Uterine sarcoma

A non-epithelial malignant neoplasm that forms from the base of the mucous membranes, connective tissue and muscle fibers of the walls of the uterus is called uterine sarcoma.

Uterine sarcoma is a rare but rather insidious disease. It is a malignant tumor, which is included in the tragic list of progressive oncological diseases. Uterine sarcoma accounts for approximately 3-5% of all malignant tumors of the uterus, moreover, the disease occurs in the body of the uterus approximately 3 times more often than on the cervix. According to statistics, women aged 45 to 57 years suffer from uterine sarcoma. It is impossible not to note such a sad fact that even with timely detection of sarcoma in the initial stage of the disease, a positive treatment result is very rarely observed.

The disease is difficult to diagnose in the early stages of development. However, a combination of treatment and the right approach bring results in the fight against the disease.

[ 1 ], [ 2 ], [ 3 ], [ 4 ], [ 5 ]

Causes of uterine sarcoma

The etiological and pathogenetic characteristics of the disease have not yet been sufficiently studied. Experts suggest that the formation of sarcoma is a polyetiological process that could provoke damage to the cellular structures of restorative tissues.

Sarcomatous formation is often preceded by other pathologies:

• benign fibromyoma that appears as a result of hormonal imbalance;
• disorders of embryonic development;
• trauma during childbirth;
• violation of the integrity of the uterine tissue after an artificial abortion or curettage for diagnostic purposes;
• tissue proliferation disorders (development of endometriotic polyps, pathological proliferation of the endometrium).

Bad habits (nicotine, alcohol, excessive use of medications), features of professional activity (presence of intoxication, hazardous production), ecology, and radiation therapy play an important role in the development of sarcoma.

Since the pathology most often occurs in women of menopausal age, its appearance can be associated with the cessation of ovulation, increased estrogen levels in the body, and neuroendocrine disorders.

Women who are at risk of developing maca sarcoma may be:

• who had breast cancer;
• with late onset of menopause (after 50 years);
• suffering from polycystic ovary syndrome;
• never given birth.

A huge role can be attributed to heredity, as well as genetic predisposition to various tumors. Sarcoma affects those tissues and organs that were previously injured. Women who have undergone chemotherapy, as well as those suffering from the herpes virus, may be at risk. Chronic intoxication, occupational diseases, bad habits such as smoking and excessive alcohol consumption, as well as excess weight are also present.

[ 6 ], [ 7 ], [ 8 ], [ 9 ]

Symptoms of uterine sarcoma

As noted above, women aged 43 to 55 are at risk. This age suggests that menopause is approaching or has already occurred. Therefore, all women over 40 should closely monitor their health and, if the first symptoms are detected, immediately consult a doctor. If the disease is detected at an early stage, then there is a greater chance of recovery.

It should be noted that with uterine sarcoma, there are very rarely any typical symptoms that could alert a woman. Uterine sarcoma is considered a "silent tumor", since even in the last stage of the disease, this insidious disease does not manifest itself in any way. Sarcoma develops in the myomatous node, posing as a uterine fibroid. When bloody discharge from the vagina begins, menstrual irregularities, bleeding, pus or leucorrhoea, attacks of aching pain in the small pelvis appear, it can be assumed that the disease has gone beyond the uterus. At this stage of the disease, the woman's appearance changes, yellowness appears on the face, weakness, loss of appetite, which leads to exhaustion of the body, anemia, and the structure of the blood also changes.

At the initial stages, the disease may proceed unnoticed for a long time, which significantly complicates the diagnosis. If sarcoma is formed inside fibromatous nodular formations, the symptoms may resemble the clinical picture of one of the forms of fibroma (benign tumor) of the uterus.

Rapid development of sarcoma can be manifested by the following signs:

• menstrual cycle disorder;
• aching pain in the lower abdomen;
• the appearance of pronounced watery discharge, which most often has an unpleasant odor.

The most striking symptoms are observed during the formation of endometriotic sarcoma or when submucous nodular formations are affected.

In the later stages, signs of sarcoma become apparent. These include:

• anemia;
• weight loss, loss of appetite, increased fatigue;
• symptoms of intoxication of the body, accumulation of fluid in the abdominal cavity.

When metastases occur, hepatitis, pleurisy, spinal damage and other pathologies may develop, depending on the organ to which the daughter cells of the tumor have been transferred.

