Leiomyosarcoma is malignant, occurring quite rarely, developing from a smooth muscle tissue tumor. Most often, leiomyosarcoma develops in the uterus, stomach, bladder, on the skin, in the small intestine. Oncologists believe that leiomyosarcoma is an aggressive tumor with a high incidence of relapse. Also for this pathology is characterized by early metastasis to distant organs.
The risk group for the incidence of leiomyosarcoma of the uterus includes women 40-50 years of age and older. With leiomyosarcoma of the skin in the risk group, persons of 40-60 years of both sexes.
The causes of leiomyosarcoma are not fully understood, and the origin of the disease remains unknown. Oncologists distinguish between the following risk factors:
- Permanent (chronic) trauma of soft tissues, which often serves as a trigger for cell mutation.
- Exposure to radiation exposure, for example, when using radiotherapy.
- The patient has a leiomyoma - a benign tumor, prone to degeneration into a malignant form.
Symptoms of leiomyosarcoma
The disease has both general manifestations, and local, or local. Common symptoms include increased fatigue, nausea, weight loss for no apparent reason. Also, there is pain and bleeding in the area of the lesion.
Symptoms of leiomyosarcoma, developed in the gastrointestinal tract: weight loss, nausea, intestinal obstruction, rupture of intestinal walls.
Symptoms of leiomyosarcoma of the esophagus: a disorder of swallowing (dysphagia).
Leiomyosarcoma on the skin, in the subcutaneous tissue, on the surface of the skin of the genital organs is manifested by neoplasm in the form of an irregular plaque shape, redness or cyanosis of the skin in the tumor region.
Symptoms of uterine leiomyosarcoma: an increase in the body, a violation of the cycle of menstruation with a tendency to heavy bleeding.
With tumors of the uterus, paraneoplastic syndrome, which is manifested by anemia , fever and migrating thrombophlebitis, is of particular importance. Fever that does not pass after antibiotic therapy, with an incomprehensible reason, with improper daytime body temperature rises with a relatively calm picture of blood, suggests a tumor process. An increase in temperature is observed in 36% of tumors in the reproductive system of women. The causes of fever are infection, tumor decay, the body's response to antigens against the tumor.
According to some authors, approximately 25% of patients with tumors have vein thrombosis, both deep and superficial, which is the first symptom of a developing malignant tumor. Poorly treatable venous thrombosis, migrating thrombophlebitis, may be the first signs of a latent malignant process. Such thrombophlebitis often outstrips the clinical manifestations of malignant process. After surgery, thromboembolism occurs in more than 55% of cases.
Stages of leiomyosarcoma
There are four main stages of the onset of this cancer:
Leiomyosarcoma does not go beyond the muscular layer:
Stage I A - involves the involvement of the myometrium or endometrium;
Stage I B - in the process affected myometrium and endometrium
Leiomyosarcoma is located within the neck and body of the uterus:
Stage II A - the presence of "ingrowth" of tumor cells in the parameters within the organ;
Stage II B - the cervix is involved in the process
Leiomyosarcoma sprouts beyond the uterus, but is limited to a small pelvis:
Stage III A - infiltration of the parameter with the involvement of the pelvic wall in the process;
Stage III B - metastases spread to the ovaries, vagina, lymph nodes;
Stage III C - the tumor sprouts through the serous membrane of the uterus.
Leiomyosarcoma goes beyond the small pelvis:
Stage IVA - the bladder and / or rectum are involved in the tumor process;
Stage IV B - metastases in various organs, including very distant ones.
Leiomyosarcoma of the uterus
This diagnosis is exposed in 45% of all cases of detection of malignant tumors associated with muscle tumors in women. It is in the uterus that leiomyosarcoma develops not as tumors in other organs. The disease proceeds without pronounced symptoms, therefore, a visit to the gynecologist at least once a year is of great importance.
At risk are patients who have been diagnosed with uterine fibroids , since benign muscular tumors can degenerate into malignant tumors. When performing operations to remove the uterus with fibroids, in elderly women, leiomyosarcoma is often found. The average age of patients with this pathology is 50-55 years. But it does not say that this type of cancer can not develop in women of a younger age. If, after the onset of menopause, a patient with myoma found a sharp increase in fibroids, it is necessary to be alerted and urgently go to the doctor. There is a high probability that the benign myoma has passed into a malignant form. And as diagnostics without laboratory tests is difficult, it is necessary to make a tumor biopsy under the control of MRI, or to agree to surgical treatment - removal of the tumor. And, the earlier the patient decides to surgical treatment, the better will be the prognosis for her life, since leiomyosarcoma has a desire for very rapid growth.
- The uterus began to increase sharply. The woman herself notes some bloating in the lower abdomen, a feeling of pressure on the organs of the small pelvis. This is accompanied by frequent constipation, numerous urge to urinate. In this case, the symptoms of bowel disturbance are absolutely not related to the pathology of the gastrointestinal tract or to the urinary tract disease. If there are any diseases in the organs of the digestive tract or bladder, the therapy does not bring relief.
- Bloody discharge in menopause . Many ladies ignore these bleedings, considering it as residual phenomena. But to ignore such a serious sign is unacceptable. Even if the patient does not have a tumor process, spotting can be symptoms of other gynecological diseases.
