Desmoid fibroma is a rare type of tumor on the connective tissue. It develops from muscle structure, fascia, tendons, aponeuroses. It is an intermediate option between benign and malignant neoplasms. 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, muscle-aponeurotic fibromatosis.
Neoplasm characteristics
Desmoid fibroma is a connective tissue tumor. It is prone to sprouting into surrounding tissues, but metastases are not secreted. It can appear on any part of the body, but in most cases it is on the front abdominal wall, back or shoulders. It looks like a tumor-like neoplasm, which is located in the muscles or associated with them. In the future, it can grow into bone tissue, joints, blood vessels, internal organs.
Histological analysis of tissue samples does not show signs of malignancy. Desmoid (pictured) is considered an intermediate stage between a benign and malignant formation. But at the same time, frequent and repeated relapses after removal are characteristic of the desmoid. The tumor aggressively develops and can spread and crash into adjacent organs, tissues, even bones, gradually destroying them.
Desmoma looks like a painless, dense mass. It has a round shape. In diameter from 0.2 to 15 cm, although in rare cases, sizes are larger. The surface is smooth, but can be with small tubercles.
Inside contains a mass of brown or gray shade with a jelly-like consistency. Inside the surface is lined with epidermis. On the walls there may be bone or cartilage tissues, calcification zones. The neoplasm grows slowly, but in some people, on the contrary, rapidly. If the dimensions are large, then it can put pressure on adjacent internal organs. Sometimes in the substance inside the growth, inflammatory processes begin. There is a high probability of a breakthrough of purulent mass in adjacent internal tissues or out.
Desmoid fibroma is formed from aponeurosis or muscle fascia. In any case, she needs connective tissue sheaths. Such neoplasms can be 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 stomach. Such growths are found in 35% of all cases.
- Extra-abdominal. Grow in other places. Found 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):
- Classical with a single focus that strikes nearby fascias.
- The defeat of the 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 into a desmoid sarcoma.
These are the main types that differ in location, character, appearance.
Causes of Desmoid Fibroma
It has not yet been possible to establish precisely the causes of such an illness among doctors and scientists. But there are a number of factors that together or separately increase the likelihood of such a neoplasm.
These include the following:
- Hyperestrogenism. This is confirmed by the fact that there is a regression in women with menopause, as well as when conducting correctly selected hormonal therapy.
- Ruptures of muscle fibers during labor.
- Genetic predisposition.
- Soft tissue injuries.
This disease affects both men and women. But at the same time, it occurs 4 times more often in the latter. Moreover, of them nulliparous women make up only 6%.
As a rule, women from 20 to 40 years old complain to specialists. But in men they are usually diagnosed in adolescence. A possible explanation is the active growth of muscle mass or excessive physical exertion that cause microtrauma of 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
Small-sized desmoid fibromas do not cause pain, discomfort. For tumors, the following is characteristic:
- Dense mobile tumor. It is located under the skin. Gradually increasing. In this case, it is precisely the desmoid fibroma that can be suspected due to the fact that it is located in a place that is often subjected to surgery or injury.
- It gradually grows and becomes unbiased.
- If located on the lower limb, then this leg swells. This happens when the tumor grows through the venous fascia or adheres tightly to the vessel wall. Because of this, the outflow of blood becomes worse. This causes swelling, swelling.
- Intraabdominal location, as a result of which there may be signs of damage to the internal organs, which are located nearby. May grow from the mesentery. When the tumor reaches a large size, it causes a displacement or squeezing of the intestine. There are problems with the digestive processes - the stomach rumbles, tormenting bloating, constipation. In some cases, symptoms of bowel obstruction appear.
- If in women it is located near the mammary glands, then because of this, her breasts increase. Both its shape and the shape of the nipple may change.
- The tumor can be both mobile and connected to adjacent tissues.
- If the desmoid fibroma grows in a man near the scrotum, then the testicle is displaced and, it seems, it has increased.
- If the neoplasm sprouts to the bones, it can cause their atrophy, fractures.
