Is Sarcoma Cancer Deadly?

Sarcoma is a general term for cancerous lumps in the soft tissue, such as muscles, fatty tissue or nerves, etc. There are many different kinds of sarcomas, some quite deadly and others less so. Overall, however, sarcomas are rare compared with other forms of cancer.

Sarcoma is a type of cancer that occurs when the cells in the soft tissues of the body grow out of control. Soft tissue sarcomas can develop in soft tissues such as fat, muscle, nerves, fibrous tissues, blood vessels, or deep skin tissues. They can occur in any part of the body. 

There are many types of sarcomas, and not all of them are cancerous, however, when the word sarcoma is part of the name of a disease, it means the tumor is cancerous (malignant). 

There are more than 50 different types of soft tissue sarcomas. Sarcomas are overall not common and some are very rare. Some can be very deadly. 

The most common types of sarcoma in adults are:

• Undifferentiated pleomorphic sarcoma (previously called malignant fibrous histiocytoma)
• Liposarcoma
• Leiomyosarcoma

Symptoms of sarcomas include:

• A lump that may or may not cause pain
  • About half of soft tissue sarcomas start in an arm or leg
  • About 4 in 10 sarcomas start in the abdomen
  • Rarely, sarcomas start in the chest, head, or neck
  • Lump grows over time (weeks to months)
• If a sarcoma occurs in the back of the abdomen (the retroperitoneum), symptoms may include blockage or bleeding of the stomach or bowels 

See a doctor right away if you have any of the following symptoms:

• A new lump or a lump that's growing
• Worsening abdominal pain 
• Blood in the stool or vomit
• Black, tarry stools 

Sarcoma cancers are believed to be caused by genetic changes (mutations), however, the exact cause for these mutations is unknown. 

Risk factors for developing sarcomas include: 

• Radiation therapy used to treat other cancers
• Family cancer syndromes 
  • Neurofibromatosis
  • Gardner syndrome
  • Li-Fraumeni syndrome 
  • Retinoblastoma 
  • Werner syndrome
  • Gorlin syndrome 
  • Tuberous sclerosis
• Damaged lymph system
• Chemical exposure 
  • Vinyl chloride (a chemical used in making plastics) 
  • Arsenic 
  • Dioxin 
  • Herbicides (weed killers) containing phenoxyacetic acid at high doses (such as might occur in people who work on farms, though there is no evidence that herbicides or insecticides cause sarcomas in levels encountered by the general public)

In addition to a patient history and physical exam, tests used to diagnose sarcomas include:

• Imaging tests
  • X-ray
  • Computed tomography (CT) scan
  • Magnetic resonance imaging (MRI) scan
  • Ultrasound
  • Positron emission tomography (PET) scan
• Biopsy

Treatments for sarcoma cancer can include one or more of the following:

• Surgery 
  • The goal of surgery is to remove the entire tumor along with at least 1 to 2 cm (less than an inch) of the normal tissue around it (wide margins)
  • Amputation
  • Limb-sparing surgery
  • Lymph node dissection
  • Chemotherapy (chemo), radiation, or both may be given before surgery (neoadjuvant treatment) or after surgery (adjuvant treatment)
• Radiation therapy 
  • External beam radiation
    • Most often used to treat sarcomas
    • Intensity modulated radiation therapy (IMRT) is used
  • Intraoperative radiation therapy (IORT)
    • One large dose of radiation given in the operating room after the tumor is removed but before the wound is closed
  • Brachytherapy (internal radiation therapy
    • Places small pellets (or seeds) of radioactive material in or near the cancer
  • Proton beam radiation (not widely available)
• Chemotherapy 
  • Ifosfamide (Ifex) 
    • The drug mesna is also given to protect the bladder from the toxic effects of ifosfamide 
  • Doxorubicin (Adriamycin)
  • Dacarbazine (DTIC)
  • Epirubicin
  • Temozolomide (Temodar)
  • Docetaxel (Taxotere)
  • Gemcitabine (Gemzar)
  • Vinorelbine (Navelbine)
  • Trabectedin (Yondelis)
  • Eribulin (Halaven)
  • Combinations: 
    • MAID (mesna, doxorubicin, ifosfamide, and dacarbazine) 
    • AIM (doxorubicin, ifosfamide, and mesna)
• Targeted drug therapy 
  • Pazopanib (Votrient)
  • Tazemetostat (Tazverik)
  • Regorafenib (Stivarga)
  • Sorafenib (Nexavar)
  • Sunitinib (Sutent)
  • Larotrectinib (Vitrakvi) (for tumors with an NTRK gene change)
  • Entrectinib (Rozlytrek) (for tumors with an NTRK gene change)

Life expectancy for sarcoma cancers is often expressed in 5-year survival rates, that is, how many people will be alive 5 years after diagnosis. 

Sarcoma cancer 5-year survival rates:

• Localized (cancer is limited to the part of the body where it started): 81%
• Regional (cancer has spread to nearby structures or to nearby lymph nodes): 57%
• Distant (cancer has spread to distant parts of the body such as the lungs): 16%