Last updated date: 29-May-2023
Originally Written in English
Symptoms, Diagnosis & Treatment of Desmoids Tumors
Desmoid tumors, also known as aggressive fibromatosis, are a rare type of non-cancerous growth that develops from fibrous tissue in the body. These tumors can occur in any part of the body, but are most commonly found in the abdomen, shoulders, arms, and legs. Desmoid tumors are considered locally aggressive, meaning they can grow and invade nearby tissues, but they do not spread to other parts of the body.
Desmoid tumors can occur sporadically or can be associated with familial adenomatous polyposis (FAP), an inherited condition that causes multiple polyps to form in the colon and rectum. Women are more commonly affected than men, and the tumors most frequently occur in young adults.
Desmoid tumors can be difficult to manage and treat due to their unpredictable growth patterns and tendency to recur after treatment. Treatment options may include surgery, radiation therapy, and medication, but the optimal approach varies depending on the location, size, and extent of the tumor, as well as the patient's overall health and individual circumstances.
How common are Desmoid Tumors?
Desmoid tumors are rare, accounting for less than 3% of all soft tissue tumors. They are most commonly diagnosed in adults aged 20-40 years, and they are slightly more common in women than in men.
How do Desmoid Tumors Affect My Body?
Desmoid tumors are locally aggressive tumors that do not metastasize or spread to other parts of the body. They can develop in any part of the body, but they are most commonly found in the abdominal wall, shoulder, thigh, and chest. As they grow, they can put pressure on nearby organs and tissues, causing pain, discomfort, and limited mobility. Desmoid tumors can also interfere with normal bodily functions, depending on their location. For example, tumors that develop in the abdomen can cause bowel obstruction, while those that develop in the chest can affect breathing.
Desmoid Tumors Vs Soft Tissue Sarcoma
Desmoid tumors and soft tissue sarcomas are both types of tumors that can arise in the soft tissues of the body. However, they have some differences in terms of their behavior and treatment.
Desmoid tumors, also known as aggressive fibromatosis, are rare tumors that arise from fibrous tissue. They are typically slow-growing, but can be locally invasive and aggressive. Unlike sarcomas, desmoid tumors do not spread to distant parts of the body, so they are not considered to be metastatic tumors.
Soft tissue sarcomas, on the other hand, are a group of malignant tumors that can arise from a variety of soft tissues, including muscle, fat, and connective tissue. They have the ability to spread to other parts of the body, and can be life-threatening if not treated promptly.
Both desmoid tumors and soft tissue sarcomas can be difficult to treat and require a multidisciplinary approach. Treatment for both types of tumors may include surgery, radiation therapy, and/or chemotherapy.
Who do Desmoid Tumors Affect?
Desmoid tumors can affect people of all ages and genders, but they are more common in young adults between the ages of 15 and 60 years. Women are slightly more likely to develop desmoid tumors than men. People with certain inherited conditions, such as familial adenomatous polyposis (FAP) and Gardner syndrome, have a higher risk of developing desmoid tumors. Additionally, prior radiation therapy to the area can also increase the risk of developing desmoid tumors.
What causes Desmoid Tumors?
The exact cause of desmoid tumors is not known. However, they are believed to be related to mutations in the APC gene, which is responsible for producing a protein that suppresses tumor growth. In some cases, desmoid tumors may also occur in individuals with a family history of the condition, or as a result of previous trauma or surgery. Hormonal factors may also play a role, as desmoid tumors are more common in women and may be influenced by estrogen levels.
What are Desmoid Tumor Symptoms?
The symptoms of desmoid tumors can vary depending on their location and size. In some cases, desmoid tumors may not cause any symptoms, while in others they may cause:
- A painless lump or mass
- Limited mobility or stiffness in the affected area
- Nausea, vomiting, and constipation (if the tumor is in the abdomen)
- Breathing difficulties (if the tumor is in the chest)
- Difficulty urinating or blood in urine (if the tumor is in the pelvis)
- Pain or numbness (if the tumor is near nerves)
these symptoms can also be caused by other medical conditions, so it is important to consult a healthcare provider for proper evaluation and diagnosis.
