Signs of bone sarcoma disease - information for patients

General information

Primary tumors of the skeletal system are rare. They are observed between the ages of 15 and 40 years, that is, children, adolescents and young people are more often affected. In 10% of cases, the disease is detected in people over 60 years of age - in them the tumor may be associated with radiation or develop against the background of benign diseases of the skeletal system. The term “bone cancer” is applied to various types of oncopathology of the skeletal system. Among malignant tumors of this system, the most common are osteogenic sarcoma (synonymous with osteosarcoma in 50-60% of cases), Ewing's sarcoma (the second most common type of tumor), chondrosarcoma , giant cell tumor , fibrosarcoma .
Secondary bone tumors (bone metastases) are much more common. The source of metastatic tumors are primary tumors of the lung, prostate, breast and thyroid gland. Bone sarcomas are a group of tumors of nonepithelial origin. In 80-90% of sarcomas, they are highly malignant tumors that are localized in different bones of the skeleton. However, the tubular bones most often affected are the femur and tibia. The tumor develops in the metaphyses (growth part) of long bones, especially in the area of ​​the knee joint. Men get sick more often than women. An increased risk of the disease is observed with genetic abnormalities: hereditary retinoblastoma and Li–Fraumeni syndrome .

Sarcoma of the knee joint

One of the very first and often dominant signs of sarcoma of the knee joint is local pain. With the rapid growth of the tumor (and the malignant course of sarcoma is most often aggressive), deformation of the knee joint appears, which can be distinguished visually: the appearance of a lump, redness, and then bluishness of the skin over the site of the lesion.

Impaired functionality increases as the tumor progresses: the range of movements decreases, the person begins to limp. Limiting physical activity brings only temporary relief. Sarcoma is characterized by constant pain, especially at night. As the malignant process spreads to the surrounding tissues, swelling increases, which often “drains” down along the blood, lymphatic vessels and nerves. Increasing pain and the inability to relieve it with non-narcotic analgesics indicate tumor growth.

Pathogenesis

Carcinogenesis is triggered under the influence of many factors, which include:

• radiation treatment for other cancers;
• suppression of tumor suppressor genes;
• prosthetics performed;
• hereditary diseases;
• complex chromosomal aberrations.

Frequent deletion, as well as loss of heterozygosity of 3q13, are specific aberrations for osteosarcomas. One of the tumor suppressor genes is localized in this region, which regulates cell proliferation and plays a major role in tumor pathogenesis. 3q12 deletion is associated with progression and poor prognosis.

An important morphological feature of bone tumors is the infiltrative nature of growth, or circular (encompasses the entire area of ​​the bone around the circumference). The tumor spreads through the bone marrow spaces and replaces them, destroys and surrounds the bone beams. Neoplastic cells are characterized by severe cellular atypia. The architecture of the newly formed bone determines the tumor potential.

Etiology and pathological anatomy

The etiological factors of synovial sarcoma, like almost all cancers, are not completely clear. Precancerous conditions (schwannoma, dysplasia of tissues adjacent to the tumor), constant severe trauma to the joints, and the presence of foreign bodies are described as the possible causes of this type of sarcoma.

On microscopic examination of a biopsy specimen, a typical finding will be spindle cells with glandular-like structures. In terms of its morphological characteristics, malignant synovioma is extremely diverse: under a microscope, between poorly differentiated round-shaped cells (or resembling epithelium), you can see cracks or cavities that are filled with mucus-like structures. With a predominance of papillary elements like synovial villi, the tumor is classified as fibrous. The neoplasm may have many glandular cells, and then it is called cellular synovial sarcoma. If we consider the WHO data, then its experts divide this tumor into monophasic spindle-shaped and biphasic histological subtypes. Determining the exact morphological variant of the neoplasm is important for further prognosis.

Classification

The most common malignant bone tumors are:

• osteogenic sarcoma;
• Ewing's sarcoma;
• chondrosarcoma;
• fibrosarcoma;
• parosteal sarcoma;
• fibrous histiocytoma;
• lymphosarcoma.

Bone sarcoma

The most common tumor develops from osteoblasts and in the process of neoplasm develops altered, atypical bone. This tumor is called differently - synonyms are osteogenic sarcoma, osteosarcoma, osteoidsarcoma, osteoblastosarcoma. Chondrosarcoma and fibrosarcoma are distinguished as independent forms.

