Synovial soft tissue sarcoma is a malignant lesion that develops from cells of the synovium, tendon and tendon sheaths. Such a neoplasm is not limited to the capsule, as a result of which it can grow into soft tissues and into hard bone structures.
In almost half of cases, synovial soft tissue sarcoma of the right ankle joint is diagnosed. Sometimes the tumor develops in the joints of the hands, forearms, in the neck and head area, and is difficult to treat.
What's in it?
In the structure of such a neoplasm are cystic cavities, necrosis and hemorrhage. The pathological formation has a soft structure, but its ability to harden and calcify is not excluded. On the cut, during visual inspection, the sarcoma resembles a fish fillet: it has a cavernous structure and is white in color. Inside the formation, a mucous discharge is observed, which looks like synovial fluid. from other malignantNeoplasms synovial soft tissue sarcoma differs in that it does not have a capsule.
This pathology is characterized by a rather aggressive course and rapid development. In most cases, it is not treatable and recurs in the next few years. Even with successful therapy, synovioma metastases can occur after 5-7 years in the lymph nodes, lung tissue or bones.
According to statistics, both men and women are equally affected by synovial sarcoma. Most often, such a tumor is diagnosed between the ages of 15 and 25, but this disease is considered rare - it is diagnosed in three people out of a million.
Reasons
The main causes that provoke the development of synovial soft tissue sarcoma are not known for certain. Nevertheless, some factors that can serve as an impetus for the onset of a malignant process have been identified as a separate group. These include:
- Hereditary predisposition.
- Ionizing radiation. Exposure of the body to radiation can provoke malignancy of cells in various tissues, for example, in bone.
- Injuries. Serious violations of the integrity of the joints sometimes cause oncological degeneration of cells.
- Chemicals. The influence of carcinogens is very dangerous and can cause a malignant process.
- Immunosuppressive therapy. The implementation of this type of treatment in certain cases leads to oncological diseases.
- Unhe althy lifestyle, bad habits.
Age of patients
Malignant synovioma is considered a disease of the young. Oncoprocess, according to doctors, in most cases is provoked by unfavorable heredity and the state of the environment. The risk group for sarcoma includes young people and adolescents living in disadvantaged ecological zones.
Symptoms of the disease
In the early stages of the malignant process, until the formation is large, clinical signs are not observed. With the progression of synovial soft tissue sarcoma, discomfort occurs in the joint area, limiting its motor function. The softer the structure of the tumor, the less pronounced the pain syndrome.
If at this stage the specialist palpates the pathological focus, he can note a tumor ranging in size from 2 to 15 cm. The oncological process has no boundaries, there is a weak mobility of the tumor, its dense or soft consistency. The skin over it characteristically protrudes, its color and temperature change.
Photo of soft tissue synovial sarcoma presented.
As the synovioma grows, it has a destructive effect on the affected tissues, they begin to collapse, the pain syndrome intensifies. The joint or limb ceases to function normally, there is a loss of sensitivity or numbness due to the pressure of the neoplasm on the nerve endings. If it has affected the neck or head, symptoms may occur, such as sensation of a foreign body when swallowing, disturbancesbreathing, voice change.
General symptoms of intoxication
In addition, the patient has general symptoms of oncological intoxication, which include:
- chronic weakness;
- subfebrile condition;
- exercise intolerance;
- weight loss.
With the development of metastases and the spread of cancer cells to regional lymph nodes, their increase in volume is noted.
Synovial sarcoma of the knee, lower leg and thigh
The malignant tumor that affected the knee joint is a non-epithelial oncological neoplasm of the secondary type. The main cause of the pathological process is metastases from neighboring lymph nodes or the hip joint. If areas of bone tissue are affected, the doctor diagnoses osteosarcoma, and if cartilage fragments are involved in the oncological process, chondrosarcoma.
When the tumor is localized in the cavity of the knee joint, the main symptom of the pathology is pain, which usually covers the entire lower limb. Against this background, the motor functions of the leg are impaired. If the tumor spreads outward, that is, it is localized closer to the skin, local swelling can be observed and the process can be diagnosed at the initial stage.
In case of damage to the ligamentous apparatus by sarcoma, the leg is deprived of all functionality, since the joint is completely destroyed. With tumors of large sizes, the blood flow in the tissues changes, an acute shortage occurs in the lower leg.oxygen and nutrients.