How are Desmoid Tumors Diagnosed?
Desmoid tumors can be difficult to diagnose since they are rare and can mimic other conditions. The diagnosis of desmoid tumors typically involves a combination of the following:
- Physical exam: A doctor may perform a physical examination to check for lumps or areas of pain or discomfort.
- Imaging tests: Imaging tests such as X-rays, computed tomography (CT) scans, magnetic resonance imaging (MRI) scans, and ultrasounds can help identify the location and size of the tumor.
- Biopsy: A biopsy involves taking a small sample of tissue from the tumor and examining it under a microscope to check for the presence of cancerous cells.
- Genetic testing: In some cases, genetic testing may be done to look for mutations that are associated with desmoid tumors.
It is important to work closely with a healthcare provider who is experienced in diagnosing and treating desmoid tumors.
How are Desmoid Tumors Treated?
The treatment of desmoid tumors depends on various factors such as the size and location of the tumor, symptoms, and the extent of spread. The treatment options for desmoid tumors include:
- Watchful waiting: In cases where the tumor is small and not causing any symptoms, doctors may recommend monitoring the tumor with periodic imaging tests.
- Surgery: Surgery is the most common treatment for desmoid tumors. The goal of surgery is to remove the entire tumor while preserving as much healthy tissue as possible. In some cases, the surgery may be followed by radiation therapy to reduce the risk of recurrence.
- Radiation therapy: Radiation therapy may be used alone or in combination with surgery to treat desmoid tumors.
- Chemotherapy: Chemotherapy drugs may be used to shrink the tumor or slow its growth. This is usually reserved for cases where the tumor is large, aggressive, or has spread to other parts of the body.
- Targeted therapy: Targeted therapy drugs are designed to target specific molecules or proteins involved in the growth and spread of cancer cells. These drugs may be used in combination with other treatments to treat desmoid tumors.
- Clinical trials: Clinical trials are research studies that evaluate new treatments for desmoid tumors. Participation in a clinical trial may be an option for some patients.
The choice of treatment depends on the individual patient and the extent of the disease. A multidisciplinary team of doctors including surgeons, oncologists, and radiation oncologists may be involved in the treatment of desmoid tumors.
Complications Regarding Desmoid Tumor Treatment
There are potential complications that may arise from the treatment of desmoid tumors, which can vary depending on the type of treatment used. Some examples of potential complications include:
- Surgery: As with any surgical procedure, there is a risk of bleeding, infection, and damage to surrounding structures. In some cases, surgery may also result in scarring or changes in appearance.
- Radiation therapy: Radiation therapy may cause skin irritation or changes in skin color, fatigue, nausea, and damage to nearby organs.
- Chemotherapy: Chemotherapy can cause nausea, vomiting, fatigue, and hair loss. It can also increase the risk of infection and cause low blood cell counts.
- Targeted therapy: Targeted therapy drugs can cause side effects such as fatigue, nausea, diarrhea, and changes in blood pressure.
- Watchful waiting: If a desmoid tumor is being monitored rather than treated, there is a risk that it could grow or spread.
It's important to discuss the potential risks and benefits of any treatment option with your healthcare provider to make an informed decision about your care.
Can I Prevent Desmoid Tumors?
Unfortunately, there are no known ways to prevent desmoid tumors from developing as the exact cause is unknown. However, early detection and prompt treatment may prevent the tumor from growing and causing complications.
What Can I Expect If I Have A Desmoid Tumor?
The prognosis and outcome of a desmoid tumor depend on various factors, such as the size, location, stage, and aggressiveness of the tumor, as well as the age and overall health of the patient.
Desmoid tumors are locally invasive, but they do not metastasize or spread to other parts of the body. However, they can cause compression or displacement of adjacent organs, nerves, and blood vessels, leading to pain, dysfunction, or even life-threatening complications.
The treatment and management of a desmoid tumor may involve surgery, radiation therapy, chemotherapy, hormone therapy, targeted therapy, or a combination of these modalities. The success of the treatment depends on the response of the tumor and the ability to remove or control the tumor without causing significant morbidity or mortality.