Classic osteosarcoma is a highly malignant intraosseous tumor. If a tumor appears in an unchanged bone, it is a primary sarcoma; if it develops after irradiation or against the background of Paget’s disease , it is a secondary sarcoma. The tumor affects the metaphysis (growth zone) of the tubular bone of the arm or leg. Active growth processes occur in the metaphyses, so they are associated with a high risk of tumor transformation. The appearance of a tumor is associated with the rapid growth of bone tissue.

The peak incidence is observed in children aged 10-14 years. They are more likely to affect the tubular bones of the leg - osteosarcoma of the femur in the hip joint, as well as the bones of the knee joint (femur and tibia). When the femur is affected, a large lesion zone of 5-10 cm is revealed. Hemorrhages , necrosis and cystic cavities are found in the newly formed bone. The tumor gradually destroys the periosteum. Sarcoma of the hip occurs at any age, but is more common in young men.

It is characterized by aggressive growth and metastasizes early. Malignant formation of the knee joint in most cases is a secondary metastatic process and less often - as a primary process. In adults, osteosarcoma affects the spine and maxillofacial bones. This type of tumor rarely develops in the area of ​​the coccyx, shoulder girdle, ulna and humerus. Primary osteosarcoma of the ribs is less common, but secondary (metastatic) tumors predominantly affect the ribs, vertebrae and pelvic bones. Malignant transformation of Paget's disease to sarcoma occurs in 1% of cases and the peak incidence occurs at 60 years of age and older. Of all the bones, the jaw is the most commonly affected. Jaw cancer is a tumor with rapid aggressive growth and early metastases to the lungs (most often). There are isolated cases of metastases to the stomach and soft tissues. Damage to the lower jaw is somewhat more common than to the upper jaw (corresponding to 58% and 42%).

Hand bone cancer in most cases is metastatic. In the bones of the skull there are various types of primary sarcomas, as well as secondary metastatic tumors from the primary focus (uterus and prostate). Skull bone tumors cause headaches due to compression of the brain. Spinal cancer is characterized by active growth and rapid progression. Patients develop movement disorders: when the lumbar region is affected, the lower limbs and the functions of the pelvic and genital organs are affected. Cervical spine cancer is very dangerous and requires immediate treatment. When the cervical vertebrae are damaged, the functions of the arms and legs are affected.

Ewing's sarcoma

This type of tumor occurs in children and adolescents. Develops in bone tissue and soft tissues. It is aggressive and metastasizes quickly. There is a high mortality rate in the first year of diagnosis. If we consider damage to the inert system, then in 70% this type of tumor occurs in tubular bones, flat bones (scapula, ribs) and pelvic bones. Compared to osteosarcoma, the diaphyses of bones are more often affected. Many patients have genetic mutations that cause disruption of cell differentiation processes.

Chondrosarcoma

The neoplasm develops from cartilage tissue. Occurs in people over 40 years of age. The course of this type of cancer is difficult to predict. In some, chondrosarcoma develops slowly, while in others it develops very quickly and metastasizes . This type of tumor affects any bone, but the femur and pelvic bones are predominant. The tumor can also develop in flat bones (scapula, skull, ribs). Most often, the course of chondrosarcomas is slow and metastases appear late. The appearance of pain is characteristic, and when the pelvis is damaged, the nerve roots are involved, therefore, in addition to pain, the function of the pelvic organs will be impaired.

Fibrosarcoma

A rare tumor that develops from connective tissue. Tumor cells are located between the collagen of the connective tissue and look like a spindle. In most cases, fibrosarcoma appears after radiation treatment.

Classification of osteosarcomas according to the TNM system

• T0 - no data on the tumor process.
• T1 - formation no more than 8 cm.
• T2 - neoplasm larger than 8 cm.
• T3—tumor continuity is disrupted.

Involvement of lymph nodes (“N”) is rare, since bones do not have a lymphatic system. But if there are metastases to the nodes, this is a poor prognostic sign. Regional lymph nodes are nodes that correspond to the location of the primary tumor.

• N0 - no metastases in the lymph nodes.
• N1 - metastases are present.

