At Saint John’s Cancer Institute and Health Center, soft tissue sarcoma cases are reviewed by a multi-disciplinary tumor board to determine the most effective, individualized treatment plans.

What are Soft Tissue Sarcomas?

Soft tissue sarcomas can occur anywhere in the body - Saint John's Cancer Institute
Soft tissue sarcomas can occur anywhere in the body – Saint John’s Cancer Institute

Unlike other tumors, soft tissue sarcomas vary in appearances and location. They are a rare group of tumors that arise from connective and supportive tissues in the body, which may include fat, muscle, nerves, bone, cartilage, vessels, or fibrous structures. There are more than 50 known types of soft tissue sarcomas. Soft tissue sarcomas are frequently identified through touch and sensation, and rarely spread to the lymph nodes, in contrast to melanoma.

Treatment for soft tissue sarcomas is dependent on specific subtypes, location in the body, size, and grade (see grade chart below). Surgery remains the standard treatment for primary sarcoma tumors, where the surgeon can calculate how much tissue around the tumor must be removed to reduce future recurrence. Additional treatments (multi-modality/multi-disciplinary) may be recommended to improve surgical outcomes.

A multidisciplinary approach is where sarcoma outcomes are reaching their fullest potential.

Some Soft Tissue Sarcomas include:

    angiosarcoma
    Angiosarcoma is one of the many types of soft tissue sarcomas.
  • Angiosarcoma
  • Dermatofibrosarcoma protuberans
  • Epithelioid sarcoma
  • Gastrointestinal stromal tumor (GIST)
  • Kaposi’s sarcoma
  • Leiomyosarcoma
  • Liposarcoma
  • Malignant peripheral nerve sheath tumor
  • Myxofibrosarcoma
  • Rhabdomyosarcoma
  • Solitary fibrous tumor
  • Synovial sarcoma
  • Undifferentiated (pleomorphic sarcoma)

For a complete list, click here: What Is a Soft Tissue Sarcoma? (cancer.org)

Symptoms

soft tissue sarcoma embeded in leg muscle
Sarcoma tumors can form in and around muscle and connective tissues

Soft Tissue Sarcomas often spread via the bloodstream and may occur anywhere in the body. Compression from the tumor may affect circulation, movement, and nerves, which can cause pain and discomfort, however, rarely does this result in fever and/or weight loss. The location of the sarcoma can mask its presence, which also affects the kinds of symptoms experienced. However, many sarcomas do not produce symptoms.

Risk Factors

Risk can be measured in several ways; genetic predisposition (family occurrence), having Li Fraumeni syndrome, neurofibromatosis (NF) type 1, Familial Adenomatous Polyposis (FAP), Gardner Syndrome, and retinoblastoma. Other factors include exposure to radiation therapy or chemotherapy, chemical carcinogens, and lymphedema.

Diagnosis & Treatment

A sarcoma diagnosis is best determined by biopsy, and imaging when necessary. Our Melanoma multidisciplinary board includes the fields of surgical oncology, radiation oncology, medical oncology, genetic specialist, and pathology. The tumor board determines a diagnosis based on the biopsy and imaging, if any were advised, to formulate a personalized treatment plan. Chemotherapy (medical treatment), immunotherapy, radiation, and target therapies may be used to treat soft tissue carcinoma. Targeted therapy, which are also medical treatments, are designed to attack specific genes or proteins that inhibit tumor growth.

Radiation therapy can be used with surgery, treating the surrounding tissue of the sarcoma.

Each of these treatments can be used alone or in combination with one another, such as surgery and radiation. Where cancer has the potential to metastasize, or has already spread to other parts of the body, chemotherapy drugs work to treat the entire body, inhibiting cancer cell growth beyond the tumor area. Immunotherapy can stimulate or suppress the immune system, creating the right conditions for the body or other treatments to have an improved response. Some cancers are immune resistant, where some cancer cells exhibit an ability to evade treatment (see Translational Immunotherapy). Immunotherapies are continuously evaluated through ongoing clinical trials, which provide greater understanding and options to develop effective treatment plans.

Radiation is typically prescribed for larger, higher-grade tumors, because it helps to reduce the size of the tumor before and after surgical removal. It can also be used to reduce cancer spread beyond the treatment area. We utilize advanced 3D imaging and radiation technologies to identify, plan, and treat soft tissue sarcomas. See additional treatment information below.