Patients with desmoid tumors require close monitoring and follow-up by their healthcare team to detect any recurrence, progression, or complications. They may also need supportive care, such as pain management, physical therapy, or psychosocial support, to improve their quality of life.
Can Desmoid Tumors be Fatal?
Desmoid tumors are generally not considered life-threatening. However, their growth and location can lead to significant morbidity and complications. For example, desmoid tumors that grow in the abdominal cavity can cause bowel obstruction or compression of nearby organs. In rare cases, if the tumor grows and infiltrates vital structures, such as blood vessels or nerves, it can become life-threatening. Additionally, the treatment of desmoid tumors can sometimes lead to complications or side effects that can be serious. Overall, the prognosis and outcomes for desmoid tumors can vary widely depending on various factors, such as the location and size of the tumor, the extent of surgical resection, and the response to treatment.
What is the Desmoid Tumor Life Expectancy?
The life expectancy for individuals with desmoid tumors depends on various factors such as the location, size, and aggressiveness of the tumor, as well as the age and overall health of the patient. It is important to note that desmoid tumors are generally considered to be a low-grade type of tumor and do not typically spread to other parts of the body, although they can be locally aggressive and cause significant complications. The treatment approach and response to treatment can also play a significant role in determining life expectancy. It is recommended to discuss individual prognosis and life expectancy with a healthcare provider who is familiar with the patient's specific case
When Should I See My Healthcare Provider?
You should see your healthcare provider if you experience any persistent or unusual symptoms, such as unexplained pain, swelling, or a lump in any part of your body. Additionally, if you have a known history of desmoid tumors or other similar conditions, it is important to undergo regular medical check-ups and screenings to monitor for any changes or recurrence of the tumors.
What Questions Should I Ask My Healthcare Provider?
If you have been diagnosed with a desmoid tumor, it's important to ask your healthcare provider any questions you have to fully understand the condition and the treatment plan. Some questions you may want to consider asking include:
- What type of desmoid tumor do I have?
- What stage is my desmoid tumor?
- What are my treatment options, and which one do you recommend?
- What are the potential side effects of treatment?
- How long will treatment last?
- Will I need surgery, and if so, what can I expect during and after the procedure?
- What is the likelihood that the tumor will come back after treatment?
- Are there any clinical trials or experimental treatments available for my condition?
- How will my quality of life be affected during and after treatment?
- What support resources are available to me, such as support groups or counseling services?
It's important to ask any questions you have to fully understand your condition and feel empowered to make informed decisions about your treatment.
Defferential Diagnosis of Desmoids Tumors
Some of the differential diagnoses of desmoid tumors include:
- Fibrosarcoma: A type of soft tissue sarcoma that grows from fibrous connective tissue.
- Liposarcoma: A type of cancer that develops in the fat cells.
- Neurofibroma: A benign tumor that arises from nerve cells.
- Gastrointestinal stromal tumor (GIST): A type of tumor that usually develops in the stomach or small intestine.
- Leiomyoma: A benign tumor that develops from smooth muscle cells.
- Schwannoma: A benign tumor that arises from the cells that cover the nerves.
- Lymphoma: A type of blood cancer that develops in the lymphatic system.
- Metastatic cancer: Cancer that has spread to other parts of the body from a primary tumor site.
It's important to note that proper diagnosis of a desmoid tumor requires a thorough evaluation by a healthcare provider, including imaging tests and biopsies.
In conclusion, desmoid tumors are rare, non-cancerous growths that can occur in various parts of the body. They can cause discomfort and interfere with the function of nearby organs or tissues. While the exact cause of desmoid tumors is unknown, they can occur in individuals with a history of trauma, surgery, or pregnancy. The diagnosis of desmoid tumors may involve imaging tests such as CT scans, MRIs, and ultrasounds, as well as biopsies. Treatment options for desmoid tumors may include watchful waiting, surgery, radiation therapy, or medication. The choice of treatment depends on the size and location of the tumor, as well as the overall health of the patient. It is important to follow up regularly with a healthcare provider for monitoring and management of desmoid tumors.