Desmoid fibroma is a rare type of tumor on connective tissues. It develops from the muscular structure, fascia, tendons, aponeuroses. It is an intermediate option between benign and malignant neoplasm. This fibroma gives metastases, but it has a tendency to aggressive growth and frequent relapses. Because of this, in oncology it is considered as a conditionally benign tumor. It is also called desmoid, musculoaponeurotic fibromatosis.
Characteristics of the neoplasm
Desmoid fibroma is a connective tissue tumor. It is prone to sprouting into the surrounding tissues, but metastases are not isolated. It can appear on any part of the body, but in most cases it is located on the anterior abdominal wall, back or shoulders. It looks like a tumor-like neoplasm that is located in the muscles or is associated with them. In the future, it can grow into bone tissues, joints, blood vessels, internal organs.
Histological analysis of tissue samples shows no signs of malignancy. Desmoid(pictured) is considered an intermediate stage between benign and malignant formation. But at the same time, frequent and multiple relapses after removal are characteristic of the desmoid. The tumor develops aggressively and can spread and crash into nearby organs, tissues, even bones, gradually destroying them.
Desmoma looks like a painless solid mass. Has a round shape. In diameter from 0.2 to 15 cm, although in rare cases the sizes are larger. The surface is smooth, but may have slight bumps.
Inside contains a mass of brown or gray hue with a consistency similar to jelly. Inside, the surface is lined with the epidermis. On the walls there may be bone or cartilaginous tissue, calcification zones. The neoplasm grows slowly, but in some people, on the contrary, rapidly. If the size is large, then it can put pressure on nearby internal organs. Sometimes inflammatory processes begin in the substance inside the growth. There is a high probability of a breakthrough of a purulent mass into adjacent internal tissues or outward.
Desmoid fibroma is formed from the aponeurosis or fascia of the muscles. In any case, it requires shells of connective tissue. Such neoplasms can be located in any zones.
Varieties
The following varieties are distinguished depending on the location:
- Abdominal desmoids. They are also called true. They develop directly on the abdomen. Such growthsoccur in 35% of all cases.
- Extra-abdominal. Grow in other places. Occurs in 65% of cases. As a rule, they are found on the arms, shoulders, buttocks. In more rare cases - on the chest, lower extremities. In women, it can grow behind the uterus, and in men, behind the scrotum.
There are several varieties of extra-abdominal desmoid fibroma (desmoid):
- Classic with a single lesion that affects nearby fasciae.
- Loss of muscle fascia and vessels of the legs or arms with uniform compaction and thickening.
- Multiple nodular neoplasms with different locations.
- Transition to a malignant neoplasm, transformation to desmoid sarcoma.
These are the main types that differ in location, character, appearance.
Causes of desmoid fibroma
Doctors and scientists have not yet succeeded in establishing exactly the causes of such an ailment. But there are a number of factors that, together or individually, increase the likelihood of such a neoplasm.
These include the following:
- Hyperestrogenia. This is confirmed by the fact that regression occurs in women with menopause, as well as with properly selected hormone therapy.
- Ruptures of muscle fibers during labor.
- Genetic predisposition.
- Soft tissue injury.
This disease affects both men and women. But at the same time in the latter it occurs 4 times more often. And of themnulliparous women account for only 6%.
As a rule, women between the ages of 20 and 40 turn to specialists with complaints. But in men, they are usually diagnosed in adolescence. A possible explanation is the active growth of muscle mass or excessive physical activity, which causes microtrauma of the muscle and connective tissue. In children, the disease is extremely rare. In medical practice, cases with a congenital form of desmoid fibroma are known.
Symptoms
Desmoid fibromas of small size do not cause pain, discomfort. Neoplasms are characterized by the following:
- Dense mobile tumor. Located under the skin. Gradually increases. In this case, it is desmoid fibroma that can be suspected due to the fact that it is located in a place that is often subjected to operations or injuries.
- Grows gradually and becomes unmovable.
- If it is on the lower limb, then this leg swells. This happens when the tumor grows through the venous fascia or tightly fuses with the vessel wall. Because of this, the outflow of blood becomes worse. This causes swelling, puffiness.
- Intra-abdominal location, as a result of which there may be signs of damage to internal organs that are located nearby. May grow from the mesentery. When the tumor reaches a large size, it causes displacement or squeezing of the intestine. There are problems with the digestive processes - the stomach grumbles, torments bloating, constipation. In somecases, symptoms of intestinal obstruction appear.
- If a woman is located near the mammary glands, then because of this, her breasts increase. Both her shape and the shape of the nipple can change.
- The tumor can be either mobile or connected to adjacent tissues.
- If a desmoid fibroma grows near the scrotum in a man, then the testicle is displaced and seems to be enlarged.
- If the neoplasm grows to the bones, it can cause their atrophy, fractures.
- If the desmoid is enlarged near the articular joint, it can cause contractures.
