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Desmoid fibroma is a rare benign tumour of connective tissue. The disease occurs in 4-6 people per million each year.
If soft tissue desmoid fibroma requires surgical treatment, it is advisable to go to clinics that specialise in treating sarcomas. Both diseases require a high level of training on the part of the surgeon to avoid the risk of recurrence.
Read this article to learn more about the symptoms and diagnosis of desmoid fibromatosis, treatment options abroad and the top clinics we recommend our patients go to.
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What is a desmoid fibroma?
Desmoid fibroma, also known as desmoid fibromatosis or desmoid fibromatosis, is a benign tumour that forms from connective tissue fibroblasts. It is not cancer, as it does not metastasise. However, large, fast-growing fibromas can be dangerous to the health and life of the patient. Desmoid fibroma remains a serious condition that requires treatment in specialised hospitals.
The disease is most often found in people between the ages of 15 and 60. Women are more likely to have these tumours than men.
Up to 10% of cases of desmoid fibrosarcoma are due to genetic predisposition. The risk group includes people with Gardner syndrome and familial adenomatous polyposis. Mutations of the CTNNB1 and APC genes are found in 89% of patients. People with desmoid fibroma are usually sent for genetic diagnosis to look for previous diseases. Risk factors also include trauma, pregnancy and taking oral contraceptives.
The optimal treatment plan for desmoid fibromas usually requires multidisciplinary teams, including sarcoma surgeons, gastrointestinal surgeons, oncologists and radiation therapists. Such an approach is used in specialised cancer centres. The doctors are experienced in modern surgical techniques and are familiar with advanced protocols for fibromatosis desmoid tumours. If you are looking for such hospitals, please contact MediGlobus by clicking on the button at the top of this website. We will advise you free of charge on treatment abroad and help you select a clinic and make travel arrangements.
Symptoms of desmoid fibroma
Desmoid tumours can occur in any part of the body. They usually form inside the abdomen, on the shoulders, arms and hips. Common sites for tumours are scars from caesarean sections or operations.
In ⅔ of patients, the tumours are painless. Such masses are less dangerous and, unless they continue to grow, do not usually require surgery. They can be detected during a check-up or screening for other diseases.
Symptoms with which patients seek medical attention include:
Pain;
Swelling in the area of the neoplasm;
Insomnia;
Difficulty moving around;
Intestinal obstruction;
Hydronephrosis;
Rarely, intestinal perforation.
How desmoid fibromatosis is diagnosed
ULTRASOUND | Ultrasound is used to determine the size and location of desmoid fibromas, especially those in the anterior abdominal wall, arms and legs. |
CT SCAN | A CT scan is often used during the diagnosis of desmoid fibromas. The technique is particularly useful for intra-abdominal lesions. Tumours arising in the abdomen most commonly involve the mesentery, although other areas may also be affected. A CT scan can provide important information needed to plan surgery, such as the location of neighbouring vessels and organs. Complications such as intussusception, bowel ischaemia and hydronephrosis are also easily detected by CT scan. |
MRI | Due to its high-quality soft tissue images, MRI is excellent for diagnosing desmoid fibromas. It is often used to diagnose tumours in the arm, leg, head, neck, abdomen and chest wall. MRI may also be indicated instead of a CT scan in patients who are allergic to iodinated contrast agents, or in children where less radiation exposure is desirable. |
Biopsy | To check whether the neoplasm is a desmoid tumour, the doctor will perform a biopsy by taking a small amount of tissue with a needle. The pathologist will examine the cells under a microscope to determine what kind of tumour it is. For some hard-to-reach neoplasms, this examination can be problematic. |
Tumour diagnosis abroad
Between 10% and 62% of patients presenting at foreign hospitals are found to have a wrong initial diagnosis. In the case of rare diseases, such as desmoid fibroma, the risk of medical error is higher. You may apply for a qualified consultation with a foreign oncologist through the international medical platform MediGlobus. Leave a request online and we will call you back shortly.
Treatment of desmoid fibromatosis
In the past, surgery was the main treatment for desmoid fibromas. Modern treatment protocols for the disease have changed the approach, and a more conservative approach to therapy is now practised.
With asymptomatic desmoid fibroma, doctors often recommend keeping the tumour under active observation. In 50% of cases, the disease does not progress and patients can avoid surgery.