Synovial sarcoma of the soft tissues of the thigh can be formed from almost all of its structures. Most of these neoplasms at the initial stage have a similar picture with benign tumor processes. The bulk of the pathologies of this localization are bone cancer and soft tissue tumors.
Sarcoma of the soft tissues of the thigh is a rather rare pathology and most often affects men aged 30-60.
Varieties of synovial sarcoma
According to the tissue structure, this tumor is divided into:
- cellular, which is formed from cells of the glandular epithelium and consists of papillomatous and cystic structures;
- fibrous, which grows from fibers similar in nature to fibrosarcomas.
According to the morphological structure, the following types of sarcoma can be distinguished:
- alveolar;
- giant cell;
- fibrous;
- histoid;
- mixed;
- adenomatous.
WHO classification
According to the WHO classification, the tumor is divided into two types:
- Monophasic synovial soft tissue sarcoma, when the malignant process consists of large light and fusiform cells. Differentiation of the neoplasm is poorly expressed, which significantly complicates the diagnosis of the disease.
- Biphasic synovial soft tissue sarcoma, when the formation consists of synovial cells and has multiple cavities. Easily determined during diagnostic procedures.
The best prognosis for a patient is observed with the development of a biphasic synovioma.
Very rare is a clear cell fasciogenic synovioma. According to the main features, it has much in common with oncomeloma, and it is extremely difficult to diagnose it. The tumor affects the tendons and fascia and is characterized by a slow pathological process.
Stages of pathology
At the initial stage, the neoplasm does not exceed 5 cm and has a low degree of malignancy. The prognosis for survival is very favorable and amounts to 90%.
At the second stage, the tumor is larger than 5 cm, but can already affect blood vessels, nerve endings, regional lymph nodes and bone tissue.
At the third stage of this oncological process, metastases are observed in the lymph nodes.
At the fourth stage, the area of the oncological process cannot be measured. In this case, damage to important bone structures, blood vessels and nerves occurs. There is multiple metastasis. The survival prognosis of such patients is zero. How is synovial soft tissue sarcoma of the thigh or lower leg treated?
Treatment and prognosis
Therapy of synovioma in 70% is based on its resection. Tumors in large joints: the hip, shoulder or knee grow into the lymph nodes and main vessels, and therefore there are frequent relapses and metastases, so specialists resort to amputation of one or another limb.
BIn general, the treatment and prognosis of synovial soft tissue sarcoma depends on the stage of its development. At the first and second stages, the pathology is successfully treated and the prognosis for the survival of patients is the most favorable. At the third stage, with a successfully amputated limb and the absence of metastases, a 60% survival rate is predicted, while at the fourth stage, when the malignant process spreads throughout the body, the prognosis is extremely unfavorable.
Surgical treatment is carried out in the following ways:
- Local removal, which is feasible only at the first stage of the disease, when the examination confirmed the good quality of the tumor. Further tactics of therapy depend on the histological examination of the removed tissues and the determination of their malignancy. The recurrence of the pathology is up to 95%.
- Wide excision, which is performed with the capture of adjacent tissues with an area of approximately 5 cm. Relapses of synovial sarcoma in this case occur in 50%.
- Radical resection, in which the tumor is removed while preserving the organ, but bringing the surgical intervention closer to amputation. In such a case, as a rule, prosthetics are used, in particular, replacement of a joint or blood vessels, plastic surgery of nerve endings, bone resection. After the operation, all defects are hidden with the help of autodermoplasty. Skin flaps and muscle tissue grafting are also used. Process relapses occur in about 20% of cases.
- Amputation, which is performed in case of damage to the main vessel,the main nerve trunk, as well as with massive growth of the tumor in the tissues of the limb. The risk of recurrence in such a case is the lowest - 15%.
Using surgical treatment simultaneously with chemotherapy and radiation, there is a chance to save the organ in 80% of clinical situations. Removal of lymph nodes along with the focus of the pathological process is carried out provided that the study confirmed the fact of malignancy of their tissues.
Radiation therapy
Radiation therapy for synovioma is carried out by the following methods:
- Preoperative or neoadjuvant, which is necessary to encapsulate the neoplasm, reduce its size, increase the efficiency of the operation.
- Intraoperative, which reduces the risk of disease recurrence by 40%.
- Postoperative or adjuvant, which is used when it is impossible to carry out surgical treatment due to the neglect of the pathological process and the collapse of the tumor.
