The term "soft tissue liposarcoma" refers to a neoplasm of a malignant nature, which, under the influence of various adverse factors, begins to form in the fatty layer. According to statistics, the disease is most often diagnosed in men aged 50 to 60 years. In most cases, soft tissue liposarcoma forms in the femoral zone. More rarely, it is localized on the buttocks and retroperitoneal space. In isolated cases, the tumor forms in other parts of the body, including in the internal organs. Currently, the only treatment for soft tissue liposarcoma is surgery. Chemotherapy and radiation therapy are prescribed as supportive.
Pathogenesis
Under the influence of various adverse factors, a neoplasm begins to form in the adipose tissue. The basis of the tumor is immature connective 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 borders 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 relapse, but metastases are not characteristic. The tumor is represented by mature cells and has a favorable prognosis.
- Myxoid. Soft tissue liposarcoma (photo of the neoplasm is presented below) consists of mature cells and lipoblasts. It is usually localized on the hips.
- Pleomorphic. Contain cells of a round or fusiform shape. Pleomorphic soft tissue liposarcoma is a low-grade species.
- Squamous. Consist of cells that are not like fat cells.
- Undifferentiated. They contain two components that can be traced in different places of the neoplasm.
Tumor density is uneven. The growth of the neoplasm can lead to serious consequences, as a result of which it is necessary to consult a doctor if the first alarming signs occur.
Features of myxoid soft tissue liposarcoma
Most often, the tumor is localized in the proximal lower extremities. A neoplasm is a single node that has an irregular shape.
Myxoid soft tissue liposarcoma (a tumor photo is shown schematically below) is represented by mature fat cells as well as spindle-shaped and round lipoblasts. For this tumor, the presence of a mucoid stroma is characteristic. Liposarcoma has a large number of blood vessels.
This type of tumor very often recurs. But at the same time, myxoid liposarcoma of soft tissues is not prone to the occurrence of metastases. In some cases, areas represented by low-grade cells are found in the tumor. In this case, the prognosis is unfavorable.
Etiology
Currently, the reasons for the development 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 timely removal of large adipose tissue.
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 liposarcoma formation can start. These include:
- The presence of neurofibromas. Often, the formation of a malignant neoplasm begins in the adipose tissue next to a benign tumor, the development of which starts in the nerve sheath.
- Various kinds of injuries.
- Regular contact of the body with carcinogenic compounds.
- Irradiation.
- Pathologies of bone structures of both congenital and acquired nature.
- Hereditary predisposition. The risk of developing the disease is higher in individuals whose relatives suffered from oncology.
Soft tissue liposarcoma is a threat not only to health, 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, in diameter it can reach 25 cm. On palpation, the tumor feels like a dense node with an uneven consistency. As a rule, the neoplasm is one, but multiple liposarcomas can be observed.
Over time, the tumor grows into the surrounding soft tissues, bone structures, compresses blood vessels and nerve fibers. The following symptoms are typical for this stage:
- 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 localization of the neoplasm.
- Limb deformity. 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. Myxoid liposarcoma of the soft tissues of the thigh, as mentioned above, compresses and often damages nerve fibers. Due to this, sensitivity is lost, paralysis and paresis occur.
- Deterioration of general well-being. In the later stages of the disease, the following pathological conditions are revealed 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 acquires a bluish tint.
Despite the fact that the myxoid type tumor is not prone to metastasis, the probability of the appearance of distant secondary foci of pathology cannot be ruled out. Liposarcoma is a malignant neoplasm, and in all cases there is a risk of its cells spreading 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 specific characteristics of muscle, adipose, or some other tissue. With malignancy, these characteristic features are erased.
Differentiation is indicated by the letter G. The value of G1 indicates that minor changes have occurred in the cells. In other words, the tumor is not at all aggressive. It extremely slowly increases 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.
The most unfavorable prognosis is liposarcoma G4. This is an undifferentiated tumor, which is represented by cells that have completely lost their specific features.
As a rule, G3 has myxoid soft tissue liposarcoma. This value indicates that the neoplasm has an average degree of malignancy. With myxoid liposarcoma of the soft tissues of the thigh G3, the prognosis directly depends on the timeliness of contacting a doctor.
