Who Is At Risk For Myxofibrosarcoma?

Myxofibrosarcoma (MFS) is one type of a soft tissue sarcoma (STS), like in other soft tissue sarcomas the neoplasm arises from connective tissues that surround the muscles and separate the muscles from the skin. It has both a fibroblastic and myxoid origin and therefore, sometimes classified as a myxoid variant type of the malignant fibrous histiocytoma.(1)

Who Is At Risk For Myxofibrosarcoma?

Older Age: Myxofibrosarcoma is common in elderly patients in the age group of 50-70 years with a mean age of 66 years and it is also common in males than in females. With age, there is an accumulation of DNA mutations in the genes and these give rise to alterations in the normal cell process which leads to the formation of cancer cells.(2)

Exposure To Radiation. It is observed that most of the soft tissue sarcomas (STS’) are seen in body areas which have been exposed to radiation earlier for treatment of other cancers or conditions. It takes about an average of 10 years for the diagnosis of STS after radiation exposure. This depends on the amount of radiation the person was exposed and higher the radiation level more risk of soft tissue sarcoma (STS)in those areas. The main sources of radiation exposure are radiotherapy, radiological investigations, natural radiation (radon), and radioactive iodine isotopes. Radiation exposure can increase the risk of getting Myxofibrosarcoma as well. Even chemotherapy is believed to increase the risk of soft tissue sarcoma (STS).(2)

Family Cancer Syndromes. Family cancer syndromes are gene mutations that are inherited and some of these syndromes might increase the risk of getting soft tissue sarcomas including Myxofibrosarcoma.(4)

Li-Fraumeni Syndrome. Li-Fraumeni syndrome is an inherited defect in the TP53 gene; these people have an increased risk of getting histiosarcoma, leiomyosarcomas, liposarcomas, and rhabdomyosarcomas. However, only 10-20 from 100 people with Li-Fraumeni syndrome will develop soft tissue sarcoma (STS).. If people with this familial syndrome are exposed to radiation the risk of developing soft tissue sarcoma (STS). increased as these people are very sensitive to radiation.(4)

Viruses

Human immunodeficiency virus (HIV). There is sufficient evidence to prove that HIV increases the risk of soft tissue sarcoma (STS)..

Kaposi sarcoma herpes virus/ human herpes virus 8 (KSHV/HH8). Kaposi sarcoma develops only in patients with KSHV/HH8 but not all patients with the infection develop Kaposi sarcoma. There is sufficient evidence to prove that Kaposi sarcoma increases the risk of soft tissue sarcoma (STS)..

Chemicals. A meta-analysis has shown that workers in the industry of polychlorophenols which is found in some of the herbicides, disinfectants and in pesticides are not at increased risk of developing STS. But morphology of STS was not taken into account in these studies therefore, there can be some association. Some of the workers from the industry of 2,3,7,8-Tetrachlorodibenzodioxin (TCDD) which is used in industries such as agriculture, metal and waste recycling, pesticide, and pulp manufacture were diagnosed with soft tissue sarcoma (STS). and this can be due to TCDD.(3)

Some of the chemicals like vinyl chloride and arsenic increase the risk of liver sarcoma but there isn’t any conclusive evidence is present that these chemical increases the risk of soft tissue sarcoma (STS)..

Conclusion

The exact cause of Myxofibrosarcoma is unknown however there are several possible risk factors identified. Older age is one of the main risk factors as most of Myxofibrosarcomas occur in elderly people. This might be due to the accumulation of abnormal cells over time due to DNA mutations. Exposure to radiation is another risk factor for soft tissue sarcoma (STS and it can be a risk factor for Myxofibrosarcoma too. The risk increased with higher amount of radiation exposure. People with previous cancer treatment with radiotherapy or who have undergone radiological investigations (high exposure) are at increased risk. People with the familial cancer syndrome Li-Fraumeni syndrome, HIV and/or KSHV/HH8, exposed to chemicals such as polychlorophenols and tetrachlorodibenzodioxin are at increased risk of developing soft tissue sarcoma (STS). These can also increase the risk of developing Myxofibrosarcoma as it is one of the types of soft tissue sarcoma (STS)..

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