Different epidemiological studies have revealed various risk factors that can predispose to STSs. Different STSs may have different risk factors along with some common factors that can predispose their development. Following is the list of soft tissue sarcoma risk factors:
- History of exposure to radiation for the treatment of another tumor/cancer has been implicated for the development of STSs, especially fibrosarcoma, undifferentiated pleomorphic sarcoma (UPS), angiosarcoma, malignant peripheral nerve sheath tumor (MPNST), and osteosarcoma. It generally takes around 10 years from the initial radiation treatment for the development of secondary cancer.
- Genetic cancer predisposition syndromes: Some inherited cancer predisposition syndromes (caused by a mutation in certain genes which are generally transferred from one generation to other) have been reported to be associated with the increased incidence rate of STSs. Following are certain examples of such genetic syndromes:
- Neurofibromatosis type 1 or Von Recklinghausen disease (caused by a mutation in the NF1 gene) may elevate the risk of developing malignant peripheral nerve sheath tumors (MPNST) and gastrointestinal stromal tumors (GISTs)
- Gardner syndrome (caused by a mutation in the APC gene) may lead to the increased incidence of the desmoid tumors
- Li-Fraumeni syndrome (caused by a mutation in the TP53 gene) may increase the chances of developing STSs, especially after a prior radiation treatment
- Gorlin syndrome or nevoid basal cell carcinoma syndrome (caused by a mutation in the PTCH1 gene) may increase the chances of developing fibrosarcoma and rhabdomyosarcoma
- Tuberous sclerosis or Bourneville disease (caused by a mutation in the TSC1 or TSC2 gene) may elevate the risk of developing rhabdomyosarcoma
- Werner syndrome or adult progeria (caused by a mutation in the RECQL2 gene) increases the possibility of developing STSs in children
- Primary familial GIST syndrome (caused by a mutation in the KIT or PDGFRA gene) may increase the risk of developing GISTs
Other syndromes, such as Beckwith-Wiedemann syndrome, Costello syndrome, Noonan syndrome, and Carney-Stratakis syndrome have also been implicated in enhancing the risk of developing STSs.
- Occupational exposure: Higher risk of STSs has also been reported in individuals with chronic exposure to certain chemicals like vinyl chloride, arsenic, Agent Orange, chlorophenols, insecticides, herbicides that contain phenoxyacetic acid generally experienced by the workers of plastic, textile, dyestuffs, paint, leather, dry-cleaning, metal, wood, and agriculture industries.
- Weakend immune system: Individuals with a weak immune system that may be due to HIV infection, autoimmune disease, or immunosuppresants in patients who have undergone an organ/hematopoietic stem cell transplant are considered at higher risk of developing certain types of STSs, for example, Kaposi sarcoma (KS).
- Kaposi sarcoma-associated herpesvirus (KSHV) infection: KSHV or Human herpesvirus 8 (HHV8) is responsible for the development of Kaposi sarcoma (KS). KSHV infection is common in some geographical location, like some areas in Africa. However, all individuals with KSHV infection do not develop KS. KSHV infection is more common in individuals infected with HIV. It may lead to suppression of the immune system and KS later in life.
- Risk of developing certain STSs increases in an individual with a history of the same or a different type of STS. Also, certain studies have reported an increased incidence of STSs in individuals with a history of trauma, especially in limbs.
- Age and Gender: Older age individuals, especially males, are generally at increased risk of developing STSs. However, there are certain exceptions, such as rhabdomyosarcoma (RMS) is more common in children.
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