Diagnostics
If the first alarming signs occur, you need to contact an oncologist. During the admission, the doctor will conduct the initial diagnosis, which consists in collecting an anamnesis and physical examination. The specialist must provide information on all available symptoms. As a rule, patients complain of swelling and pain in the neoplasm.
To make an accurate diagnosis, the oncologist draws up a referral for a comprehensive examination, including:
- General and biochemical blood and urine tests. Based on their results, the doctor gets the opportunity to assess the degree of kidney function, identify inflammatory processes, as well as detect disorders in the hematopoiesis system (which indicates bone marrow damage).
- Roentgenography. The study is conducted to assess the state of bone structures and identify the degree of damage. In addition, the doctor gets the opportunity to find out the exact location and size of soft tissue liposarcoma.
- CT, MRI. These studies are conducted to assess the state of soft tissues. In the process of their conduct, the doctor receives the information that is necessary for choosing treatment tactics. Clinically significant is an indicator of the degree of spread of the oncological process.
- Radionuclide scan. The essence of the method is to assess the condition of all bones of the skeleton of the patient. Scanning is performed to detect metastases.
- Ultrasound
- Cytological and histological studies.
The final stage is a differential diagnosis. Then, based on the results of all the studies conducted, the doctor is determined with the tactics of patient management.
Surgical treatment
Myxoid liposarcoma requires an integrated approach. But in all cases, you can not do without surgical intervention.
There are several methods of surgical treatment. The choice of method of intervention is carried out by the doctor. The specialist takes into account not only the severity of the disease, but also the location of the tumor.
At an early stage of development, the neoplasm is excised along with the surrounding tissues. As a rule, a layer of 3 to 5 cm wide is cut off. At later stages, a radical resection is shown, but with the preservation of the lower limb. Liposarcoma is removed not only with the surrounding soft tissues, but also with bone structures. In the presence of metastases, they are also excised. The final stage is plastic surgery. The surgeon replaces the removed bone with a prosthesis.
If the lesion is extensive and a very large area of bone is damaged, amputation of the lower limb is indicated. The cut off leg is without fail sent to the laboratory. This is necessary in order to assess the degree of radicality of the surgical intervention.
At the latest stage of the development of pathology, the operation is impractical. Severe patients are shown symptomatic therapy aimed at improving well-being. But there is no hope of cure in this case. Such patients are hospitalized in the palliative department.
Radiation and chemotherapy
Surgery is the main treatment for myxoid soft tissue liposarcoma. However, the effectiveness of the operation will be minimal, if not supplemented by radiation and chemotherapy. This is due to the high risk of relapse.
Radiation therapy is carried out both before and after surgery. Prior to intervention using this method, it is possible to achieve tumor arrest. 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: Prednisone, Methotrexate, Vincristine, Adriamycin, Cyclophosphamide.
Currently, regional chemotherapy is considered the most effective. The classical treatment regimen is as follows: intravenous administration of "Vincristine" (on the 1st and 8th day), "Doxorubicin" (on the first day). From the first to the fifth day, Dacarbazine is indicated. If necessary, the oncologist makes adjustments to the treatment regimen. Medicines are prescribed strictly in accordance with individual health features.
If, based on the results of the diagnosis, the doctor finds that the patient is not operable, the treatment tactics in this case involve aggressive radiation and chemotherapy.
Forecast
The outcome of the disease directly depends on the degree of differentiation of the neoplasm. If the patient timely contacted a medical institution, and the tumor was detected at an early stage of development, the prognosis is favorable. In 75% of patients, five-year survival is observed. Ten-year, respectively, in 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 are 2 times less. If the radical operation was carried out in a timely manner, in addition, it was supplemented by radiation and chemotherapy, the prognosis is good. In 30% of cases, complete recovery is observed.
As for the children. For them, the forecast is the most favorable. 90% of patients have a five-year survival rate.
Finally
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 compresses the surrounding tissues and nerve fibers. In addition, liposarcoma can grow into muscles and bone structures. The main treatment for the disease is surgery. It is supplemented by radiation and chemotherapy. In extremely severe cases, palliative treatment is indicated.