Osteosarcoma is characterized by the development of hematogenous metastases, which in the diagnosis of o. By the time of diagnosis, 20% of patients already have small metastases in the lungs. Metastases can also be in the pleura, pericardium, bones, kidneys, and central nervous system.

• M0 - no distant metastases.
• M1 - distant metastases are present (“a” - to the lungs, “b” - to organs and tissues).

An important point is histological examination, which confirms the diagnosis, determines the type of tumor and its malignancy. There are five forms of osteosarcomas, with different degrees of differentiation.

Metastatic bone lesions

The course of metastatic bone lesions is not asymptomatic - pain is the first manifestation of bone metastases. Most patients describe it as unbearable. Patients with metastases also experience:

• pathological fractures of tubular bones;
• vertebral compression fractures;
• spinal cord compression;
• hypercalcemia.

A quarter of breast cancer have fractures of long bones associated with destruction of the cortical layer. radiation therapy or surgery is required .

Benign bone formations

Bones are affected by benign tumors ( chondroblastoma , fibrous dysplasia , osteoclastoma , aneurysmal cyst and others), with which a malignant formation must be differentiated.

Aneurysmal bone cyst

A benign tumor-like bone lesion, which is characterized by the proliferation of connective tissue. It contains cavities filled with blood. A distinctive feature of this cyst is its spread through the epiphyseal plate, as well as to adjacent bones. The cyst appears at any age, but more often in adolescents and children. Its most common location is the bones of the spine, the distal part of the femur, the proximal part of the humerus and tibia, and the pelvic bones. A secondary cyst is formed after damage to benign chondroblastoma , fibrous dysplasia , giant cell tumor and malignant neoplasms - osteogenic sarcoma .

Chondroblastoma

A benign tumor that forms at the articular ends of a bone. It develops from chondroblasts and resembles mature cartilage tissue. It is characterized by slow growth and a favorable course, but there is information about cases of a malignant course or malignancy by chondroblastoma. Like all bone tumors, it affects children and adolescents, and in rare cases, older people. Clinical manifestations are pain and swelling of the joint. Treatment is surgical. The prognosis is favorable.

Stages of soft tissue sarcoma

Immediately after the disease is detected, doctors try to stage it - to understand how far it has spread throughout the body and damaged tissue. This information allows us to understand the patient’s approximate prognosis and select the most appropriate treatment methods for him.

Staging of soft tissue sarcomas is carried out according to the international TNM

, each letter of which has its own meaning:

• " T
  " describes the size of the underlying neoplasm;
• “ N
  ” – damage to nearby lymph nodes;
• “ M
  ” - the presence of metastases - additional foci of the disease in other areas of the body.

Using " G"

"indicate:

• The degree of difference between tumor cells
  and healthy ones is from 1 to 3. One is assigned to cells that are most similar to normal tissues, and 3 is assigned to highly altered cells;
• The number of dividing cells
  is from 1 to 3. The fewer there are, the lower the score.
• Necrosis
  : a score from 0 to 2 is given to determine the amount of dying tissue - the higher the number, the more there is.

Then the total number of points is calculated: G1 – from 2 to 3; G2 – from 4 to 5; G3 – from 6 to 8.

Stages:
1A
: the size of the sarcoma does not exceed 10 centimeters, the remaining tissues are healthy.
1B
: the tumor may be larger than 15cm, but its cells are very similar to normal, and other organs are not affected.
2
: the tumor is no more than 5cm in diameter, but is graded G2 or G3.
3A
: sarcoma from 5 to 10 cm, G2 or G3.
3B
: 10 to 15 cm, G2 or G3, but other tissues are healthy.
Or, if the tumor is located in the abdomen, any size of the main lesion and damage to the nearest lymph nodes. 4
: a tumor of any diameter and type G, which has damaged not only nearby lymph nodes, but also distant tissues, such as the lungs.

