The term "soft tissue liposarcoma" refers to a malignant neoplasm that, under the influence of various adverse factors, begins to form in the fat layer. According to statistics, the disease is most often diagnosed in men aged 50 to 60 years. In most cases, soft tissue liposarcoma is formed in the femoral area. Somewhat less often, it is localized on the buttocks and retroperitoneal space. In isolated cases, the tumor is formed in other parts of the body, including in the internal organs. Currently, the only treatment for soft tissue liposarcoma is surgery. Chemotherapy and radiotherapy are prescribed as maintenance.
Pathogenesis
Under the influence of various adverse factors, a neoplasm begins to form in the adipose tissue. The basis of the tumor is immatureconnective tissue cells called lipoblasts.
Over time, the neoplasm increases in size. Liposarcoma grows deep into the soft tissues, affecting the muscles, periarticular zones and fascia. As a rule, the boundaries of the tumor are well defined. The neoplasm can reach up to 25 cm in size. The tumor has a yellow color and a granular structure.
Liposarcomas are divided into several types:
- Highly differentiated. They are characterized by frequent episodes of relapses, but metastases are not characteristic. The tumor is represented by mature cells and has a favorable prognosis.
- Mixoid. Soft tissue liposarcoma (a photo of the neoplasm is presented below) consists of mature cells and lipoblasts. As a rule, it is localized on the hips.
- Pleomorphic. Contain cells of a round or spindle-shaped form. Pleomorphic soft tissue liposarcoma is a poorly differentiated type.
- Round cell. Made up of cells that don't look like fat.
- Undifferentiated. Contain two components that can be traced in different places of the neoplasm.
The density of the tumor is uneven. The growth of a neoplasm can lead to serious consequences, as a result of which it is necessary to consult a doctor at the first warning signs.
Features of soft tissue myxoid liposarcoma
Most often the tumor is localized in the proximal parts of the lower extremities. The neoplasm is a single node that has an irregular shape.
Mixoidsoft tissue liposarcoma (a photo of the tumor is schematically shown below) is represented by both mature fat cells and spindle-shaped and round lipoblasts. This tumor is characterized by the presence of a mucoid stroma. Liposarcoma has a large number of blood vessels.
This type of tumor recurs very often. But at the same time, myxoid liposarcoma of soft tissues is not prone to the occurrence of metastases. In some cases, areas represented by poorly differentiated cells are found in the tumor. In this case, the prognosis is unfavorable.
Etiology
Currently, the causes of myxoid liposarcoma of the soft tissues of the thigh have not been established. In rare cases, the neoplasm is the result of malignancy of the lipoma. In this regard, doctors recommend removing large wen in a timely manner.
Despite the fact that the causes of the disease are unknown, doctors identify a number of provoking factors, under the influence of which the process of formation of liposarcoma can start. These include:
- Presence of neurofibromas. Often, the formation of a malignant neoplasm begins in adipose tissue next to a benign tumor, the development of which starts in the sheath of nerve fibers.
- All kinds of injuries.
- Regular body contact with carcinogenic compounds.
- Irradiation.
- Pathologies of bone structures, both congenital and acquired.
- Hereditary predisposition. The risk of developing the disease is higher in peoplewhose relatives suffered from cancer.
Liposarcoma of soft tissues poses a threat not only to he alth, but also to human life. It is able to grow into joints and bone structures, destroying them.
Clinical manifestations
At the initial stage of development of soft tissue liposarcoma, there are no symptoms. The first clinical manifestations occur when the tumor begins to grow in size. The neoplasm can be seen with the naked eye, it can reach 25 cm in diameter. On palpation, the tumor is felt as a dense knot with an uneven consistency. As a rule, the neoplasm is one, but multiple liposarcomas can also be observed.
Over time, the tumor grows into the surrounding soft tissues, bone structures, compresses the blood vessels and nerve fibers. This stage is characterized by the following symptoms:
- Painful sensations. In every fifth person with liposarcoma, the tumor grows into the bone structures and compresses the nerve endings. This explains the occurrence of high-intensity pain in the area of tumor localization.
- Deformation of limbs. Myxoid liposarcoma of the soft tissues of the thigh (a photo of the affected part of the body is presented below) negatively affects the joints. The latter begin to deform, the appearance of the lower limb also changes. For the same reason, patients are often diagnosed with thrombosis, edema, thrombophlebitis, ischemia.
- Violation of the sensitivity of the lower extremities. myxoidliposarcoma of the soft tissues of the thigh, as mentioned above, compresses and often damages the nerve fibers. Due to this, sensitivity is lost, paralysis and paresis occur.
- Deterioration of general well-being. In the later stages of the course of the disease, the following pathological conditions are detected in patients: signs of intoxication, muscle weakness, fever, a sharp decrease in body weight, loss of appetite. The skin in the area of liposarcoma becomes hot and becomes bluish.
Despite the fact that a myxoid-type tumor is not prone to metastasis, the possibility of distant secondary foci of pathology cannot be ruled out. Liposarcoma is a malignant neoplasm, and in all cases there is a risk of spreading its cells through the blood vessels.
Classification
Tumors are usually divided according to the degree of differentiation. This term refers to the process of structure formation, during which cells acquire the specific features of muscle, fat or any other tissue. When malignant, these characteristic features are erased.
