Left, right, left, right - Ryan’s feet methodically found firm ground as he ran the last 15 kilometres of his marathon. His breathing had slowed down, and he was in the zone. Well, some heart rate zone that is.
Ten kilometres to go - he felt his heart rate rising as it got harder to breathe. Five kilometres to go - he was almost at the finish, when a sharp shooting pain in his leg forced him to take notice. He had this lump for quite a few years now and the pain was getting worse every day.
After a litany of tests and chamber visits, doctors confirmed his worst fears. His lump was malignant. It’s a rare cancer called fibrosarcoma.
If you develop fibrosarcoma, you have a soft tissue tumour in the tendons, bones, or ligaments. The most common sign is a new lump. There are two varieties of fibrosarcoma - infantile (congenital) fibrosarcoma and adult-type fibrosarcoma.
A rare soft tissue cancer or sarcoma, fibrosarcoma occurs when a soft tissue tumour develops in the connecting tissue that connects parts of your body. Such tissues also include ligaments and tendons.
Occasionally, fibrosarcoma forms inside bones or in fibrous connective tissue covering bones. It usually forms in soft tissue deep inside the leg, especially the shin or thigh bone, upper arm, trunk, or knees. Sometimes, it can start in the head or neck.
Usually, there are two varieties of fibrosarcoma, which are dramatically different.
Infantile (congenital) fibrosarcoma—This variety usually occurs after birth or a little bit after. It grows fast but doesn’t spread as much and is usually curable.
Adult-type fibrosarcoma - This type is usually found in adults between 20 and 60. However, it can also impact older children and adolescents. Unlike the infantile variety, adult-type fibrosarcoma is usually more aggressive and more challenging to treat.
Fibrosarcoma isn’t very common in adults and children—the adult variety accounts for around 10% of soft tissue sarcoma diagnoses. Infantile fibrosarcoma remains one of the most common sarcomas in children under five years of age, but it is quite rare overall. It impacts less than 5 out of 1 million infants.
Fibrosarcoma symptoms usually don’t surface quickly. Because it develops in soft tissues, you might not notice any physical changes until the tumour grows and presses on a blood vessel or nerve.
Usually, symptoms for fibrosarcoma are :
A soft lump in the arms, legs or trunk that is painless to the touch.
Tingling or “pins and needles” feeling, or sharp, burning and aching pain.
Strange swelling (Because the tumour is pressing against blood vessels)
Fibrosarcoma symptoms can be like those of other less severe conditions. Only a doctor can determine whether the changes are because of fibrosarcoma or something more benign, common and less-harmful.
What Are the Causes of Fibrosarcoma?
Researchers have not been able to pinpoint precisely what causes fibrosarcoma; however, genetic mutation and cell changes most likely play a part.
Most fibrosarcomas have the same alterations in their cell DNA. Such changes can cause cells to multiply rapidly and form cancerous tumours.
Medical experts have found a common mutation in infantile fibrosarcoma. Around 90% involve issues with the NTRK gene family (there are three NTRK genes). If these genes don’t work as they should, tumours can form.
Researchers have found some inherited conditions that might boost your risk of developing fibrosarcoma. They are :
Familial adenomatous polyposis.
Li-Fraumeni syndrome.
Neurofibromatosis type 1
Nevoid basal cell carcinoma syndrome.
Retinoblastoma
Tuberous sclerosis
Werner syndrome.
Other conditions that are associated with fibrosarcoma include :
Bone infarction (paucity of blood flow causes bone cells to wither away)
Chronic (long-term) osteomyelitis.
Fibrous dysplasia
Paget’s disease of the bone
Risk factors which are linked to the environment and the previous medical history :
Previous radiation therapy aimed towards the area that has the tumour.
Previous severe burn at the tumour site.
Exposure to chemicals like arsenic, vinyl chloride or thorium dioxide.
Exposure to particular metals in orthopedic implants like nickel, chromium or cobalt.
Diagnosis of Fibrosarcoma
Healthcare providers perform a litany of tests when diagnosing fibrosarcoma. They will use what they pick up to establish a stage and grade for your fibrosarcoma. The tests are
Magnetic resonance imaging (MRI): It is the most common imaging test for diagnosing fibrosarcomas. It can determine the location and size of the tumour and whether it impacts blood vessels or nerves.
Computed tomography scan (CT scan): This test uses X-rays and a computer to craft 3D images of your bones and soft tissues.
Biopsy: A doctor might pick out soft tissue from the lump (core needle biopsy), or they might decide to cut it out entirely (excisional biopsy). A pathologist will then scan this tissue for cancer cells in a lab.
Immunohistochemistry (IHC): This lab test uses proteins named antibodies to find out whether the tumour is a fibrosarcoma or might be some other kind of soft tissue tumour.
Treating fibrosarcoma isn’t easy. It involves surgery to get rid of the cancer. Other treatments for this ailment are radiation therapy, therapy with medicines and ablation therapy.
Medications that help treat fibrosarcoma include targeted therapy, immunotherapy, and chemotherapy. Your course of treatment will depend on the kind of sarcoma you have.
