Sarcoma
Illustration photo
Sarcoma is cancer of connective and supporting tissues of the body and can occur in any location and organ. Soft tissue sarcomas constitute about 90 percent of all sarcomas, of which GISTS (tumors originating from the connective tissue of the gastrointestinal tract) is the largest histological subgroup. The rest are bone sarcomas.
A total of 570 people were diagnosed with sarcoma in Norway in 2023, including abdominal and retroperitoneal soft tissue sarcomas and gynaecological soft tissue sarcomas.
The diagnosis of sarcoma is based on tissue or cell samples. Histological diagnosis of sarcoma is difficult and requires specialist expertise. All tissue samples suspected of sarcoma must be primary or re-examined at one of the university hospitals. Immunohistochemical and molecular examinations will also often be necessary. It is necessary to distinguish different tumour types from each other and to be able to adapt the treatment for the patients.
Risk factors
There are some factors that can increase the risk of soft tissue cancer.
- Chronic lymphedema (kreftforeningen.no) may be congenital or may occur after radiation therapy or removal of lymph nodes. Also, some benign connective tissue tumors can become malignant.
- High-dose radiation therapy. Soft tissue sarcoma can occur in places where radiation therapy has previously been given to another form of cancer, but this is very rare. As a rule, this type of sarcoma takes at least five to ten years to develop after radiation therapy. However, the risk is very small due to improvements in radiation therapy.
- Viruses are the cause of Kaposi's sarcoma – a rare form of soft tissue sarcoma in blood vessels, skin or mucous membranes. The cause is a special type of herpes virus called HHV-8. This can occur in older people and by weakened immune systems, for example in HIV-infected or organ transplant recipients.
- Immunosuppressive medicine after transplants, which is supposed to prevent the new organ from being rejected, causes the immune system to be unable to repair cell damage, increasing the risk of soft tissue sarcomas.
- Some chemicals are suspected of developing some forms of soft tissue sarcomas: vinyl chloride (PVC), certain types of herbicides (phenoxy acids) and dioxin.
In general, soft tissue sarcomas are very unlikely to be hereditary. Heredity has been demonstrated in connection with Li-Fraumeni syndrome, (sml.snl.no) where a congenital defect in a gene called P53 is central.
Source: helsenorge.no
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New cases
570 people were diagnosed with sarcoma in Norway in 2023.
Proportion of sarcomas in 2023 and 2021-2023 by location. From Fig. 2.3 in Annual Report Quality Register for Sarcoma 2023 (Norwegian only).
Most patients with sarcoma have abdominal and retroperitoneal sarcomas and sarcoma of the soft tissues of the extremities and trunk.
Aging
Number of bone and soft tissue sarcomas in 2023 by age. From Fig. 2.4 in Annual Report Quality Register for Sarcoma 2023 (Norwegian only).
As expected, the majority of patients with soft tissue sarcoma are adults, and the number increases in each age group up to 80 years.
We see an indication that the number of bone sarcomas is greater in children and young adults and at 61-80 years than at 41-60 years. This is as expected from the literature. Note that the figure only shows the number of patients and that age-specific incidence rates cannot be deduced from the figure. Since the number of patients with cancer in children and young adults is low, for example, the age-specific incidence rates for bone sarcomas will be considerably higher in these groups than in patients from 61 to 80 years, even though the absolute number is similar.
Survival
5-year relative survival of sarcoma in Norway is at a good level compared to results reported internationally.
The survival rate for patients with soft tissue sarcoma is higher than for patients with bone sarcoma. Both bone sarcomas and soft tissue sarcomas have many subgroups and survival varies within the groups.
5-year relative survival for bone sarcomas by regional health authority (catchment area), 2014-2023. Fromfig. 2.21 in the Annual Report Quality Register for Sarcoma 2023 (Norwegian only).
The prognosis for patients with bone sarcoma is related to several factors, such as histological subtype, degree of malignancy and metastatic status at diagnosis. The figure includes all subgroups and is thus an unselected cohort. We see that 5-year relative survival is 67 percent. There are some differences between the regions, but this is presumably due to small numbers and natural variation. The result is comparable to other European countries. A similar study from the Netherlands has shown that the 5-year survival rate for all patients with bone sarcoma is 58.9 percent.
5-year relative survival for soft tissue sarcomas in extremities and trunks, distributed by catchment area, 2014-2023. From Fig. 2.36 of the Annual Report Quality Register for Sarcoma 2023 (Norwegian only).
The figure shows 5-year relative survival for soft tissue sarcomas in the extremities and trunk. All patients are included in the analysis, regardless of tumour size, location, degree of malignancy or metastatic status.
5-year relative survival for soft tissue sarcomas of the extremities and trunk. All patients are included in the analysis, regardless of tumor size, location, degree of malignancy or metastatic status. The figure shows that 5-year relative survival is 74%, which is the same level as reported internationally.
5-year relative survival in Helse Vest seems to be higher than in the rest of the country. We have done supplementary analyzes to look more closely at details related to histological subtypes and metastatic status. The difference seems mainly to be about patients with localized disease, and we see differences only for certain subtypes. Whether it may be related to more patients in Helse Vest
received additional treatment with chemotherapy is unclear.
5-year relative survival for abdominal and retroperitoneal soft tissue sarcomas (not GIST), distributed by regional health authority, 2014-2023. From fig. 2.50 in the Annual report Quality register for sarcoma 2023. (Norwegian only).
The figure includes all patients, not just those treated with a curative aim. There is no significant regional differences in survival for these patients. 5-year relative survival is 59%.
Development over time
Incidence of sarcoma cases in the period 1993-2023, divided by bone, soft tissue and gender. From fig. 2.1 in Annual report Quality register for sarkoma 2023.
In the first part of the time period, there was an increase in the incidence rate for soft tissue sarcomas in both women and men. This is due to an increase in the incidence rate of GIST (see annual report 2021 for details). The incidence rate for bone sarcoma has been stable throughout the period.
Quality registry for sarcoma
The quality registry for sarcoma contains detailed data on patients in Norway with sarcoma. The registry started recording clinical information for patients with a diagnosis from 01.01.2018. The Quality Registry began collecting clinical information in January 2019.
The registry collects data on assessment and treatment of this patient group. The purpose is to use the data from the registry to illustrate practice in hospitals, which can be of help in assessing practice in individual hospitals and for the patient group as a whole.
Examples of results from the Sarcoma Registry's annual report 2023
Steps you can take to reduce your risk of getting sarcoma
General advice is:
- Be physically active
- Have a healthy diet
- Be smoke-free
Pay special attention if a bump
- Growing fast
- are larger than five cm in diameter
- sitting deep (in the muscles or deeper)
- Comes again after being removed
Source: helsenorge.no
More about cancer prevention on kreftregisteret.no
Questions about cancer
The Cancer Registry of Norway is a research institution. Our professionals therefore do not answer questions about diagnosis, assessment, treatment and follow-up from patients or their relatives.
Questions about this should be directed to your own GP, treating institution or the Cancer Society Advisory Service tel: 21 49 49 21
Useful Links
Facts about soft tissue sarcoma - kreftforeningen.no
Facts about bone sarcoma - kreftforeningen.no
Support association for all affected by sarcoma
Sarcoma - National action programme with guidelines for diagnosis, treatment and follow-up