- Specific discharge from the genital tract without signs of bleeding. The unusual color of the secretions, their strange smell, should alert and facilitate an early visit to the treating doctor.
- The feeling of pressure, raspiranie and heaviness in the region of the lone articulation also indicate that the uterus is greatly enlarged. Drawing pains that occur without a cause, discomfort, when trying to coit unpleasant feelings should not be ignored.
Diagnosis of leiomyosarcoma
The main diagnostic methods for suspected involvement of a given malignant tumor are:
- The blood test is general and detailed.
- Biopsy - taking a small piece of pathological tissue with the subsequent examination of it under a microscope for the installation of tissue affiliation and the pathology phase.
- Ultrasound and X-ray examination are necessary to obtain information about the size of the tumor and the degree of its germination into neighboring organs.
- MRI and CT are needed to determine the structure of the tumor and detect metastases in distant organs.
Excretory urography is needed to determine the presence of tumor germination in the organs of the excretory system (bladder).
- Irrigoscopy will show whether there is sprouting of leiomyosarcoma in the rectum.
Diagnosis of uterine leiomyosarcoma consists in the fact that the doctor of a woman who is in the menopause period should be wary of the suddenly changing, rapidly growing benign uterine tumor. Prior to surgery or biopsy tumor tissue, it is difficult to accurately diagnose leiomyosarcoma. MRI gives information about the presence of a tumor, but this is not enough.
The exact methods include a biopsy of tumor tissue under the control of MRI. In some clinics, MRI and blood tests for serum lactate dehydrogenase are combined, which contributes to the accurate diagnosis of uterine leiomyosarcoma.
Treatment of leiomyosarcoma
The main method of treatment of uterine leiomyosarcoma is its surgical removal together with the organ, that is, the uterus. At the stage of the I and II tumor growth processes, the leiomyosarcoma does not go beyond the uterine body in 70-75% of patients. This means that survival in the first 5 years after surgery is 50% if the tumor does not go beyond the body.
Experts are convinced that with leiomyosarcoma I and II stage as early as possible should be performed such an operation as total abdominal hysterectomy (complete removal of the uterus ). Bilateral salpingo-oophorectomy, that is, removal of the ovaries with the fallopian tubes is recommended for patients who are in the menopause or metastatic cancer.
In 3% of women with uterine leiomyosarcoma, tiny metastases occur in the ovaries, so gynecologists-oncologists advise that ovaries be removed to all patients. The situation is complicated by the fact that tumor growth can stimulate hormones produced by the ovaries. This is the right approach, theoretically. To date, scientists have not yet received enough information and can not argue that the number of recurrent tumors in young women after bilateral salpingo-oophorectomy is less than in patients who did not have ovaries removed.
Chemotherapy and radiotherapy, necessarily prescribed after the operation, is called "adjuvant therapy". Adjuvant pelvic irradiation can reduce the likelihood of cancer recurrence in the pelvic region, but it has not been shown to affect the risk of tumor metastasis in the other organs (lungs, liver), and if a relapse occurs, it is localized in other organs in 80 % of cases.
In the postoperative period the patient is under medical (constant!) Control. For examinations, you must come every three months for the first three years. And every six months to undergo CT examination.
The spread of leiomyosarcoma to the cervix and beyond the uterus itself, suggests that the prognosis is very unfavorable. The tumor grows to large sizes and very often recurs. The prognosis depends on various factors: the nature of the tumor, its size, the type of DNA of the tumor cells, the state of the hormonal receptors, the features of the division of the tumor cells and much more. But none of these factors makes it possible to predict the further development of the disease.
Unfortunately, surgical treatment does not prevent the development of metastases that spread through the body with blood flow, that is, hematogenous way.
Even with the surgical method of treatment, 70% of patients have a relapse of the disease within one year / year and a half.
Therapy of leiomyosarcoma III and IV stage and recurring (recurrent) leiomyosarcoma. In such cases, treatment is strictly individual. Optimal option - surgical treatment, including the removal of all tumors, if possible. Radiation therapy is used to reduce the size of the tumor and increase the chance of success of surgical intervention.
The body's response to chemotherapy is usually sluggish. The most effective combination of drugs for chemotherapy. This gives a positive response of the body to treatment in 55% of cases. According to experts, even with the most effective drugs, the disease does not progress only in less than 1 year.
Prognosis of leiomyosarcoma
The prognosis for leiomyosarcoma depends entirely on the stage at which the disease was diagnosed and treated. In the first and second stages the neoplasm does not go beyond the uterus, so the prognosis in such cases is more optimistic. But, despite the latest technology in 70% of cases, with the most favorable initial development of the process, metastases appear within the first year. Leiomyosarcoma metastasizes (spreads) to the liver, stomach, lungs and other organs located far beyond the small pelvis. Lethal outcome occurs due to metastasis and cancer intoxication.
The prognosis of leiomyosarcoma is quite unfavorable if the lesion is outside the uterus. Quite bad signs are necrosis and multiple hemorrhages in the tissue of the tumor itself, if its size is more than 8 centimeters. If the tumor diameter is less than 5 centimeters, patients can expect survival for 5 years, which is, on average, 15-30% of patients.
Prevention of leiomyosarcoma: compulsory medical examinations every six months for the earliest possible detection of the disease.