July is Sarcoma and Bone Cancer Awareness Month. In honor of this and all the patients who have fought and are currently fighting these diseases, the Paris Charter Against Cancer would like to contribute to the discussion and present some facts about the disease as well as what you can do to decrease your risk of being diagnosed with Sarcoma or Bone Cancer.
What is Bone Cancer?
Bone cancer refers to malignancies that originate in the bones. These cancers can be primary (originating in the bones) or secondary (metastatic – originating elsewhere and spreading to the bones).
The most common types of Bone Cancers are:
- Osteosarcoma: Typically occurs in bones like the femur of teenagers and young adults. This is the most common type of bone cancer.
- Ewing Sarcoma: Typically found in bones or the soft tissue surrounding the bones, and primarily affects children and young adults
- Chondrosarcoma: Found in the cartilage cells and is more common in adults
- Chordoma: A rare type of cancer that is found in the spine and at the base of the skull.
What is a Sarcoma?
Sarcomas cover a broad category of cancers that originate in the connective tissue. This includes bones and cartilage but also affects the muscles, fat, blood vessels, nerves, and cartilage.
Sarcomas are typically separated into bone sarcomas and soft tissue sarcomas. Bone Sarcomas are cancers that start in the bone, and include osteosarcomas and Ewing sarcoma. Bone cancers are technically a subset of sarcomas i.e. bone sarcomas. Soft tissue sarcomas are cancers that originate in soft tissues. These include liposarcomas (found int the fat cells), leiomyosarcomas found in the smooth muscle cell), Rhabdomyosarcoma (found in the skeletal muscle cells), fibrosarcoma (found in fibrous tissue), angiosarcoma (found in blood or lymph vessels), and synovial sarcoma (found near joints).
Risk Factors
Risk factors for bone cancer include:
Past radiotherapy treatment(s):
Although considered to be a minor risk, previous exposure to high doses of radiation from things such as radiotherapy can potentially cause cancerous changes in bone cells at later stages in life.
Having bone conditions, such as Paget’s disease of the bone:
Paget’s disease of the bone disrupts the bone renewal cycle and can lead to weakness, deformities, and on rare occasions, cancer of the bone. This disease primarily affects people aged 50 or older. Other types of Paget’s disease are also linked to different cancers.
Having Li-Fraumeni Syndrome:
Li-Fraumeni Syndrome presents a change in the TP53 gene. This gene is responsible for creating a protein called tumor protein 53 (P53). This protein is a tumor suppressor and prevents your cells from dividing and growing too rapidly or in an uncontrolled manner, thus preventing the formation of tumors. With the change in the gene due to the syndrome, the P53 protein cells cannot function properly, thus allowing cells to divide uncontrolled and potentially become cancerous. People with this syndrome are more likely to develop a number of cancers, including sarcomas, bone cancers, breast cancer, gliomas, etc., particularly at a young age.
Having a history of other conditions such as retinoblastoma and umbilical hernia:
Studies have found that people who have had a retinoblastoma may be more likely to develop bone cancer, as there is a genetic linkage in possible causes. Retinoblastoma 1 (RB1) is a tumor-suppressing gene that, like the TP53 gene, regulates the cell cycle and suppresses tumor formations. Malfunctions of this gene can lead to the development of retinoblastoma. In regards to the genetic link to bone cancer, studies have shown that in certain instances, patients diagnosed with osteosarcoma, chondrosarcoma or dedifferentiated chondrosarcoma possess an altered RB1 gene as well.
Additionally, research has found that infants born with an umbilical hernia are more prone to developing Ewing sarcoma.
Risk Factors for Sarcoma include:
Chemicals:
Exposure to chemicals such as arsenic, vinyl chloride, herbicides (in particular phenoxyacetic acid), and wood preservatives (particularly those containing chlorophenols)
Radiation:
Exposure to high doses of radiation, either from previous cancer treatments or from other sources
Lymphedema:
In rare cases, having untreated lymphedema (long-term swelling in the arms or legs) can cause soft tissue sarcoma.
Genetic factors:
Several genetic conditions have been linked to increased sarcoma risks. Gardner syndrome, Gorlin syndrome, L-Fraumeni syndrome, neurofibromatosis type 1, von Hipple-Lindau disease, tuberous sclerosis, and Werner syndrome have all been linked to increased risks of developing sarcoma.
Prevention & Screening
Bone Cancer:
Unfortunately, there are currently no known lifestyle-related or environmental causes of bone cancer, and the risk factors associated with this disease are unpreventable (with the exception of radiation exposure), making it very difficult to prevent bone cancer actively.
There are no widely recommended screening tests for bone cancer. However, if you have any of the associated conditions that increase your risk or are experiencing symptoms, speak to a medical professional for advice.
Sarcoma:
Avoiding exposure to known risk factors such as chemicals and radiation is recommended. If you have been diagnosed with any of the linked genetic conditions, speak to your primary care provider about concerns regarding sarcoma and develop a plan to assess your risk. However, sarcomas can still develop without known risk factors, making prevention difficult.
If you present with symptoms or are found to be at higher risk, ultrasounds or biopsies of concerning areas can be taken to check for soft tissue sarcomas, however, presently there are no regular screening tests that can be undertaken as preventative measures.
Statistics
- In 2020, an estimated 34,270 people were diagnosed with Kaposi sarcoma (a form of angiosarcoma that appears on the ski or mucosal surfaces, particularly in the GI tract).
- 73% of those cases were in Africa
- The Global Cancer Observatory ranks Kaposi sarcoma as the 31st most common cancer in the world and the 30th in cancer mortality.
- 86.6% of deaths from Karposi sarcoma in 2020 were in Africa
- Osteosarcoma is more common in males than in females
- The most common ages of diagnosis is between 15-19 in males and 10-14 in females.
- Ewing sarcoma is the second most common malignant bone tumor.
- The average age of diagnosis is between 10 and 15 years, for both males and females, although males are three times more likely to be diagnosed.
Institute for Health Metrics and Evaluation (IHME) Graphs of Death and Incidence Rate of Soft Tissue and Extraosseous Sarcomas as well as Malignant Neoplasm of Bone and Articular Cartilage Cancer 1980-2021
Institute for Health Metrics and Evaluation (IHME) World Data Map showing the Soft Tissue and Other Extraosseous Sarcoma Deaths per 100,000 for both sexes in 2000 vs. 2021
Institute for Health Metrics and Evaluation (IHME) World Data Map showing the Malignant Neoplasm of Bone and Articular Cartilage Deaths per 100,000 for both sexes in 2000 vs. 2021
