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by Dr. C.H. Weaver M.D. Medical Editor 6/2022

Sarcomas are a varied group of cancers arising from fibrous tissue, adipose tissue (fat cells), skeletal muscle, smooth muscle, blood vessels, lymph vessels, nervous tissue, mesenchymal cells, bone, and cartilage. These cancers vary widely from benign tumors that can be cured by surgery to highly malignant wide spread cancers that need to be treated with multi-modality therapy (surgery, radiation therapy, and chemotherapy). 

Sarcomas comprise 3% of all cancers in adults and 7% of cancers in children. Because of the rarity of each type of sarcoma, it may be important for patients to be treated in specialty centers with access to clinical research studies.

Risk factors for developing a STS include past treatment with radiation, exposure to certain chemicals such as Thorotrast (thorium dioxide), vinyl chloride or arsenic.

Sarcoma Bone CancerConnect 490

Treatment is Tailored to the Specific Type of Sarcoma

  • Osteosarcoma
  • Ewing’s Sarcoma and Primitive Neuroectodermal Tumors (PNET)
  • Soft Tissue Sarcoma(Adult and Childhood): Alveolar Soft-part Sarcoma Angiosarcoma Chrondrosarcoma Dermatofibrosarcoma Protuberans Epithilioid Sarcoma Fibrosarcoma Leiomyosarcoma Liposarcoma Malignant Fibrous Histiocytoma Malignant Hemangiopericytoma Malignant Mesenchymoma, Malignant Schwannoma (Malignant Peripheral Nerve Sheath Tumor) Non-Bone Osterosarcoma Peripheral Neuroectodermal Tumors Synovial Sarcoma Unidentifiable Sarcomas Rhabdomyosarcoma Childhood Adult Kaposi’s Sarcoma Gastrointestinal Stromal Sarcoma (GIST)

Systemic Therapy

Surgery is the cornerstone of sarcoma treatment however many patients with ultimately need systemic therapy. Systemic therapy is any treatment directed at destroying cancer cells throughout the body. Many patients have small amounts of cancer that have spread away from their site of origin or remain after surgery. These cancer cells are difficult to detect and are referred to as micro-metastatic or minimal residual disease (MRD). Their presence is what causes a cancer recurrence following local treatment with surgery and/or radiation therapy. An effective systemic treatment is needed to cleanse the body of MRD to improve a patient’s duration of survival and potential for cure.

Systemic Therapy: Precision Cancer Medicines, Chemotherapy & Immunotherapy

Precision cancer medicines, chemotherapy and immunotherapy are all forms of systemic treatment that circulate in the blood and can destroy cancer cells throughout the body. Chemotherapy, once the only systemic therapy is increasingly being replaced by more targeted precision cancer medicines and immunotherapy. The use of precision medicines and immunotherapy is determined by performing NGS-biomarker tests that identify which therapy will be most effective.. Precision cancer medicines and immunotherapies can be used both instead of and in addition to chemotherapy.

Chemotherapy

Chemotherapy is any treatment involving the use of drugs to kill cancer cells. Cancer chemotherapy may consist of single drugs or combinations of drugs, and can be administered through a vein, injected into a body cavity, or delivered orally in the form of a pill. Chemotherapy is not “targeted” to the cancer and acts more generally in the body’s cells which can result in more side effects.

· Starting Chemotherapy? An Overview

Immunotherapy

Immunotherapy is also a standard treatment for cancer and can be used alone or in combination with other systemic therapies. Immunotherapy either works to stimulate your immune system to attach the cancer or remove obstacles that prevent your immune system from working normally.

· Immunotherapy-An Overview

Precision Cancer Medicines

Not all cancer cells are alike. They may differ from one another based on what genes have mutations. Molecular testing can be performed to test for certain genetic mutations or the proteins they produce, and the results can help identify newer precision cancer medicines that target cancer cells with specific genetic mutations.

