Home > Cancer and Treatments > Sarcoma: Soft Tissue, Connective Tissue & Bone Cancers

July is Sarcoma Awareness Month

Sarcomas are cancerous tumors that arise in the connective tissue of the body. Normal connective tissue includes fat, blood vessels, nerves, bones, muscles, deep skin tissues, and cartilage.

Each year, about 9,800 Americans are diagnosed with sarcoma, which can develop in both children and adults. With more than 50 different sub-types, sarcomas are divided into two main groups: bone tumors and soft tissue sarcomas. All sarcomas share certain microscopic characteristics and have similar symptoms.

Most sarcomas are soft tissue sarcomas, with about 7,800 diagnoses each year. But overall, soft tissue sarcomas comprise about 0.5% of all cancers diagnosed.


Sarcoma Types


Types of Soft Tissue Sarcoma
  • Liposarcoma
  • Fibrosarcoma
  • Dermatofibrosarcoma Protuberans
  • Malignant Fibrous Histiocytoma
  • Synovial Cell Sarcoma
  • Epitheloid Sarcomas
  • Rhabdomyosarcomas
  • Pleomorphic Rhabdomyosarcoma
  • Alveolar Rhabdomyosarcoma
  • Embryonal Rhabdomyosarcoma
  • Leiomyosarcomas
  • Extraosseous Tumors(Giant cell, osteosarcomas, Ewings)
  • Myoxoma
  • Mesenchymomas

Types of Vascular Sarcomas
  • Hemangioendothelioma
  • Angiosarcomas
  • Hemangiopericytoma
  • Malignant Neurilemoma(Malignant Schwannoma & Neurosarcoma)
  • Alveolar Soft-Parts Sarcoma
  • Kaposi's Sarcoma

The second group of sarcoma is bone cancer. It is very rare with approximately 2,000 new cases diagnosed in the United States each year. The incidence is slightly higher in males than females and no race has a higher incidence than another, although, Ewings sarcoma is even more rare among African and American blacks. Due to the rarity and severity of bone cancer, a bone cancer specialist such as an Orthopedic Oncologist should be consulted in the treatment of the disease.


Types of Bone Cancer
  • Parosteal osteogenic sarcoma
  • Periosteal osteosarcoma
  • Multifocal sclerosing osteogenic sarcoma
  • Osteogenic Sarcoma of the Jaw and Skull
  • Osteogenic Saroma in Paget's Disease
  • Postirradiation Osteogenic Sarcoma

Source: Sarcoma Alliance.


Uterine Sarcoma
Uterine sarcoma is a cancer of the muscle and supporting tissues of the uterus (womb).

Most uterine sarcomas fall into 1 of 2 categories, based on the type of cell they developed from:

  • Endometrial stromal sarcomas develop in the supporting connective tissue (stroma) of the endometrium.
  • Uterine leiomyosarcomas start in the muscular wall of the uterus known as the myometrium.

Another type of cancer that starts in the uterus is called carcinosarcoma. These cancers start in the endometrium and have features of both sarcomas and carcinomas. They can be classified with uterine sarcomas, but many doctors believe they are more closely related to carcinomas. These cancers are also known as malignant mixed mesodermal tumors or malignant mixed mullerian tumors.

Source: American Cancer Society.

Risk Factors


Anything that increases your risk of getting a disease is called a risk factor. Risk factors for soft tissue sarcoma include the following inherited disorders:
  • Retinoblastoma.
  • Neurofibromatosis type 1 (von Recklinghausen disease or NF1).
  • Tuberous sclerosis.
  • Familial adenomatous polyposis (FAP).
  • Li-Fraumeni syndrome.
  • Werner syndrome.
  • Basal cell nevus syndrome.

Other risk factors for soft tissue sarcoma include past treatments with radiation therapy during childhood or for certain types of cancer.

  • Retinoblastoma.
  • Breast cancer.
  • Lymphoma.
  • Cervical cancer.

Source: NCI

Prevention/Screening


Prevention

The only way to prevent some soft tissue sarcomas is to avoid exposure to risk factors whenever this is possible. However, most sarcomas develop in people with no known risk factors, so there is no way known at this time to prevent most cases. And for people receiving radiation therapy, there is usually little choice.

Source: American Cancer Society.


Screening

People with a strong family history of sarcomas or other cancers occurring at a young age may wish to discuss the benefits and disadvantages of genetic testing with their doctor. The genetic testing results should always be explained by a genetic counselor or a specially trained doctor who can interpret the results and advise high-risk patients about early cancer detection.

Families with a history of certain inherited conditions caused by mutated tumor suppressor genes have an increased risk of developing soft tissue sarcomas. The mutated genes can be detected by genetic testing, so family members should discuss this option with their doctors. They should let their doctor know about any lumps or growths right away.

No screening tests and exams are recommended for people who have no family history of sarcoma or other sarcoma risk factors. For these people, the best approach to early detection is to notify their health care professional of any unexplained lumps or growths or other symptoms that may be caused by a soft tissue sarcoma.

Source: American Cancer Society.

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.

Cancer Type, Point of Origin and Common Age

Osteosarcoma: Originates in osteoid, common locations: knees, upper legs, upper arms. Occurs between ages 10-25.

Chondrosarcoma: Originates in cartilage, common locations: pelvis, upper legs, shoulders. Occurs between ages 50-65.

Ewing's Sarcoma: Originates in immature nerve tissue, usually in bone marrow: pelvis, upper legs, ribs, arms. Occurs between ages 10-20.

Source: Sarcoma Alliance.

Take Action


Learn about the University of Michigan Comprehensive Cancer Center Multidisciplinary Sarcoma Clinic.

Clinical Trials Currently Enrolling at the University of Michigan

1. UMMC 2009.085 Randomized, Double-Blinded, Placebo-Controlled, Multi-institutional, Phase II.5 Study of AZD0530, a Selective Src Kinase Inhibitor, in Patients with Recurrent Osteosarcoma Localized to the Lung.

2. UMSS 2008.102 Randomized, open-label, multi-center phase III study to evaluate the efficacy and safety of nilotinib versus imatinib in adult patients with unresectable or metastatic gastrointestinal stromal tumors.

3. UMCC 2008.081 An Open-label, Multi-center, Phase 2 Study of Denosumab in Subjects with Giant Cell Tumor of Bone.

4. UMCC 2007.016/SARC 006 A phase II Trial of Neoadjuvant Chemotherapy in Sporadic and Neurofibromatosis Type I Associated High Grade Unresectable Malignant Peripheral Nerve Sheath Tumor.

5. UMCC 2005.075 A Multicenter, Open-label Single-arm Study of YONDELIS(trabectedin) for subjects with locally advanced or metastatic soft tissue sarcoma who have relapsed or are refractory to standard of care treatment.

6. UMCC 2004.010 Phase II Evaluation of Ifosfamide Plus Doxorubicin and Filgrastim Versus Gemcitabine Plus Docetaxel and Filgrastim in the treatment of localized poor prognosis soft tissue sarcoma.

Other Clinical Trials

ClinicalTrials.gov

Southwest Oncology Group/SWOG

American Cancer Society - What's New in Soft Tissue Sarcomas Research and Treatment?


Support at UM

UM Support Group [PDF]: Young Adults with Cancer(patients ages 18 to 30): walk-in, meets 1st Tuesday of each month, from 7-9pm in the Cancer Center

UM PsychOnc Clinic

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