Soft-tissue sarcoma

The only reliable way to determine whether a soft-tissue tumor is benign or malignant is through a biopsy. The two methods for acquisition of tumor tissue for cytopathological analysis are:

• Needle aspiration biopsy, via needle
• Surgically, via an incision made into the tumor

A pathologist examines the tissue under a microscope. The pathologist may be the most important person in the treatment of sacomas, because they are responsible for making the proper diagnosis. Pathologists at expert sarcoma centers are invaluable in identifying the type of sarcoma responsible for a patient's symptoms. If cancer is present, the pathologist can usually determine the type of cancer and its grade. Here, grade refers to a scale used to represent concisely the predicted growth rate of the tumor and its tendency to spread, and this is determined by the degree to which the cancer cells appear abnormal when examined under a microscope. Low-grade sarcomas, although cancerous, are defined as those that are less likely to metastasise. High-grade sarcomas are defined as those more likely to spread to other parts of the body. For soft-tissue sarcoma, the two histological grading systems are the National Cancer Institute system and the French Federation of Cancer Centers Sarcoma Group system.^[9][10][11]

Soft-tissue sarcomas commonly originate in the upper body, in the shoulder or upper chest. Some symptoms are uneven posture, pain in the trapezius muscle, and cervical inflexibility [difficulty in turning the head].^[12] The most common site to which soft-tissue sarcoma spreads is the lungs.^[13]

Treatment

In general, treatment for soft-tissue sarcomas depends on the stage of the cancer. The stage of the sarcoma is based on the size and grade of the tumor, and whether the cancer has spread to the lymph nodes or other parts of the body (metastasized). Treatment options for soft-tissue sarcomas include

• Surgery is the most common treatment for soft-tissue sarcomas, and usually the only way to achieve a cure. The tumor is removed leaving a safe margin of surrounding healthy tissue to decrease the chances of its recurrence.
• Radiation therapy may be used as a neoadjuvant before surgery to shrink tumors, or as an adjuvant after surgery to kill any cancer cells that may have been left behind. In some cases, it can be used to treat tumor that cannot be surgically removed.
• Chemotherapy may be used with radiation therapy either before or after surgery to try to shrink the tumor or kill any remaining cancer cells. There is evidence to suggest that doxorubicin chemotherapy as an adjuvant can reduce recurrence at the original site or elsewhere in the body.^[14] Evidence also suggests chemotherapy can increase the length of time patients live, but this is less certain evidence. The use of chemotherapy to prevent the spread of soft-tissue sarcomas has not been proven to be effective. If the cancer has spread to other areas of the body, chemotherapy may be used to shrink tumors and reduce the pain and discomfort they cause, but is unlikely to eradicate the disease. A combination of docetaxel and gemcitabine could be an effective chemotherapy regimen in patients with advanced soft-tissue sarcoma.^[15][16]
• Outcomes are better for patients who are seen (and ideally treated) at expert sarcoma centers.^[17] In the United States, these are generally found in NCI-designated cancer centers.[1]

Research

Soft-tissue sarcoma research requires significant effort due to its rarity; successful research requires substantial collaboration. Year by year, the medical field is learning that the various types cannot be lumped together and each sarcoma needs to be considered a different type of cancer.^[18]

As a novel form of treatment used in other cancers, immunotherapy may have an role in treating soft-tissue sarcomas like alveolar soft part sarcoma and pleomorphic undifferentiated sarcoma. However, as of 2023, only alveolar soft part sarcoma has a regulatory approval for such an agent, in this case atezolizumab.^[19]

Example of sarcoma immunology research: the Immunological Constant of Rejection

When the

ICR signature is independently associated with postoperative MFS in early-stage STS, independently from other prognostic features, including CINSARC. A robust prognostic clinicogenomic model integrating ICR, CINSARC, and pathological type, and suggested differential vulnerability of each prognostic group to different systemic therapies.[20]

Epidemiology

Soft-tissue sarcomas are very uncommon cancers. They account for less than 1% of all new cancer cases each year.^[21]

In 2023, about 14,300 new cases were diagnosed in the United States.^[21] Soft-tissue sarcomas are more commonly found in older patients (>50 years old), although in children and adolescents under age 20, certain histologies are common (rhabdomyosarcoma, synovial sarcoma).^[13]

Around 3,300 people were diagnosed with soft-tissue sarcoma in the UK in 2011.^[22]

Notable cases

• Actor Robert Urich died from synovial sarcoma.^[23]
• Actress Michelle Thomas died from desmoplastic small-round-cell tumor, a rare abdominal soft-tissue sarcoma.^[24]
• It Is Written evangelist Henry Feyerabend died from sarcoma in his leg.^[25]
• Video game concept artist Adam Adamowicz died from complications of a rare muscle sarcoma on February 9, 2012, at age 42.^{[citation needed]}
• Professional wrestler Jake Roberts revealed he has muscle cancer.^{[citation needed]}
• Professional wrestler
  Zack Ryder revealed he had synovial sarcoma as a teenager.^[26]
• India's ex-finance minister Arun Jaitley died from sarcoma on August 24, 2019.^[27]
• Writer Rachel Caine died from the disease on November 1, 2020.^[28]
• YouTuber Technoblade died from the disease in June, 2022.^[29]

See also

• Soft tissue sarcoma in cats and dogs

References