Types of uterine sarcoma

The World Health Organization identifies a fairly large number of varieties of uterine sarcoma. All of them have different localization in relation to the organ, as well as the course and rapidity of growth.

Let's look at the most common types of sarcomas.

Sarcoma of the cervix

A rather rare type of tumor that begins its development from muscle and connective tissue cells, from the body of the fibromyoma, blood supply vessels or mucous membranes. If you look at such a tumor in section, it resembles the appearance of "boiled fish", and on the outside the formation has a matte structure with small necrotic zones and hemorrhages. Often, sarcoma can be confused with a polyp, which is capable of ulceration. The parent sarcoma can form from the mucous membranes: in such cases, the formation resembles a bunch of grapes.

Cervical sarcoma is much less common than uterine sarcoma. In such cases, the diagnosis of "cervical cancer" is usually established, since during the examination, the sarcoma is very similar to cancer, and only after a histological examination is an accurate diagnosis of "cervical sarcoma" established. The tumor grows within the anterior and (or) posterior lip, with the phenomenon of irreversible changes in the cells, the metabolism is disrupted, which can lead to cell degeneration (necrobiosis). Usually, the process of tumor development occurs in the cervical canal. The development of the disease is preceded by fibroma of the cervix or polyps.

As for the duration of the disease, it can be said that from the moment the first signs of cervical sarcoma are detected, patients live on average about 2 years. Patients die from sarcomatous pneumonia, as metastases penetrate the lungs. There may also be profuse uterine or intra-abdominal bleeding, pyelonephritis, uremia (compression of the ureters), peritonitis (rupture of the sarcoma in the abdominal cavity), and also from liver failure.

Sarcoma of the uterine body

Uterine body sarcoma (leukomasarcoma) is a fairly common form of sarcoma that appears on the smooth muscles of the uterus, which is combined with uterine myoma. This insidious disease affects women aged 43 to 52 years. The tumor is localized in the wall or in the cavity of the uterus, less often - located under the mucous membrane of the uterus, or occurs in the outer part of the uterus, progressing to the lower pelvic cavity. The tumor spreads to the internal and parietal organs of the peritoneum, giving metastases. Later, metastases appear in the lungs, bones, liver, etc.

This disease begins its development in the tissues of the endometrium or myometrium, within the uterus. Gradually, such a tumor progresses, spreading to the pelvic area, growing into the parametrium and sending metastases to the ovaries. In the absence of treatment, metastasis continues to the lymphatic system, external genitalia.

Uterine stromal sarcoma

A tumor growing from the stroma of the mucous membranes of the uterine walls. The course of such a disease can be both aggressive and relatively benign, which is due to the biological characteristics of the neoplasm (parameters, genomic disorders, rate of progression). Endometrial stromal sarcoma of the uterus, according to the most common version of specialists, develops from nascent stromal cells, or appears as a result of metaplasia of neoplastic cells. Such a tumor is clinically manifested by bloody discharge from the vagina, with a bimanual examination, an increase in the size of the uterus can be detected, sometimes with a heterogeneous consistency.

Endometrial stromal sarcoma of the uterus is distinguished by a high degree of malignancy. There is an aggressive course of the disease, as a result of which additional tumor nodes may appear, both in nearby and distant organs (metastases). The cause of death in about 90% of cancer patients is not a tumor, but metastases. Endometrial stromal sarcoma of the uterus is characterized by a rather unfavorable prognosis with a high degree of hematogenous metastasis, as a result of which the sarcoma spreads through the blood vessels to any human organ. The number of mitoses under an enlarged microscope is more than 10 in 10 fields of vision. Endometrial sarcoma of the uterus is observed in women aged 45 to 50 years. The disease progresses mainly during menopause and is a tumor in the form of an ecophytic form. In endometrial stromal sarcoma of the uterus, uniform cells are formed that resemble normal endometrial stromal cells. In medicine, three types of endometrial sarcomas are distinguished. These are endometrial stromal nodule, endometrial stromal sarcoma of high and low malignancy. The most dangerous degree is endometrial sarcoma of the lower degree, since at this stage the tumor already noticeably spreads beyond the small pelvis, actively penetrating through tissue barriers.

