Introduction:
Tumors are abnormal growths of tissues and can be found in any part of the body. It can be cancerous or noncancerous. Desmoid tumors are noncancerous and present in the connective tissues. Most commonly, they are found in the abdomen, legs, and arms. They are not like fatty tumors. Fatty tumors will grow under the skin and are called lipomas, but desmoid tumors grow in the connective tissues that form the ligaments and tendons. "Desmoid" derives from the Greek word "Desmos," meaning tendon or band-like. There are various terms for this tumor, as follows:
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Aggressive fibromatosis.
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Desmoid fibromatosis.
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Deep fibromatosis.
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Hereditary desmoid disease.
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Familial infiltrative fibromatosis.
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Musculoaponeurotic fibromatosis.
Desmoid tumors can be slow-growing and do not require immediate treatment, or they can grow quickly and require surgery, radiation therapy, chemotherapy, or other drugs for treatment.
Desmoid tumors are not classified as cancers as they do not spread to the adjacent or other body parts. But they can act more like cancers as they are aggressive and may invade the adjacent tissues or organs sometimes.
What Is a Desmoid Tumor?
Rare, benign (noncancerous) tumors called desmoid tumors form in the network of connective tissue, which supports and connects your bones, ligaments, muscles, and organs. Desmoid tumors can occur anywhere in the body, but they usually affect the arms, legs, or abdomen.
In contrast to malignant tumors, desmoid tumors do not metastasize. The majority of desmoid tumors do not create problems and develop slowly. However, some develop quickly and could eventually get large enough to press against surrounding organs, nerves, or tissue. Medical professionals can treat desmoid tumors, although they frequently reoccur.
What Are the Types of Desmoid Tumors?
According to their location, medical professionals classify desmoid tumors. Types consist of:
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Abdominal Wall Desmoid Tumors: Abdominal wall desmoid tumors harm the tissue surrounding your belly's organs, including the stomach in the upper abdomen.
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Intra-abdominal Desmoid Tumors: These tumors grow in the tissue surrounding the intestines, called the mesentery.
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Extra-Abdominal Desmoid Tumors: Tumors called extra-abdominal desmoid tumors occur outside of the belly and abdominal wall. Extra-abdominal desmoid tumors can impact the head and neck in addition to commonly developing in the shoulders, upper arms, chest wall, and upper legs.
What Are Its Salient Features?
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Frequency: Reports show that desmoid tumors account for nearly 0.03 percent of all tumors. The prevalence can be even 13 percent in patients with a history of familial polyposis of the colon.
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Sex: These tumors are more commonly known to occur in women post-childbirth. The frequency is twice in females when compared to males. In children, the incidence related to gender is equal.
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Age: These tumors can occur in all age groups but are more frequently seen involving individuals aged ten to 40 years.
What Are the Symptoms?
The symptoms of desmoid tumors depend on the involved site. Desmoid tumors most often happen in the abdomen, arms, and legs, and they can be found anywhere in the body. The most commonly observed symptoms are as follows:
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Swelling or presence of any mass.
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Pain, especially in the affected area.
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There can be a loss of function in the affected area.
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Cramping and nausea can be experienced by patients having desmoid tumors in the abdomen.
What Are the Causes?
The cause of desmoid tumors is not yet clear. The explained mechanism is that these tumors may form because of alteration in the DNA of connective tissue.
Are There Any Risk Factors?
Yes, certain factors may increase the risk of getting desmoid tumors, and they are as follows:
1. Younger adults in their 20s and 30s are more likely to develop desmoid tumors. In elderly and pediatric patients, this tumor is uncommon.
2. Genetic syndromes causing colon polyps, like in people having familial adenomatous polyposis (FAP).
3. Individuals who have had recent injury or surgery.
4. Having high levels of estrogen.
5. Being assigned as female at birth (AFAB).
6. Pregnancy: The abdominal wall expands during pregnancy, and estrogen levels rise. This is where most desmoid tumors associated with pregnancy originate.
7.Trauma: According to research, there is an increased risk of acquiring a desmoid tumor if a person has had a significant injury or if they have been hurt in the same area of their body repeatedly.