It might also depend on where the cancer is in the body, its rate of growth, and whether it has metastasised.
Surgery
Surgery aims to get rid of all the cancer cells. Sometimes, surgeons need to remove a limb, like an arm or a leg, to get all the cancer. This is called amputation. However, surgeons also try to save the limb whenever possible.
On occasion, surgeons might not remove all the cancer without impacting nearby nerves or organs. They work to remove as much of the fibrosarcoma as possible.
Radiation therapy
Radiation therapy depends on powerful energy beams to treat cancer. This energy could come from X-rays, other sources or protons. For sarcoma, you may have radiation therapy before, during or post surgery to get rid of the cancer.
Chemotherapy
Chemotherapy battles cancer using powerful medicines. Certain types of sarcoma might respond more to chemotherapy than others. Doctors might use chemotherapy before or post surgery. It can also help in the treatment of sarcoma that grows unchecked or spreads to other body parts.
Targeted Therapy
Targeted therapy for cancer is a treatment which implements medicines to attack particular chemicals in cancer cells. By blocking such chemicals, targeted therapy can cause cancer cells to die.
Immunotherapy
Immunotherapy for cancer is a treatment method with medicines that assists the body’s immune system in killing cancer cells. The immune system fights off diseases by attacking germs and other cells that should not be in the body. Cancer cells survive because they hide from the immune system. Immunotherapy assists the immune system in finding and terminating cancer cells.
Ablation Therapy
This is a procedure which applies treatment straight to the cancer cells to harm them. Certain types of ablation apply energy to the cancer cells, causing them to heat up .
Procedures which do this include radiofrequency ablation and microwave ablation. Depending on extreme cold to terminate cancer cells is known as cryoablation.
Sometimes, ablation involves using alcohol to harm the cancer cells. Ablation might be used as a treatment method when surgery isn’t feasible. It could also be used when cancer metastasises.
So, you have been diagnosed with fibrosarcoma. What are you going to do apart from embarking on a regime of self-care? Here’s what you should do :
Figure out your care plan - As your doctor, what can you expect regarding recovery and follow-up appointments? Being in the loop about what comes next can ensure your focus is on what you can control, not what you can’t.
Deal with the anxiety - Regular checkups and tests post treatment are vital in case the cancer recurs. This can, however, cause anxiety as well. Speak to a therapist, who might be able to help you manage anxiety.
Seek help—You’ll probably need assistance while going through treatment. Your loved ones will probably be anxious to do what they can. Let them know how they could help.
Concentrate on wellness - Eat right, exercise enough and sleep enough to beat back cancer.
Rest - Cancer and treatment can leave you feeling drained. Rest as much as you can.
Combat stress- Cancer is stressful. Meditation, relaxation techniques and deep breathing might help with the stress that comes with cancer and its treatment.
In conclusion, a fibrosarcoma diagnosis can be disconcerting and challenging, it is vital to remember that there are options for treatment, and with early detection and the right care, you could well be on your road to recovery.
Understanding the condition, asking for professional advice and following a structured treatment plan can make a huge difference in your journey. Together with medical treatments, prioritising self-care, combating stress and relying on a supportive network can help you navigate this difficult time.
Take each step as it comes, be informed and concentrate on your wellness. With the right mindset and support, you can face these challenges with strength and resilience.
What is fibrosarcoma?
Fibrosarcoma is a rare type of cancer that originates in the fibrous connective tissue, often in the soft tissues such as muscles, tendons, or ligaments. It can also occur in bones, though it's less common. The tumor consists of malignant fibroblasts that can grow and spread to other parts of the body.
What are the symptoms of fibrosarcoma?
Symptoms of fibrosarcoma can vary depending on the tumor's location. Common signs include:
A painless lump or swelling in the affected area
Pain or tenderness near the tumor site, especially if the tumor presses against nerves or other structures
Restricted movement or discomfort in the affected area
In cases of metastatic spread, symptoms may include unexplained weight loss, fatigue, or other generalized symptoms.
What causes fibrosarcoma?
The exact cause of fibrosarcoma is not known. However, some factors may increase the risk, such as:
Genetic mutations
Previous radiation therapy (as a result of a previous cancer treatment)
Certain inherited conditions like Li-Fraumeni syndrome
Chronic inflammation or injury, though these are not definitive causes
How is fibrosarcoma diagnosed?
Diagnosing fibrosarcoma typically involves:
Physical examination by a healthcare provider
Imaging tests such as X-rays, MRI, or CT scans to locate the tumor and determine its size and spread
A biopsy, where a sample of tissue from the tumor is examined under a microscope for signs of cancer
Blood tests may be performed to assess overall health, though they are not used to diagnose fibrosarcoma directly.
What are the treatment options for fibrosarcoma?
Treatment for fibrosarcoma generally depends on the tumor's size, location, and whether it has spread. Common treatments include:
Surgery to remove the tumor and surrounding tissue
Radiation therapy to target and destroy remaining cancer cells
Chemotherapy may be used if the cancer has spread or is not removable through surgery
Targeted therapy or immunotherapy may also be options for advanced cases.
