Summary about Disease
Desmoid tumors, also known as aggressive fibromatosis, are rare, benign (non-cancerous) tumors that arise from fibrous connective tissue. They can occur anywhere in the body, but are most common in the abdomen, arms, and legs. While not cancerous in the sense that they don't metastasize (spread to distant sites), they can be locally aggressive, infiltrating surrounding tissues, and causing pain, swelling, and functional limitations. They do not spread to other parts of the body like cancer.
Symptoms
Symptoms vary depending on the location and size of the tumor. Common symptoms include:
A palpable mass or lump
Pain or tenderness
Swelling
Restricted range of motion
In some cases, if the tumor is compressing nerves or blood vessels, numbness, tingling, or weakness.
Abdominal desmoid tumors may cause abdominal pain, bowel obstruction, or bleeding.
Causes
The exact cause of desmoid tumors is not fully understood. However, the following factors are associated with an increased risk:
Genetics: Some cases are linked to familial adenomatous polyposis (FAP), a genetic condition that increases the risk of colon cancer. Mutations in the APC gene are often implicated.
Hormones: Estrogen may play a role, as desmoid tumors are more common in women, particularly during or after pregnancy.
Trauma: In some cases, prior surgery or trauma to the area where the tumor develops has been reported.
Sporadic: Many cases occur without any identifiable risk factors.
Medicine Used
Treatment for desmoid tumors is highly individualized and depends on the tumor's size, location, growth rate, and the patient's symptoms. Treatment options include:
Active Surveillance: For slow-growing or asymptomatic tumors, a "watch and wait" approach may be appropriate, with regular monitoring to assess for any changes.
Surgery: Surgical removal of the tumor may be an option, but complete resection can be challenging due to the tumor's infiltrative nature.
Radiation Therapy: Radiation may be used to control tumor growth or shrink the tumor, especially when surgery is not feasible or after surgery to prevent recurrence.
Systemic Therapies: Several medications may be used to shrink or control desmoid tumors, including:
Hormonal therapies: Tamoxifen or toremifene (anti-estrogens).
Nonsteroidal anti-inflammatory drugs (NSAIDs): Sulindac.
Chemotherapy: Doxorubicin, vinblastine, methotrexate.
Tyrosine kinase inhibitors: Sorafenib, pazopanib.
Is Communicable
No, desmoid tumors are not communicable. They are not caused by an infection and cannot be spread from person to person.
Precautions
There are no specific precautions to prevent desmoid tumors, as the exact cause is largely unknown. Individuals with FAP should undergo regular screening for desmoid tumors.
How long does an outbreak last?
Desmoid tumors are not an "outbreak" situation. They are individual tumors that can grow over time. The growth rate is variable, and some may remain stable for years, while others grow more rapidly. The duration of symptoms depends on the tumor's size, location, and growth rate, as well as the effectiveness of any treatment.
How is it diagnosed?
Diagnosis typically involves:
Physical Examination: A doctor will feel for any lumps or masses.
Imaging Studies: MRI (magnetic resonance imaging) is the most common imaging technique used to visualize the tumor, assess its size and location, and evaluate its relationship to surrounding structures. CT scans may also be used.
Biopsy: A biopsy is essential to confirm the diagnosis. A small sample of tissue is removed from the tumor and examined under a microscope by a pathologist. This helps to differentiate desmoid tumors from other types of tumors.
Timeline of Symptoms
The timeline of symptoms can vary greatly.
Initial Stage: Often, the first sign is a painless lump or swelling. This may be present for some time before it is noticed.
Progression: As the tumor grows, symptoms may develop, such as pain, tenderness, restricted range of motion, or, depending on location, abdominal discomfort or neurological symptoms. The speed of progression varies greatly between individuals.
Treatment: Symptom resolution or control depends on the chosen treatment and the tumor's response to that treatment.
Important Considerations
Multidisciplinary Approach: Management of desmoid tumors often requires a multidisciplinary team, including surgeons, medical oncologists, radiation oncologists, radiologists, and pathologists.
Individualized Treatment: Treatment should be tailored to the individual patient, considering the tumor's characteristics, the patient's symptoms, and their overall health.
Recurrence: Desmoid tumors have a high rate of local recurrence even after complete surgical removal. Therefore, close follow-up is essential.
Quality of Life: Given the potential for pain, functional limitations, and the need for ongoing treatment, quality of life considerations are important in the management of desmoid tumors.
Research: Due to the rarity of desmoid tumors, ongoing research is crucial to better understand their causes, improve treatment options, and ultimately find a cure.