How Can It Be Diagnosed?
When you visit your physician, specific questions will be asked to know about your symptoms and medical history, and later, the following examinations will be done to come to a diagnosis and stage the disease along with planning the treatment:
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Physical examination.
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Ultrasound of the abdomen can be performed to assess the tissues in that area.
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Imaging tests. Certain imaging tests, like CT (computed tomography) and MRI (magnetic resonance imaging), will be recommended.
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A biopsy, wherein a part of the affected tissue is removed or the affected tissue is completely removed, will be done to come to a diagnosis. The sample can be collected using a needle or surgically, and this may depend on the location, type, and extent of the lesion.
How Can It Be Treated?
There are various treatment options available, and they are as follows:
1) Wait and Watch Method: Tumor growth can be monitored initially. If the lesion or tumor is asymptomatic, you can go ahead with the wait-and-watch method with regular assessment using imaging techniques. Certain tumors can shrink independently, but others may require medical or surgical management.
2) Surgery: This option is followed in patients who have signs and symptoms. The tumor can be completely removed or partially removed. Partial removal is done in cases where the tumor has extended to adjacent structures involving important structures like nerves, etc. Surgery can be used along with radiotherapy or chemotherapy in such cases.
3) Radiation Therapy: Certain radiation beams can help shrink the tumor tissues. It is usually used as an adjunct to surgery.
4) Chemotherapy: Certain strong medications can kill tumor cells. Again, this can sometimes be used as an adjunct to surgery or radiation therapies.
Certain other drugs have proven to be beneficial in managing symptoms of desmoid tumors, including anti-inflammatory drugs, targeted therapies, and hormone therapies.
5) Hormonal Therapy: Drugs like Tamoxifen, which prevent the hormone estrogen from entering cells, appear to affect certain desmoid cancers.
6) Non-steroidal Anti-inflammatory Drugs (NSAIDs): These over-the-counter and prescription medications have the potential to reduce pain and swelling while gradually shrinking a tumor. Our physicians may prescribe them in addition to or instead of other treatments.
7) Ablation: To eradicate the tissue of smaller tumors, our doctors may suggest radiofrequency ablation, which involves intense heat, or cryoablation, which involves intense cold.
8) Targeted Therapy: More recent, licensed medications like Sorafenib and Pazopanib target the cellular routes that desmoid tumors utilize to increase, potentially saving surgery. Clinical trials have revealed even better efficacy from emerging medications, which could soon be accessible.
How Is the Prognosis?
The prognosis is good and has high rates. If the diagnosis is made in earlier stages, the prognosis is excellent. The desmoid tumor has the potential to cause local infiltration, and this may influence the prognosis as if it involves certain vital structures or organs, it may be challenging to treat it completely, or it may take more time for recovery. Reports have also shown that people who have familial adenomatous polyposis and suffer from intra-abdominal desmoid tumors may be fatal for them.
Desmoid tumors are noncancerous tumors that may affect any part of the body. If you develop any mass in your body or find any abnormal symptoms, you should consult your physician soon. Early diagnosis and treatment will improve the prognosis. Online medical platforms are playing a crucial role, especially in this pandemic, in allowing you to consult a specialist or physician at the ease of your home. Hence, consult a physician or specialist online for more details about desmoid tumors.
What Are the Survival Rates of Desmoid Tumors?
Tumor size, location, and response to treatment are among the variables affecting survival rates. The American Society of Clinical Oncology (ASCO) reports that five years after diagnosis, almost 98 percent of patients with desmoid tumors are still alive.
Conclusion
A desmoid tumor is a benign development that originates in the soft tissues. These tumors can be detected throughout the body, but seldom in more than one location at a time. Desmoid tumors do not metastasize to other parts of the body. Some desmoid tumors do not or only grow slowly, causing no issues. In rare cases, they may diminish or disappear on their own. Desmoid tumors might grow more quickly. They can cause nerve, blood vessels, muscle, tendon damage, and gut blockage. Although these alterations are rarely fatal, they might impact one's quality of life. Desmoid tumors can be treated, although they frequently return.
