What are Desmoid Tumours?
Desmoid tumours are a non-fatal and noncancerous kind of tumour that may be found growing in the connective tissues of the body. Since the arms, abdomen, and legs are areas that have a high concentration of connective tissues, these are the areas where desmoid tumours may most likely develop. Although desmoid tumours may not require immediate intervention, their growth should be monitored to gauge the correct time for medical or surgical intervention.
Nomenclature of the Desmoid Tumour?
The clinical terms for Desmoid tumours are Aggressive Fibromatosis, Deep fibromatosis, Desmoid fibromatosis, Familial infiltrative fibromatosis, Hereditary desmoid disease, or Musculoaponeurotic fibromatosis. The name itself is derived from the Greek word “Desmos”, which translates to tendons.
What are the Dangers Involved?
Desmoid tumours can be of various progressions and some can spread to other tissues of the body, leading to fatal consequences. However, the propensity of the latter is quite rare. Desmoid tumours may be more common in people suffering from a congenital type of colon cancer known as familial adenomatous polyposis (FAP). With such comorbidities, desmoid tumours can be quite serious.
How are Desmoid Tumours Formed?
In general, cancerous cells are formed due to changes in the DNA of certain cells, leading to uncontrolled and unwanted growth of these cells, which can inhibit the functioning of other healthy cells. The exact causes of Desmoid tumour remain a mystery to doctors and scientists to this day. However, scientists have reached certain vague conclusions about the origin of desmoid tumours. These tumours are said to be caused by mutations in adenomatous polyposis coli (APC) genes that result in the unwanted growth of connective tissue cells. According to some other scientists, increased production of oestrogen during pregnancy can also be a catalyst to the formation of desmoid tumours. These tumours are formed either sporadically or are the result of comorbidities like FAP which often leads to Colorectal Cancer.
Symptoms of Desmoid Tumour
Since the area which can be affected by these tumours is vast, symptoms can often be quite erratic and heterogeneous. A visible area of swelling anywhere in the body, particularly in and around the arms, abdomen, and legs can often be an early indication of the formation of a desmoid tumour. If the affected area causes pain, and there is a certain amount of loss of functionality, it may mark the beginning of a desmoid tumour. When a desmoid tumour begins to form in the abdomen, it may cause unusual cramping and feelings of nausea.
How is it Diagnosed?
If desmoid tumours are identified in the early stages, they can be cured. However, the method of diagnosis for this cancerous ailment is complex as it often involves a biopsy.
- Ultrasound: Ultrasound frequency sound waves are used in this process to develop images of the affected area that can often confirm the existence of a desmoid tumour.
- Imaging Techniques: Procedures and diagnostic tests like Magnetic Resonance Imaging (MRI) are often used to see if and how a desmoid tumour may be attached with connective tissue. Computerised Tomography (CT) is also prescribed by most doctors as diagnostic tests to confirm the presence of a desmoid tumour.
- Biopsy: As a last resort, a biopsy may be conducted to confirm the existence of a desmoid tumour. In this diagnostic method, a tiny piece of the tumour is surgically extracted, for inspection of its cell structure and formation. This inspection reveals whether it is a desmoid tumour.
Treatment of Desmoid Tumours
Desmoid tumours are easily curable ailments that can be treated using pharmacological as well as a surgical intervention at the right time. Although the most highly suggested form of treatment for most desmoid tumours is to wait and observe their growth, here are some forms of treatments when the tumour has progressed beyond the non-fatal stages.
- NSAIDs: Non-steroidal anti-inflammatory drugs (NSAIDs) like Imatinib are often used to slow the rate of growth of desmoid tumours. Treatment using these drugs are generally aimed at shrinking the desmoid tumour to more manageable proportions.
- Hormonal Treatment: Hormonal medications that inhibit the production of hormones that may further promote the growth of a desmoid tumour, is also a popular method of treatment. For example, anti-oestrogens and anti-prostaglandins are therapeutically effective in treating desmoid tumours.
- Chemotherapy: This is a harsher form of pharmacological treatment that is used to reduce the size of a tumour. This treatment can be continued until the tumour subsides entirely or is manageable enough to operate on.
- Radiotherapy: This form of treatment can continue in unison with other forms of treatment. However, for desmoid tumours in the abdominal regions, this may not be a safe line of treatment since radiation is said to cause significant damage to certain internal organs.
- Novel molecular-targeted Therapy: This is a drug therapy that uses a newly developed kind of drug known as receptor kinase inhibitors.
Apart from these, there are some new investigational forms of treating desmoid tumours that are still undergoing clinical trials. These are Radio-frequency Ablation, Cryoablation, Angiogenesis inhibitors, and the use of several combinations of chemotherapy agents.
While desmoid tumours are rather rare, they usually occur in people between the ages of 20 to 30 years. Desmoid tumours can also occur during pregnancy and due to repeated physical trauma to a particular part of the body. However, with early identification, effective observation, and timely treatment they can be cured easily.