Staging

Stage

Stage Grouping

Description (Trunk and Extremities)

IA

· T1
· N0
· M0
· G1 or GX
The cancer is 5 cm (2 inches) or smaller (T1).
It has not spread to nearby lymph nodes (N0) or to distant sites (M0).
The cancer is grade 1 (G1) or the grade cannot be assessed (GX).

IB

· T2, T3, T4
· N0
· M0
· G1 or GX
 The cancer is:
  • Larger than 5 cm but not more than 10 cm (T2) OR
  • Larger than 10cm but not more than 15 cm (T3) OR
  • Larger than 15 cm (T4).

It has not spread to nearby lymph nodes (N0) or to distant sites (M0). The cancer is grade 1 (G1) or the grade cannot be assessed (GX).

II

· T1
· N0
· M0
· G2 or G3
The cancer is 5 cm (2 inches) or smaller (T1).

It has not spread to nearby lymph nodes (N0) or to distant sites (M0).
The cancer is grade 2 (G2) or grade 3 (G3).

IIIA

· T2
· N0
· M0
· G2 or G3
The cancer is larger than 5 cm (2 inches) but not more than 10 cm (T2).

It has not spread to nearby lymph nodes (N0) or to distant sites (M0). The cancer is grade 2 (G2) or grade 3 (G3).

IIIB

· T3 or T4
· N0
· M0
· G2 or G3
The cancer is:
  • Larger than 10 cm but not more than 15 cm (T3) OR
  • Larger than 15 cm (T4).

It has not spread to nearby lymph nodes (N0) or to distant sites (M0). The cancer is grade 2 (G2) or grade 3 (G3).

IV

· Any T
· N1
· M0
· Any G
The cancer is any size (Any T) AND it has spread to nearby lymph nodes (N1).
It has not spread to distant sites (M0). It can be any grade.
OR
· Any T
· Any N
· M1
· Any G
The cancer is any size (Any T) AND it has spread to nearby lymph nodes (N1).
It has spread to distant sites such as the lungs (M1). It can be any grade.

Treatments

Personalized treatments for soft tissue sarcoma differ based on histological subtypes, location, size, and grade, which are determined in the diagnosis through our multidisciplinary approach. One or more treatments may be utilized to achieve the best clinical outcome.

preparing for surgery - sarcoma treatment
Sarcoma treatment options are discussed during your consultation.

Surgery

Removal of cancerous tissues, gland, or affected organ

  • The main treatment options of most sarcomas
  • Addresses the treatment area and a small portion of the surrounding area
  • May be a final treatment for some tumors
  • May involve removal of lymph nodes near the primary tumor
  • Can be utilized with other types of treatment

Radiation

Focused beams of energy that disrupt tumor growth by damaging the DNA of the cancer

  • Can be given before or after surgery
  • Typically used in larger, higher-grade tumors
  • Often used to treat an area where the cancer is likely to recure
  • Can be utilized with other types of treatment
mRNA expression for cancer recurrence
Saint John’s Cancer Institute actively researches risk for cancer recurrence, adjusting medical treatment as appropriate.

Chemotherapy

Drug treatment that addresses cancers that have or can spread to other areas

  • Works to stop or slow cancer cell growth
  • Used effectively for some subtypes of sarcoma
  • Can be given orally, intravenously, or in other ways
  • Doses are carefully selected to target the specific type and stage of the cancer
  • Can be utilized with other types of treatment

Targeting Therapy (Precision Medicine)

Drug treatments that seek very particular proteins or enzymes within cancer cells

  • Inhibits cancer cell replication and growth
  • Targets specific genes and proteins
  • Can mark cancer cells so that they can be destroyed by the immune system
  • Can prevent new blood vessels from forming that can boost tumor growth
  • Can be utilized with other types of treatment
immunotherapy
A multidiscinplinary approach may include immunotherapy, improving outcomes for patients

Immunotherapy

Helps to fight cancers by helping the body defend itself

  • Effective against many kinds of cancers
  • Supports conditions where cancer has spread throughout the body
  • Blocks proteins that prevent an effective immune response
  • Given intravenously over a period of weeks
  • Can be utilized with other types of treatment

If you have questions regarding soft tissue sarcomas or would like an expert opinion, please call today or click here to schedule an appointment.