Metastases in uterine sarcoma

Sarcoma is capable of spreading metastases through the blood or lymph flow, or growing into neighboring organs.

Smooth muscle sarcomas develop the slowest, so they can metastasize somewhat later. Such uterine sarcomas release their particles into the blood, from where they enter the respiratory and skeletal systems, liver, and external genitalia. When daughter cells spread to the lungs, the left side is most often affected: the right lung is affected less often. Metastatic lesions of the peritoneum and omentum tissues are usually accompanied by fluid accumulation in the abdominal cavity.

Most often, sarcoma metastasizes to the appendages: this condition is especially common with endometrial sarcoma, and a little less often with the mixed form of mesodermal formation.

The process of metastasis spreading that has begun can quite quickly provoke a lethal outcome. Metastasis dissemination can be chaotic, and the sequence of such a process is quite difficult to determine.

Diagnosis of uterine sarcoma

Uterine sarcoma is extremely difficult to diagnose based only on the collected anamnesis and clinical picture of the disease. The set of characteristic signs of the disease must be confirmed by other additional studies that provide more accurate information.

Examination of the external condition of the genitals, mirror and bimanual examination may allow one to assume the presence of oncological pathology. This conclusion is facilitated by the determination of nodular formations and tubercles in the uterus, daughter neoplasms in the vagina.

[ 10 ], [ 11 ], [ 12 ], [ 13 ]

Uterine sarcoma on ultrasound

Ultrasound is probably the most popular type of examination and further monitoring of fibroid growth, determining the size of the pathology, the degree of deformation and damage to the uterine walls. Uterine sarcoma on ultrasound does not provide such a clear idea of the tumor that would be required: such a pathology does not have bright acoustic symptoms. At the same time, stable growth of the neoplasm during examination in dynamics (obvious development of the tumor during the year), as well as changes in the structure of fibromatous nodular formations simultaneously with characteristic clinical signs can become a reason for the use of surgical intervention. Ultrasound, in addition, helps to observe the condition of nearby lymph nodes and organs, to assess the possibility of metastasis.

Of course, it is impossible to clearly identify sarcoma using this method, but you can notice an increase in the tumor focus during a control examination, for example, during the year by an amount that corresponds to the pregnancy period of about 5 weeks. In this case, rapid tumor growth is considered. Also, with the help of ultrasound, you can track how fibromatous nodes change during menopause and in the postmenopausal period. Ultrasound examinations give a clear definition for making a decision on surgical intervention, and also help to examine neighboring organs for a tumor. The ultrasound method for uterine sarcoma determines pathological changes, localization, size of nodes and recognizes deformation of the uterus.

The method of aspiration biopsy followed by cytology of secretions allows to detect exfoliated elements of the neoplasm.

The hysteroscopy method provides the opportunity to pay attention to pathological changes in tissues: it is possible to detect tumors with different outline patterns, as well as perform a targeted biopsy of the formation.

The histological method is the "gold standard" in diagnosing malignant tumors in the uterus. The material required for analysis can be removed using colposcopic biopsy, excision, curettage, etc. Sometimes separate analytical scraping of the mucous membrane of the uterine cavity is performed, this method is especially relevant in the case of endometrial tissue invasion, with stromal endometrial neoplasm. In other variants of tumor location, changes in the structure of the inner lining of the uterus are not detected. Immunohistochemical examination is also indicative: malignant sarcoma cells are positively tuned to vimentin (almost 96%), and locally - to actin. Markers of mesenchymal differentiation are represented by desmin, cytokeratins, actin, collagen type IV, vimentin.

Among the auxiliary methods, we can highlight magnetic resonance imaging or computed tomography of the pelvis, x-ray of the lungs, colonoscopy, rectosigmoidoscopy, blood tests (presence of anemia).

A definitive diagnosis can only be made on the basis of histology after removal of the sarcoma.

