Uterine leiomyosarcoma is a rare malignant tumor of the body of the uterus that arises from muscle tissue (myometrium). The disease can develop in about 1-5 out of every 1000 women who have previously been diagnosed with fibroids. The average age of patients ranges from 32 to 63 years. Most cases of the disease occur in women over 50 years of age. Compared to other types of oncological processes in the uterus, this type of cancer is the most aggressive. Uterine leiomyosarcoma accounts for up to 2% of all malignant uterine tumors.
Oncology in gynecology occurs annually. Women of reproductive age are more likely to develop cancer. Many patients with leiomyosarcoma have a history of other gynecological diseases. In 75% of patients, cancer is combined with uterine fibroids.
Epidemiology
Each year, about six out of a million women are diagnosed with uterine leiomyosarcoma. The disease is often discovered incidentally when a woman undergoes a hysterectomy (removal of the uterus) due to the large size or number of fibroids. It is quite difficult to detect the development of the oncological process before surgery. This is due to the fact that most women have several myoma nodes. And to make a diagnosis, it is necessary to conduct a biopsy of each of them.
Reasons
The exact cause of uterine corpus leiomyosarcoma is unknown. The oncological process often occurs spontaneously, without apparent reason. Researchers suggest that certain factors contribute to certain types of cancer. These include:
- genetic and immunological abnormalities;
- environmental factors (e.g. exposure to ultraviolet rays, certain chemicals, ionizing radiation);
- overweight;
- stress.
In people with cancers, including leiomyosarcoma, malignancies can develop due to abnormal changes in the structure and location of certain cells, known as oncogenes or suppressor genes. The former control the growth of cells, the latter control their division and death. The specific reason for the change in these genes is unknown. However, studies show that abnormalities in DNA (deoxyribonucleic acid), which is the carrier of the body's genetic code, are the basis of cellular malignancy.transformations. These abnormal genetic changes can occur spontaneously for unknown reasons and in rare cases can be inherited.
The occurrence of leiomyosarcoma may be associated with specific genetic and environmental risk factors. Some hereditary conditions that run in families can increase the risk of developing the disease. These disorders include:
- Gardner's syndrome is a rare hereditary disease characterized by the appearance of adenomatous polyps in the intestine, multiple skin lesions and osteomas of the bones of the skull.
- Li-Fraumeni syndrome is a rare disease with a hereditary pathology. It is characterized by the development of a cancerous disease due to mutations in the gene responsible for the development of a malignant process in the body.
- Werner's Syndrome (or Progeria) is a disease that manifests itself in premature aging.
- Neurofibromatosis is a condition characterized by skin discoloration (pigmentation) and tumors in the skin, brain, and other parts of the body.
- Immune deficiency syndromes (HIV, primary, secondary immunodeficiency). Immune system disorders due to certain causes. For example, defeat by a virus, corticosteroids, radiation, and so on.
No exact link has been found between leiomyosarcoma and these disorders.
Signs and symptoms
The symptoms of uterine leiomyosarcoma vary depending on the exact location, size and progression of the tumor. In many womenthe disease is asymptomatic. The most common sign of a malignant process is abnormal bleeding during menopause. Unusual discharge is an important factor that can indicate not only uterine leiomyosarcoma, but also other gynecological diseases.
Common symptoms associated with cancer are feeling unwell, tired, chills, fever and weight loss.
Signs and symptoms of uterine leiomyosarcoma may include:
- Vaginal bleeding.
- Formation in the pelvic region, which can be identified by touch. Observed in 50% of cases.
- Pain in the lower abdomen occurs in about 25% of cases. Some tumors are very painful.
- Unusual feeling of fullness and pressure in the pelvic region. In some cases, bulging of the tumor is noted.
- Vaginal discharge.
- Lower abdomen enlargement.
- Increased urination due to tumor compression/pressure.
- Lower pain.
- Pain during intercourse.
- Hemorrhage. Bleeding may occur with large tumors.
- Heart attack. Hemorrhage in the tumor can lead to tissue death.
Uterine leiomyosarcoma can spread locally to other areas of the body, especially the lungs and liver, often causing life-threatening complications. The disease tends to recur in more than half of the cases, sometimes inwithin 8-16 months of initial diagnosis and treatment.
Diagnosis
To diagnose uterine leiomyosarcoma, a histological examination is performed. Examination of fibrous tissue is a key diagnostic aspect that distinguishes malignant leiomyosarcoma from benign leiomyoma. To assess the size, location, progression of the tumor, an additional examination is prescribed. For example:
- computed tomography (CT) scan;
- magnetic resonance imaging (MRI);
- transvaginal ultrasound (ultrasound).
CT scans use a computer and x-rays to create a film that shows cross sections of certain tissue structures. MRI uses a magnetic field and radio waves to produce cross-sectional images of individual organs and tissues in the body. During an ultrasound examination, reflected sound waves create an image of the uterus.
Laboratory tests and specialized diagnostics may also be performed to determine possible infiltration of regional lymph nodes and the presence of distant metastases.
Stages of disease
One of the biggest problems with cancer diagnosis is that cancer metastasizes (spreads) beyond its original location. The stage is indicated by a number from 1 to 4. The higher it is, the more the cancer has spread throughout the body. This information is necessary forplanning the right treatment.
The following stages of uterine leiomyosarcoma are distinguished:
- Stage I - the tumor is only in the uterus.
- Stage II - Cancer has spread to the cervix.
- Stage III - Cancer extends beyond the uterus and cervix but is still in the pelvis.
- Stage IV - cancer has spread beyond the pelvis, including the bladder, abdomen, and groin.