Causes

The causes of bone tumors are not well understood. To a certain extent, bone cancer is caused by:

• Chronic diseases of the skeletal system ( osteomyelitis , Paget's disease , benign tumors ).
• Smoking.
• Injuries.
• Foreign body in the bone (orthopedic implants). Tumors of the skeletal system are more often detected in patients who have had implants used for osteosynthesis or have undergone joint replacement. Dentures use hexavalent chromium, which is a carcinogen.
• Previous radiation therapy , which induces the development of osteosarcoma. Radiation-induced sarcomas develop 3 years after irradiation.
• State of immunodeficiency .
• Skeletal abnormalities ( Olier disease ).
• Mutations of the p53 gene and the retinoblastoma Rb1 gene.
• Hereditary diseases ( retinoblastoma , Li-Fraumeni syndrome , Rothmund-Thompson syndrome , Werner syndrome , absent thumb).
• Benign bone tumors that can become malignant.
• Taking the drug Natpara (a drug for the treatment of hypoparathyroidism ). This drug increases the risk of osteosarcoma.

There is evidence of an increased risk of osteosarcoma in children born weighing more than 4500 g and above average height. However, in most patients no specific factors are identified.

About risk factors for the development of sarcomas

Most sarcomas develop for reasons unknown to scientists. However, some factors increase the risk of sarcoma formation. Thus, the development of pathology is associated with significant influences of ionizing and oncogenic substances, provoking the rapid growth of atypical, poorly differentiated structures. In addition, the pathogenic effect on the body of certain chemicals and viruses was established. Key risk factors include:

• radioactive exposure (the use of radiotherapy for various diseases, for example, breast cancer),
• exposure to chemicals (asbestos, wood preservatives, etc.),
• genetic failures (for example, children with a hereditary type of retinoblastoma are at significant risk of soft tissue sarcoma);
• previous bone diseases;
• disruptions in the functioning of the immune system;
• bone injury.

For the development of Ewing's sarcoma, the rate of bone growth and hormonal characteristics are of no small importance. Among the reasons that contribute to the formation of pathology are the presence of a history of benign formations (they can become malignant), mechanical injuries, inherited tendencies, and others.

Symptoms and manifestations of bone cancer

A characteristic manifestation of malignant bone formation of any location is constant, aching pain, disturbing for several weeks or months, not eliminated by analgesics and increasing over time.

For a long time, patients associate the appearance of pain with a former injury, so they do not consult a doctor and this becomes the reason for a late diagnosis. Pain from bone tumors does not depend on physical activity and bothers patients even at rest. Sarcoma is characterized by pain that worsens at night and relief does not occur in any position of the limb. Symptoms of osteosarcoma also include swelling of the tissue in the area of ​​the tumor. But local swelling appears later - at first it is a deeply located tumor in the soft tissues, and then the swelling area increases, the affected area swells, and a venous network appears on the skin, sometimes the skin turns red.

When joints are affected, swelling appears in their area. Cancer of the knee joint or any other joint disrupts the function of the joints - they bend poorly and movement is limited. Movements are impaired not only when there is a tumor in the joint, but also in the nearest joint when the bone is damaged. A tumor of the joints changes their shape. In some cases, effusion appears in the joint cavity.

Photo of osteosarcoma of the knee joint

Bone cancer is accompanied by fragility, so patients often experience spontaneous fractures due to minor trauma. When the tumor process affects the spine, difficulties appear when walking, weakness in the limbs, and paretic phenomena. A tumor of the leg bones is accompanied by numbness of the limb and lameness. Symptoms of advanced bone sarcoma include abdominal pain, nausea and vomiting, which is associated with increased levels of calcium in the blood ( hypercalcemia ) due to bone breakdown. In the advanced stage, general symptoms are also noted - fatigue and weight loss. The appearance of general symptoms indicates a process with metastasis.

Symptoms of jaw cancer

Osteosarcoma that occurs in the thickness of the jaw is called central, and that developing in the peripheral parts is called peripheral. For a long time, signs of bone cancer do not appear. Then pain appears that is not associated with tooth pathology. Long-lasting pain should alert the patient. The tumor process can be recognized using radiography and MRI. With a tumor formation in the lower or upper jaw, pain in the teeth occurs for no reason and radiates to the ear and eye. It gets worse at night and becomes unbearable, forcing patients to wake up and take painkillers.

Palpation of a tumor in any part of the jaw is painful. In addition, osteogenic sarcoma of the jaw changes the sensitivity of the trigeminal nerve and causes changes in tooth position. The teeth within the tumor become mobile. paresthesia and anesthesia occurs (Vincent's symptom).