Treatment of uterine sarcoma

There are several methods of treating uterine sarcoma, but the most common is the surgical method followed by chemo-radiotherapy. Surgical treatment allows, first of all, to determine the stage of the disease with subsequent removal of the main part of the tumor. This method of treatment is used at an early stage of the disease. In other cases, when the disease is detected late, surgery is not enough. Other methods of treatment are needed here. As for radiation therapy, this is one of the most effective methods of treating uterine sarcoma today. It should be noted that surgical treatment or chemotherapy alone will not give positive results. This method of treatment "works" only in combination. The combination of these two methods of treatment allows you to get rid of the roots of the tumor. A folk method of treating uterine sarcoma is widely used. There is a huge variety of folk medicine recipes, however, cases are different. For example, completely hopeless patients who have tried all possible types of treatment for uterine sarcoma recover using this or that recipe of folk healers. Very often it is recommended to use the aloe flower. This wonderful plant can have an antitumor effect, while restoring immunity. Take one large aloe leaf, crush it and pour one liter of alcohol. Infuse for two weeks, after which you need to take one tablespoon before meals three times a day before meals.

The most commonly used therapeutic measures for uterine sarcoma are chemotherapy, surgical removal of the tumor, or combined therapy of the pathology.

Considering the fact that sarcoma tends to unimpeded lymphogenous metastasis, specialists consider the best treatment option to be surgical. It involves a large-scale extirpation of the uterus with ovaries, possibly involving the greater omentum (high probability of metastasis spread). Nearby lymph nodes may also be removed.

Depending on the stage, additional radiation and/or chemotherapy may be prescribed.

Chemotherapy for uterine sarcoma

Chemotherapy for uterine sarcoma is determined by the use of therapeutic regimens using anthracyclines - the so-called antitumor antibiotics. Such drugs include daunorubicin, doxorubicin, idarubicin, or epirubicin, which is actively used in world practice. At the present time, the effect and presence of a positive effect from the drug Ifosfamide, its use both in monotherapy and in combination with other antitumor agents are being studied.

Positive dynamics with such chemotherapy can be 15-30%.

Combination therapy is generally more effective, but also more toxic. Positive effects are seen with combination therapy with docetaxel and gemcitabine (53% effectiveness), and repeated control treatment is possible if tumor recurrence is suspected.

In some cases, the use of hormonal therapy, gestagen and antiestrogens, and high doses of progesterone is allowed when diagnosing low-grade stromal sarcoma.

Combination radiation therapy and its role in the effectiveness of treatment of uterine sarcoma has not yet been sufficiently studied.

[ 14 ], [ 15 ], [ 16 ], [ 17 ]

Prevention of uterine sarcoma

First of all, prevention of uterine sarcoma consists of regular preventive examinations by a gynecologist. If a benign tumor of the uterus is detected, it is necessary to undergo timely treatment, but if the disease is not detected, it is recommended to seek advice approximately 2-3 times a year. It is also necessary to follow all the doctor's prescriptions and recommendations when treating female diseases, especially if this concerns hormonal imbalances. Experts also advise not to delay childbirth, it is advisable to have two or three children. If you do not plan to have children, you should use modern methods of protection against unwanted pregnancy, avoid abortions. You should also stick to a diet, limiting the consumption of animal fats. Eat more vegetables and fruits. And, of course, we cannot fail to mention a healthy lifestyle. Give up bad habits such as smoking, drinking alcohol, spend more time outdoors and play sports.

Preventive measures include regular visits to a gynecologist, medical examinations, both in childhood and during menopause. It is important to detect and treat neuroendocrine disorders, inflammatory processes of the uterus and appendages, fibroids, and polyposis in time.

A balanced hormonal background also plays a role in disease prevention, so it is necessary to follow the following principles:

• choose contraceptives correctly, always consulting with a doctor;
• prevent unwanted pregnancy, avoid artificial abortions;
• establish a full and active sex life;
• avoid stressful situations, do not overwork, lead a healthy lifestyle;
• treat any infectious and inflammatory diseases promptly.

Prognosis of uterine sarcoma

The prognosis for uterine sarcoma is ambiguous: tumors that form from fibromatous nodular formations (with uncommon metastases) have a more favorable course, but endometrial sarcoma does not have such a positive trend. According to statistics, the survival rate of patients over a five-year period is as follows:

• at the first stage of sarcoma – 47%;
• at the second degree – about 44%;
• at the third degree – 40%;
• with fourth degree tumors – only 10%.

Intensive research currently being conducted by molecular geneticists allows us to hope that in the near future we will receive an answer to the numerous questions associated with the diagnosis of uterine sarcoma. Scientists are working hard to allow medicine to control the course of the pathology, increase the adequacy of therapeutic measures and preserve and prolong the lives of patients.