Treatment
Uterine leiomyosarcoma is a rare but clinically aggressive malignancy. The choice of treatment tactics is carried out depending on various factors, such as:
- primary tumor location;
- disease stage;
- grade of malignancy;
- tumor size;
- tumor cell growth rate;
- tumor operability;
- spread of metastases to lymph nodes or other organs
- age and general he alth of the patient.
Decisions regarding the use of specific interventions should be made by physicians and other members of the medical committee after careful consultation with the patient and on the basis of the particular case.
Surgery
The main form of treatment for uterine corpus leiomyosarcoma is the removal of the entire tumor and any affected tissue. A complete surgical removal of the uterus (hysterectomy) is usually performed. Removal of the fallopian tubes and ovaries (bilateral salpingo-oophorectomy) may be recommended for women who are in the menopausal period, as well asin the presence of metastases.
After the removal of the uterus, the consequences for the body are to stop regular menstrual bleeding. This means that the woman will no longer be able to have children. But since uterine leiomyosarcomas usually occur in older women, hysterectomy after age 50 should not be a problem. Usually women already have children or no longer plan to become pregnant. However, existing methods of assisted reproductive technology are a possible solution for couples who want to have a baby.
In addition to the loss of fertility, after removal of the uterus, the consequences for the body can be expressed in the following symptoms:
- loss of sex drive;
- hormonal imbalance;
- psychological disorders;
- appearance of secretions;
- pain;
- weakness.
Treatment of patients with metastatic and/or recurrent disease should be determined on an individual basis. The best option is to completely remove the tumor. However, this is not always possible. The patient needs regular check-ups to prevent relapse.
Chemotherapy and Radiation Therapy
After surgery, medical treatment is prescribed in combination with chemotherapy and radiation therapy. In some cases, radiation therapy may be used before surgery to shrink the tumor. At stages 3 and 4, it does not always give a positive result.
To destroy tumor cells, the doctor prescribes special drugs in the form of tablets or injections. Certain combinations of chemotherapy drugs may also be used. Research is currently underway to develop new chemotherapeutic combinations that may be useful in the treatment of leiomyosarcoma.
Possible Complications
Leiomyosarcoma is a type of soft tissue sarcoma. Before, during and after diagnosis and treatment of a uterine tumor, the following possible complications may occur:
- Stress, anxiety, apathy due to uterine cancer.
- Heavy and prolonged menstrual bleeding can lead to anemia.
- The tumor can undergo mechanical damage, such as twisting, which can lead to excruciating pain. Polypoid tumors are known to cause cervical prolapse in some cases.
- Some tumors grow to large sizes and even protrude from the uterus, affecting the adjacent reproductive organs.
- Cancer can spread in any direction, even regionally. It can affect the gastrointestinal tract or urinary tract.
- Delay in diagnosis can lead to spread of metastases.
- Metastases in the early stages of uterine leiomyosarcoma occur due to the high vascularity (blood supply) of the uterus. As a rule, the lungs are usually affected first.
- Swelling can also adversely affect adjacent/surrounding structures such as nerves and joints, leading to discomfortor loss of sensation.
- Side effects of chemotherapy and radiation.
- Sexual dysfunction can occur as a side effect of surgery, chemotherapy or radiation therapy.
- Tumor recurrence after incomplete surgical removal.
Leiomyosarcoma of the uterus. Forecast
The primary treatment for patients with newly diagnosed leiomyosarcoma is surgical removal of the uterus and cervix. Approximately 70-75% of patients are diagnosed with the disease at stages 1-2, when the cancer has not yet spread beyond the organ. The 5-year survival rate is only 50%. Women with metastases that have spread outside the uterus and cervix have a very poor prognosis.
To assess the patient's condition, specialists use the following characteristics of an oncological tumor:
- size;
- cell division rate;
- progression;
- location.
Despite complete surgical removal and best available treatments, approximately 70% of patients may relapse on average 8-16 months after initial diagnosis.
After treatment
In case of gynecological diseases complicated by oncology, a hysterectomy is prescribed. This forced measure is aimed at saving the life of the patient. The postoperative period after removal of the uterus is to observe and comply with all recommendations by the patient. For example:
- limit physical and sexual activity for 6 weeks;
- wearing a brace;
- rest and sleep;
- don't use tampons;
- do not visit baths, pools, use showers.
How often should I see a gynecologist? Examinations are recommended every 3 months during the first three years after diagnosis. Computed tomography is performed every six months or a year for control. If you experience any unusual symptoms in the postoperative period after removal of the uterus, you should immediately consult a doctor.
Where to go?
Oncogynecologists treat leiomyosarcoma of the body of the uterus. And, I must say, quite successfully. One of the leading scientific and medical institutions for cancer in our country is the Herzen Cancer Center in Moscow. The clinic carries out a wide range of modern methods of research and treatment of oncological diseases, including uterine cancer. Malignant tumors of the female genital organs occupy a special place in oncology. It is these gynecological diseases that are most often found in women. What to do, this is the scourge of modern society. Every year, more than 11,000 patients receive specialized medical inpatient care at the Herzen Cancer Center in Moscow.
In conclusion
Leiomyosarcoma of the body of the uterus is a rare tumor that accounts for only 1% to 2% of all malignant neoplasms of the uterus. Compared to other types of uterine cancer, it is a tumoraggressive and associated with high rates of progression, relapse and mortality.
Treatment of malignant neoplasms is mainly carried out through surgery and additional therapeutic measures, which include radiation therapy and chemotherapy. The prognosis of uterine leiomyosarcoma mainly depends on the stage of the cancer and other factors.
Medical centers and hospitals specializing in sarcomas are researching new treatments for people with soft tissue sarcomas, including new chemotherapy drugs, new drug combinations, and various biological therapies that involve the immune system in fighting cancer.