The purpose of precision cancer medicine is to define the genomic alterations in the cancers DNA that are driving the growth of that specific cancer. Precision cancer medicine utilizes molecular diagnostic testing, including DNA sequencing, to identify cancer-driving abnormalities in a cancer’s genome. Once a genetic abnormality is identified, a specific targeted therapy can be designed to attack a specific mutation or other cancer-related change in the DNA programming of the cancer cells. Precision cancer medicine uses targeted drugs and immunotherapies engineered to directly attack the cancer cells with specific abnormalities, leaving normal cells largely unharmed.

Several precision medicines been approved for the treatment of advanced sarcomas or are being developed in clinical trials. In order to identify which drugs can be used your physician needs to have NGS-biomarker testing performed on the cancer tissue or blood. The following are known targets for available precision cancer medicines. 

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Screening and Prevention of Sarcomas

Information about the prevention of cancer and the science of screening appropriate individuals at high risk of developing cancer is gaining interest. Physicians and individuals alike recognize that the best “treatment” of cancer is preventing its occurrence in the first place or detecting it early when it may be most treatable.

The chance of an individual developing cancer depends on both genetic and non-genetic factors. A genetic factor is an inherited, unchangeable trait, while a non-genetic factor is a variable in a person’s environment, which can often be changed. Non-genetic factors may include diet, exercise, or exposure to other substances present in our surroundings. These non-genetic factors are often referred to as environmental factors.

Heredity or Genetic Factors in Soft Tissue Sarcoma

Most soft tissue sarcomas are sporadic (not hereditary). However, soft tissue sarcomas occur with greater frequency in patients with:1

  • Retinoblastoma
  • von Recklinghausen’s disease (neurofibromatosis)
  • Gardner’s syndrome
  • Werner’s syndrome
  • tuberous sclerosis
  • basal cell nevus syndrome
  • Li-Fraumeni syndrome (p53mutations)

For more information on these specific genetic disorders, there are specific web sites devoted to each disease which include information on the risk of developing a soft tissue sarcoma.

Environmental or Non-Genetic Factors

Radiation therapy for other cancers is associated with an increased incidence of soft tissue sarcomas. Some soft tissue sarcomas (for example, leiomyosarcoma) have been linked to Epstein-Barr virus infection in patients with acquired immune deficiency syndrome.

Other environmental factors may influence soft tissue sarcomas as well, but given the wide variety of histological types of soft tissue sarcoma and the relative rarity of each type, many risk factors may go unrecognized.

Prevention of Soft Tissue Sarcoma

Risk factors for soft tissue sarcoma are still poorly understood, and there are no known preventive strategies.

Sarcoma Bone Newsletter 490

Screening and Early Diagnosis of Soft Tissue Sarcoma

For many types of cancer, progress in the areas of cancer screening and treatment has offered promise for earlier detection and higher cure rates. The term screening refers to the regular use of certain examinations or tests in individuals who do not have any symptoms of a cancer but are at high risk for that cancer. When individuals are at high risk for a type of cancer, this means that they have certain characteristics or exposures, called risk factors that make them more likely to develop that type of cancer than those who do not have these risk factors. 

The risk factors are different for different types of cancer. An awareness of these risk factors is important because 1) some risk factors can be changed (such as smoking or dietary intake), thus decreasing the risk for developing the associated cancer; and 2) persons who are at high risk for developing a cancer can often undergo regular screening measures that are recommended for that cancer type. Researchers continue to study which characteristics or exposures are associated with an increased risk for various cancers, allowing for the use of more effective prevention, early detection and treatment strategies.

There are no current screening techniques or effective preventative measures for soft tissue sarcoma. However, more than 70 percent of soft tissue sarcomas occur in areas of the extremities, trunk, and head and neck that are palpable (can be felt with the hands). Patients and parents should be aware of such painless “growths” and discuss them with their physician as soon as they are noticed. Retroperitoneal soft tissue sarcomas (soft tissue sarcomas that develop in the space behind the abdominal lining) often present with numbness due to nerve compression, swelling of an extremity, or bladder symptoms. Such symptoms should alert parents to seek medical attention at the earliest sign.

Reference

  1. Clark MA, Fisher C, Judson I, et al. Soft-tissue sarcomas in adults. Medical Progress. New England Journal of Medicine 2005;353:701-711.