Differentiation is denoted by the letter G. The value of G1 indicates that minor changes have occurred in the cells. In other words, the tumor does not behave aggressively at all. It grows extremely slowly in size and is not prone to metastasis. Such a neoplasm in its characteristics is very similar to a lipoma, which is a benign tumor.
Worst prognosishas G4 liposarcoma. This is an undifferentiated tumor, which is represented by cells that have completely lost their specific features.
As a rule, myxoid liposarcoma of soft tissues has a G3 score. This value indicates that the neoplasm has an average degree of malignancy. With myxoid liposarcoma of the soft tissues of the hip G3, the prognosis directly depends on the timeliness of the visit to the doctor.
Diagnosis
When the first warning signs appear, you need to contact an oncologist. During the appointment, the doctor will conduct an initial diagnosis, which consists in collecting an anamnesis and a physical examination. The specialist needs to provide information regarding all the symptoms present. As a rule, patients complain of swelling and pain in the tumor area.
To make an accurate diagnosis, the oncologist issues a referral for a comprehensive examination, including:
- General and biochemical blood and urine tests. Based on their results, the doctor is able to assess the degree of functioning of the kidneys, identify inflammatory processes, and also detect abnormalities in the hematopoietic system (which indicates damage to the bone marrow).
- X-ray. The study is carried out in order to assess the state of bone structures and identify the degree of damage. In addition, the doctor gets the opportunity to know the exact location and size of soft tissue liposarcoma.
- CT, MRI. These studies are carried out to assess the condition of soft tissues. In the course of their implementation, the doctor receives information,necessary for the choice of treatment tactics. Clinically significant is the indicator of the degree of spread of the oncological process.
- Radionuclide scanning. The essence of the method is to assess the condition of all the bones of the patient's skeleton. Scanning is done to detect metastases.
- Ultrasound.
- Cytological and histological examination.
The final stage is the differential diagnosis. Then, based on the results of all the studies, the doctor is determined with the tactics of managing the patient.
Surgical treatment
Myxoid liposarcoma requires an integrated approach. But in all cases, surgery is indispensable.
There are several methods of surgical treatment. The choice of the method of intervention is carried out by the doctor. The specialist takes into account not only the severity of the disease, but also the localization of the tumor.
At an early stage of development, the neoplasm is excised along with the surrounding tissues. As a rule, a layer 3 to 5 cm wide is cut off. At later stages, a radical resection is indicated, but with the preservation of the lower limb. Liposarcoma is removed not only along with the surrounding soft tissues, but also bone structures. In the presence of metastases, they are also excised. The final stage is the plastic surgery. The surgeon replaces the removed bone with a prosthesis.
If the lesion is extensive and a very large area of the bone is damaged, amputation of the lower limb is indicated. Severed leg in a mustare sent to the laboratory. This is necessary in order to assess the degree of radicalness of the surgical intervention.
At the very late stage of the development of pathology, the operation is not advisable. Severe patients are shown symptomatic therapy aimed at alleviating well-being. But there is no hope for a cure in this case. Such patients are admitted to the palliative unit.
Radiation and chemotherapy
Surgery is the main treatment for myxoid soft tissue liposarcoma. However, the effectiveness of the operation will be minimal if it is not supplemented with radiation and chemotherapy. This is due to the high risk of relapse.
Radiotherapy is given both before and after surgery. Before the intervention using this method, it is possible to stop the growth of the tumor. In this case, both the neoplasm itself and 2-3 cm of nearby tissues fall into the irradiation field.
After surgery, radiation therapy is indicated if for some reason the tumor was not completely removed or it had a high malignancy.
Chemotherapy is an auxiliary method of influencing a neoplasm. It is also indicated for patients with metastases. As a rule, doctors prescribe the following drugs: Prednisolone, Methotrexate, Vincristine, Adriamycin, Cyclophosphamide.
Currently, regional chemotherapy is considered to be the most effective. The classic treatment regimen is as follows: intravenousthe introduction of "Vincristine" (on the 1st and 8th day), "Doxorubicin" (on the first day). From the first to the fifth day, "Dacarbazine" is shown. If necessary, the oncologist makes adjustments to the treatment regimen. Medicines are prescribed strictly in accordance with individual he alth characteristics.
If, based on the results of the diagnosis, the doctor found out that the patient is not operable, the treatment tactics in this case include aggressive radiation and chemotherapy.
Forecast
The outcome of the disease directly depends on the degree of differentiation of the neoplasm. If the patient applied to a medical institution in a timely manner, and the tumor was detected at an early stage of development, the prognosis is favorable. 75% of patients have a five-year survival rate. Ten years, respectively, for 25%.
At a later stage of soft tissue liposarcoma, the prognosis is less favorable. In this case, the chances of even a five-year survival rate are 2 times less. If the radical operation was carried out in a timely manner, in addition, it was supplemented with radiation and chemotherapy, the prognosis is good. In 30% of cases, there is a complete recovery.
As for the children. For them, the prognosis is the most favorable. Five-year survival is observed in 90% of patients.
In conclusion
Myxoid soft tissue liposarcoma is a malignant neoplasm. Under the influence of various adverse factors, the process of tumor formation in the fatty layer starts. Over time, it increases in size and compressessurrounding tissues and nerve fibers. In addition, liposarcoma is able to grow into muscles and bone structures. The main treatment for the disease is surgery. It is complemented by radiation and chemotherapy. In extremely severe cases, palliative care is indicated.
