uterus where they are known as
fibroids
● Lipoblastomas: benign fat tissue tumors most often seen in children
● Lymphangiomas: benign tumors of lymph vessels
● Myxomas: benign tumors that usually are in muscles but do not start from muscle
cells
● Neurofibromas: tumors of nerve tissue that are usually benign. Neurofibromas of
large nerves, such as those in the upper arms or neck can become cancer.
Neurofibromas are very common in people with an inherited condition called
neurofibromatosis (also called von Recklinghausen disease) They're much less
common in people without this condition.
● Neuromas: benign tumors of nerves that can be painful
● PEComas: a family of tumors made up of abnormal cells called perivascular
epithelial cells. Although most of these tumors are benign, some rare PEComas
are malignant (cancer). The most common types of PEComas are
angiomyolipomas and lymphangioleiomyomas. Angiomyolipoma is a benign
tumor that most often affects the kidney. Lymphangioleiomyomatosis (or LAM) is a
rare disease of women in which the many lymphangioleiomyoma tumors grow into
the lung tissue and interfere with lung function.
● Rhabdomyomas: benign tumors of skeletal and heart muscle
● Schwannomas (neurilemmomas): benign tumors of the cells that coat nerves
● Tenosynovial giant cell tumors (also called nodular tenosynovitis): benign
tumors of joint tissue

Spindle cell tumors

Spindle cell tumor and spindle cell sarcoma are descriptive names used because the
cells look long and narrow under the microscope. Spindle cell tumor is not a specific
diagnosis or a specific type of cancer. The tumor may be a sarcoma, or it can be
sarcomatoid— meaning another type of tumor (like a carcinoma) that looks like a
sarcoma under the microscope.

Tumor-like conditions of soft tissue

Some changes in soft tissues are caused by inflammation or injury and can form a mass

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that looks like a soft tissue tumor. Unlike a real tumor, they don't come from a single
abnormal cell, they have limited ability to grow or spread to nearby tissues, and never
spread through the bloodstream or lymph system to other parts of the body. Nodular
fasciitis and myositis ossificansare 2 examples which affect tissues under the skin
and muscle tissues, respectively.

Hyperlinks

1. www.cancer.org/treatment/understanding-your-diagnosis/what-is-cancer.html
2. www.cancer.org/cancer/osteosarcoma.html
3. www.cancer.org/cancer/ewing-tumor.html
4. www.cancer.org/cancer/rhabdomyosarcoma.html
5. www.cancer.org/treatment/treatments-and-side-effects/physical-side-
effects/lymphedema.html
6. www.cancer.org/cancer/melanoma-skin-cancer.html
7. www.cancer.org/cancer/gastrointestinal-stromal-tumor.html
8. www.cancer.org/cancer/kaposi-sarcoma.html
9. www.cancer.org/cancer/uterine-sarcoma.html
10. www.cancer.org/cancer/rhabdomyosarcoma.html

References

Helman LJ, Maki RG. Sarcomas of soft tissue. In: Niederhuber JE, Armitage JO,
Doroshow JH, Kastan MB, Tepper JE. Abeloff‘s Clinical Oncology. 5th ed. Philadelphia,
PA. Elsevier: 2014: 1753-1791.

MacNeill AJ, Gupta A, Swallow CJ. Randomized Controlled Trials in Soft Tissue
Sarcoma: We Are Getting There! Surg Oncol Clin Am. 2017;26:531-544.

Medscape. Benign and Malignant Soft-Tissue Tumors. March 27, 2017. Accessed at
https://emedicine.medscape.com/article/1253816-overview on March 27, 2018.

National Comprehensive Cancer Network, Clinical Practice Guidelines in Oncology

(NCCN Guidelines®), Soft Tissue Sarcoma, Version 1.2018 -- October 31, 2017.
Accessed at www.nccn.org/professionals/physician_gls/pdf/sarcoma.pdf on March 27,
2018.

Singer S, Maki R, O’Sullivan B. Soft tissue sarcoma In: DeVita VT, Heilman S,
Rosenberg SA, eds. Cancer: Principles and Practice of Oncology. 9th ed. Philadelphia,

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Pa: Lippincott Williams & Wilkins; 2011:1533-1577.

Villalobos VM, Byfield SD, Ghate SR, Adejoro O. A retrospective cohort study of
treatment patterns among patients with metastatic soft tissue sarcoma in the US. Clin
Sarcoma Res. 2017;7:18.

Last Revised: November 23, 2021

Key Statistics for Soft Tissue Sarcomas

The American Cancer Society's estimates for soft tissue sarcomas in the United States
for 2022 are:

● About 13,190 new soft tissue sarcomas will be diagnosed (7,590 in males and
5,600 in females).
● About 5,130 people (2,740 males and 2,390 females) are expected to die of soft
tissue sarcomas.