Surgery
If possible, the surgeon will try to remove the desmoid fibroma during surgery. If it can be excised completely, the disease has a positive prognosis. However, neoplasms often form near important organs, blood vessels or nerves. Surgery in this area can be problematic and dangerous. Often doctors suggest other treatments instead. However, the decision on whether surgery is necessary is made on a case-by-case basis.
The resection of a desmoid fibroma is usually extensive. Surgical treatment of intra-abdominal desmoid fibroma may require extensive surgery. This includes the removal of sections of the bowel, vascular structures and adjacent abdominal organs.
Radiation therapy
Radiation therapy is a method of treating tumours with controlled radiation. State-of-the-art equipment used in foreign clinics allows the rays to be delivered precisely to the tumour without damaging healthy tissue. This reduces the harm caused by the treatment and the number of side effects.
Radiotherapy is used to treat inoperable desmoid fibromas as well as tumours that have relapsed. Treatment is prescribed after surgery in cases where the neoplasm cannot be completely excised. This reduces the risk of recurrence.
Drug therapy
Different groups of drugs are used to treat desmoid fibromatosis:
anti-estrogenic drugs;
non-steroidal anti-inflammatory drugs;
cytotoxic chemotherapy;
tyrosine kinase inhibitors (targeted therapy).
Some of these drugs can cause serious side effects, so they must be taken under the strict supervision of your doctor.
Radiofrequency ablation
Some medical centres use radiofrequency ablation to treat desmoid fibromas. This is a minimally invasive procedure that shrinks tumours using high temperatures.
The prognosis for desmoid fibroma
The prognosis for the treatment of patients in foreign hospitals is optimistic. Depending on the size and location of the tumour, 76% to 99% of patients live more than 5 years.
In 20-30% of cases, desmoid fibroma recurs. Risk factors for recurrence include:
large tumour size;
the young age of the patient;
tumour location in the extremities;
tumours located in the mesentery;
presence of the CTNNB1 S45F genetic mutation;
Desmoid tumours usually recur 4-5 years after surgery. They can be dangerous and are more difficult to treat. The 10-year survival rate for patients with recurrent disease is 63%.
Story of a patient with a desmoid tumour
New Yorker Curt Robinson was diagnosed with a desmoid fibroma at the age of 43. It was a painful mass in the neck area. The pain was so severe that the man could not turn his head or put on a T-shirt by himself. A few months later he went to the doctors, who carried out a biopsy and diagnosed desmoid fibromatosis.
Immediately after confirming the diagnosis Kert was referred to a surgeon. Unfortunately, the tumour was found to be inoperable. Because of the proximity of blood vessels and nerves, surgery would have been too dangerous. However, the doctor advised Kert to take part in a drug trial, which at the time was not yet part of the treatment protocol for desmoid fibroma. Targeted therapy is now available in specialised medical centres to treat this disease. The drug was developed to help patients with kidney cancer, but it began to be used in desmoid when it was found to be 7 times more effective than standard therapy. In Kert’s case, the growth of the tumour was stopped completely for two years.
The man was able to avoid dangerous surgical treatment and return to a normal life. After a course of physiotherapy to restore full mobility to his arm and hand, he is no longer in pain and can sleep peacefully again.
Where can desmoid fibroma be treated abroad?
Summary
Desmoid fibroma is a rare and dangerous type of benign tumour. Patients with a mild form of this disease do not require serious intervention. However, if the tumour grows and causes problems – pain, swelling, insomnia, digestive problems – it will require systematic treatment.
Desmoid fibromas are treated in cancer centres by doctors who specialise in sarcomas. Therapy options include surgery, radiation therapy, chemotherapy, targeted therapy, hormone therapy and drug therapy.
The average five-year survival rate for desmoid fibroma is 76-99%. With recurrence, which a third of patients are at risk of, the treatment prognosis is 63%.
MediGlobus specialists recommend the following clinics for the treatment of desmoid fibromas: Liv, Medicana and Koç clinics in Turkey, Israeli Ichilov and Assuta centres, Cologne University Hospital in Germany and Navarra University Hospital in Spain, and the Vienna Private Clinic in Austria.
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Sources:
- 1. National Cancer Institute Center for Cancer Research
- 2. Journal of Computer Assisted Tomography: Current Update on Desmoid Fibromatosis
- 3. National Organisation for Rare Disorders
- 4. The Liddy Shriver Sarcoma Initiative