- If the desmoid increases near the articular joint, it can cause contractures.
- If inflammation begins in the desmoid fibroma, and the purulent mass breaks into adjacent tissues and organs, this causes a clinical picture that is characteristic of intoxication. The patient has a fever, chills, weakness. If pus penetrates the abdominal cavity, then it will be possible to notice signs of peritoneal irritation, which indicate the development of peritonitis.
It is necessary to distinguish a desmoid from a lipoma or hematoma (especially in places where often a person receives injuries or wounds).
Diagnostics
Diagnostics includes the following activities:
- Inspection This applies to desmoid fibroids, which are located outside and represent a dense neoplasm. Palpation does not cause pain. In the early stages of development, it is not soldered to tissues and can easily shift. If this is a rather 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 scars.
- Ultrasound of the tumor. Such an examination indicates that the tumor is missing a capsule. It grows into the fascia. A vessel may be located. It is a single chamber cavity. Inside it is a substance with a jelly consistency. The ultrasound image 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 cell 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 greater the likelihood of a re-appearance of the neoplasm or its transition into a malignant tumor (in this case, it is a sarcoma). A biopsy is taken between the desmoid fibroma and healthy tissues. This helps to choose the tactics of conducting the operation and the boundaries of the excised tissue region.
- 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 health.
- CT scan. This is a series of radiography. Pictures are taken in the form of sections of the study area. For soft tissues, the information content of the procedure is less, great for bone structures. Detects the presence of calcifications.
In addition, serum estradiol is carried out to determine the need for hormone therapy. Radiography for the bones in the area where the growth is located must be performed to determine if they are affected. If the neoplasms are located in the pelvis, then cystography and excretory urography are required.
In order to assess the general condition of the patient and prepare him for surgery, a general examination is carried out, which includes analysis of urine and blood, an electrocardiogram, and a coagulogram.
Treatment
Due to the fact that there is a high probability of relapse, it is recommended to carry out complex treatment of desmoid soft tissue fibroma, which includes surgical intervention and radiation therapy. Additionally, chemical and hormonal treatment can be used. According to statistics, if only monomethodics are used, which includes only surgical intervention, then relapses are recorded in 70% of cases. If complex treatment is carried out, the risk of re-appearance of the tumor is significantly reduced.
Surgical removal
It involves excision of the tumor along the edges of healthy tissues. Quite often, the entire fascia on which the tumor 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 place. Irradiation includes several courses that the patient passes after the wound heals from surgery.
First, a wider area is treated. The total dose is 40 Gy. After 3 months, the course is repeated. In this treatment, only the central region is exposed, and the dose will be 2 times less.
Antiestrogens
In addition, antiestrogens are also used. This is due to the fact that increased fibroma growth activity in women with a significant level of estrogen, as well as with the occurrence of regression after menopause.
This became the basis for the use of Tamoxifen. The course lasts from 5 to 10 months. Additionally, once a month they use Zoladex. It contains substances that are similar to gonadoptropin-releasing hormone.
This method of treatment of the desmoid of the abdominal wall is even allowed to be used as the main one, but only in cases where there are contraindications for surgical intervention.
Gestagens
Hormone therapy also includes the use of gestagenic agents, for example, Megestrol, Progesterone. They lower estrogen levels.
Additionally
Chemotherapy is also underway. Apply funds from the group of cytostatics - this is Vinblastine and Methotrexate. The course lasts from 3 to 5 months. Be sure to monitor the biochemical parameters of blood.
Conclusion
With a desmoid of the anterior abdominal wall, the prognosis of treatment is favorable if the corresponding tissues into which fibroma can grow are removed. Over the next 3 years, relapse is observed in 60% of cases. If several treatment methods are combined, this can lead to the complete elimination of the tumor.
When wondering what you can eat with desmoid fibroma, know that you need to avoid too high-calorie and fatty foods.
In the diet should be products 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 flavorings.
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 medication for desmoid fibroma.