These statistics include both adults and children.

The most common types of sarcoma in adults are:

● Undifferentiated pleomorphic sarcoma (previously called malignant fibrous

histiocytoma)
● Liposarcoma
● Leiomyosarcoma

Certain types occur more often in certain parts of the body more often than others. For
example, leiomyosarcomas are the most common type of sarcoma found in the
abdomen (belly), while liposarcomas and undifferentiated pleomorphic sarcomas are
most common in legs. But pathologists (doctors who specialize in diagnosing cancers
by how they look under the microscope), may not always agree on the exact type of
sarcoma. Sarcomas of uncertain type are very common.

Visit the American Cancer Society’s Cancer Statistics Center1 for more key statistics.

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Hyperlinks

1. cancerstatisticscenter.cancer.org/

References

American Cancer Society. Cancer Facts & Figures 2022. Atlanta, Ga: American Cancer
Society; 2022.

Last Revised: January 12, 2022

What's New in Soft Tissue Sarcoma

Research?
Research is ongoing in the area of soft tissue sarcomas . Because soft tissue sarcomas
are rare and there are so many different types, it's has been hard to study it well. Still,
scientists are learning more about causes and genetic differences in types of sarcomas,
and they're looking for ways to improve treatments.

Basic research

Scientists have made progress in understanding how certain gene changes in soft
tissue cells cause sarcomas to develop. This information is already being used in new
tests to diagnose and classify sarcomas. This is important because knowing the exact
type of sarcoma will help doctors select treatment tailored for each person. It's hoped
that this information will also lead to new ways to treat these cancers, based on specific
differences between normal and cancer soft tissue cells.

Classification

Classification of most cancers, including sarcomas, is based mostly on the way they
look under a microscope. Recent research has shown that several different kinds of soft
tissue sarcomas can look a lot alike under the microscope. By using new tests,

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researchers have found that most cancers that used to be called malignant fibrous
histiocytoma (MFH) are actually high-grade forms of liposarcoma,
rhabdomyosarcoma1, leiomyosarcoma, other sarcomas, and even carcinomas or
lymphomas. Tests to clearly classify the many types of soft tissue sarcoma are another
key to deciding on the best treatment for each person.

Treatment

Researchers are looking for new and better ways to combine treatments, for example,
using surgery, radiation, and chemo together, as well as new ways to treat soft tissue
sarcomas.

Radiation

Doctors are looking at the best way to use radiation treatment. Studies are comparing
radiation use before vs. after surgery to find out which has a greater impact on wound
healing and long-term side effects. They're also looking at different types , doses, and
schedules for radiation in an effort to find better and safer ways to use this treatment.
Research is also being done to figure out when radiation is needed after surgery and
when it's not.

Chemotherapy

Active research in chemotherapy for soft tissue sarcomas includes studies of new drugs
and new ways to give the drugs that are available.

Targeted therapy

There's a lot of active research on the use of targeted drugs. These drugs specifically
block substances in or on cancer cells that cause the cancers to grow. Targeted
treatments are used for many kinds of cancer and doctors are trying to find out if they
might also be helpful against sarcomas.

Anti-angiogenesis drugs

Drugs that block new blood vessel formation may help kill sarcomas by keeping them
from being fed by blood vessels. These drugs are being tested in many studies.

Other treatments

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Many other treatments are being tested and are only available in clinical trials.
Examples include vaccine treatments and T-cell therapies for people with advanced soft
tissue sarcomas. The use of heat (hyperthermia) and cold (cryosurgery) to destroy
tumors is also being explored. Most of these studies are in very early stages, and it will
be awhile before doctors know that they work well enough to be part of regular
treatment for soft tissue sarcoma.

Hyperlinks

1. www.cancer.org/cancer/rhabdomyosarcoma.html

References

Fiore M, Ford S, Callegaro D, et al. Adequate Local Control in High-Risk Soft Tissue
Sarcoma of the Extremity Treated with Surgery Alone at a Reference Centre: Should
Radiotherapy Still be a Standard? Ann Surg Oncol. 2018 Feb 22.

Last Revised: April 6, 2018

Written by

The American Cancer Society medical and editorial content team

(www.cancer.org/cancer/acs-medical-content-and-news-staff.html)

Our team is made up of doctors and oncology certified nurses with deep knowledge of
cancer care as well as journalists, editors, and translators with extensive experience in
medical writing.

American Cancer Society medical information is copyrighted material. For reprint

requests, please see our Content Usage Policy (www.cancer.org/about-
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