- If inflammation begins in the desmoid fibroma, and the purulent mass breaks into nearby tissues and organs, this causes a clinical picture that is characteristic of intoxication. The patient has a fever, chills, weakness. If pus enters the abdominal cavity, then signs of peritoneal irritation may be noticed, which indicate the development of peritonitis.
It is necessary to distinguish a desmoid from a lipoma or hematoma (especially in places where a person often gets injured or wounded).
Diagnosis
Diagnosis includes the following activities:
- Inspection. This applies to desmoid fibromas, which are located outside and are a dense neoplasm. Palpation does not cause pain. In the early stages of development, it is not soldered to tissues and can easily be displaced. If this is a fairly large growth, then it fits snugly into place. It can even grow into the periosteum. The skin over the neoplasm does not change. Often located in areas near injuries or wounds after surgery, as indicated by scarring.
- Tumor ultrasound. Such an examination indicates that the neoplasm does not have a capsule. It grows into the fascia. A vessel may be located. It is a single chamber cavity. Inside it is a substance with the consistency of jelly. On the ultrasound image, it looks like a dark cavity. Sometimes dense structures are located next to it - calcifications or calcifications.
- Biopsy. The procedure is carried out to determine the structure of cellular structures. This also applies to connective tissue fibers intertwined with each other. Cells with mitoses are often found - this is an incorrect division of the structure. The more of them, the more likely the reappearance of the neoplasm or its transition to a malignant tumor (in this case, it is a sarcoma). A biopsy is taken in the area between the desmoid fibroma and he althy tissues. This helps to choose the tactics of the operation and the boundaries of the excised tissue area.
- Magnetic resonance imaging. Thanks to it, you can see various neoplasms even with the smallest sizes, determine its location, the involvement of nearby tissues in the pathological process. This technique is completely safe for human he alth.
- Computed tomography. This is a series of radiographs. Pictures are taken in the form of sections of the studied area. For soft tissues, the information content of the procedure is less, it is excellent for bone structures. Detects the presence of calcifications.
Additionally exercise estradiolserum to determine the need for hormone therapy. An x-ray of the bones in the area where the growth is located should be taken to determine if they are affected. If the neoplasms are located in the pelvis, then cystography and excretory urography are required.
To assess the general condition of the patient and prepare him for surgery, a general examination is carried out, which includes a urine and blood test, an electrocardiogram, a coagulogram.
Treatment
Due to the high likelihood of recurrence, it is recommended to carry out complex treatment of soft tissue desmoid fibroma, which includes surgery and radiation therapy. Additionally, chemical and hormonal treatment can be used. According to statistics, if only monomethodology is used, which includes exclusively surgical intervention, then relapses are recorded in 70% of cases. If complex treatment is carried out, then the risk of recurrence of the tumor is significantly reduced.
Surgical removal
Involves excision of the tumor along the edges of he althy tissue. Quite often, the entire fascia on which the tumor has appeared is removed. This is done to reduce the risk of relapse.
Radiation therapy
After surgical removal, radiation therapy is performed. This is due to the fact that, according to statistics, the tumor grows 30 cm from the main site. Irradiation includes several courses that the patient undergoes after the wound from the surgical intervention has healed.
First treat a wider area. The total dose is 40 Gy. After 3 months, the course is repeated. In this case, only the central area is treated, and the dose will be 2 times less.
Antiestrogen
In addition, antiestrogens are also used. This is due to the increased activity of fibroma growth in women with significant levels of estrogen, as well as the occurrence of regression after menopause.
This became the basis for the use of "Tamoxifen". The course lasts from 5 to 10 months. Additionally, Zoladex is used once a month. It contains substances that are similar to gonadotropin-releasing hormone.
This method of treating abdominal wall desmoid is even allowed to be used as the main one, but only in cases where there are contraindications to surgical intervention.
Gestagens
Hormone therapy also includes the use of progestogens, such as Megestrol, Progesterone. They reduce estrogen levels.
Extra
Chemotherapy is also being done. They use drugs from the group of cytostatics - these are Vinblastine and Methotrexate. The course lasts from 3 to 5 months. Be sure to monitor the biochemical parameters of the blood.
Conclusion
In case of desmoid of the anterior abdominal wall, the treatment prognosis is favorable if the corresponding tissues are removed, into which the fibroma can grow. Over the next 3 years in 60%cases relapse. If several treatments are combined, this can lead to the complete elimination of the tumor.
When wondering what to eat with desmoid fibroma, know to avoid high-calorie and fatty foods.
The diet should contain foods with antioxidant activity. It is also recommended to reduce the amount of food after lunch and in the evening. You need to eat only freshly prepared dishes, without preservatives, dyes and flavors.
Dishes should be prepared using gentle culinary technologies, i.e. boil, stew, bake in the oven. In any case, the attending physician will give specific recommendations on the diet, focusing on the severity of the disease. But the diet will only be an addition to the medical treatment of desmoid fibroma.