Photo of sarcoma of the lower jaw

As the jaw grows, it becomes deformed. The tumor quickly grows, spreads to the surrounding tissues and compresses them, causing exophthalmos and congestion in the soft tissues. Lymph nodes are often not enlarged. The mucous membrane of the alveolar process at the site of the tumor is red and swollen, and when the mucous membrane is injured, ulcers appear on the gums. In some cases, osteosarcoma festeres (the temperature rises, weakness and malaise appear).

Clinical manifestations

Symptoms of synovial sarcoma begin with the onset of pain in the area of ​​the affected joint or soft tissue. If the tumor is dense and has calcifications, then the pain syndrome is more pronounced, and if it is cystic, the pain is not so noticeable. As the tumor progresses and surrounding tissues (vessels, synovial membranes, bone tissue) are involved in the oncological process, the pain increases. It is accompanied by a limitation in the range of both active and passive movements of the affected joint.

Upon palpation, a dense or soft (depending on the structure) formation is determined, sometimes without clear boundaries. The diameter of the tumor varies widely: from 2-3 to 20 cm. General symptoms characteristic of cancer of any location and origin increase quite quickly: periodic increase in body temperature, often to low-grade and febrile levels, malaise, weakness, loss of appetite, decreased weight.

Tests and diagnostics

• X-ray of the affected area in two projections. X-ray examination covers the entire affected bone and joints nearby. This is necessary in order not to lose sight of the “step-by-step” metastases. With osteosarcoma, the x-ray picture may be different. A large tumor node with unclear outlines. The cortex and medulla of the bone are affected. The tumor destroys the periosteum, often revealing a “cloud-shaped” component, which is associated with uneven ossification of the tumor. In the newly formed bone, osteosclerotic and osteolytic (sparse) areas alternate. However, areas of bone change become visible on x-ray when the tumor grows to a certain size. Therefore, to avoid diagnostic errors, you need to use MRI and CT.
• MRI and CT provide layer-by-layer visualization of bone, bone marrow, soft tissue and connective tissue. They are carried out with contrast. These methods clarify the size of the lesion, the exact location and spread of the tumor beyond the bone.
• To detect distant metastases, a CT scan of the chest and abdominal pelvis is performed. Chest X-ray also reveals metastases in the lungs.
• To identify lesions in other bones, skeletal scintigraphy (radionuclide scanning) is prescribed.
• The main method to confirm the disease is a biopsy and histological examination of the obtained material. The patient's bone tissue is collected and the type of tumor is determined. Tissue collection is carried out by excision of the bone, which makes it possible to obtain sufficient material. If the lymph nodes are enlarged, a biopsy (puncture) is also performed.
• Histological examination. To make a diagnosis, detection of neoplastic tissue (osteoid) is mandatory. The amount of osteoid is not important because it is different in different fields of view. Osteoid is produced by tumor cells and may be found adjacent to or surrounding the cells.
• Angiography. Detects vascular damage.
• Molecular genetic diagnostics. It determines the presence of mutations in the genome. This is necessary to determine the need for targeted therapy (biological).

Specific markers for osteosarcoma are currently unknown. Sometimes patients are prescribed a CEA test. Let's find out how necessary this is. Carcinoembryonic antigen , what is it? This is a tissue marker of cancer. What does this marker show? This type of protein is detected in minute quantities in a healthy person, but during the oncological process its level increases.

Determination of this antigen is used to diagnose the following tumors:

• colon and rectum;
• stomach;
• mammary gland;
• pancreas;
• lungs;
• metastases to the liver and bones (the sensitivity of the method in the metastatic process is much lower).

This test is not specific for malignant tumors, since an increase in antigen is observed in inflammatory and autoimmune diseases, benign tumors (intestines, liver, pancreas), liver cirrhosis , hepatitis , Crohn's disease , tuberculosis , pneumonia , cystic fibrosis . During a malignant process, the level increases throughout the entire period of the disease. CEA analysis is used to monitor the effectiveness of treatment of malignant neoplasms. A decrease in its level during the course of treatment indicates a positive result. The norm of carcinoembryonic antigen for non-smokers: 0-3.8 ng/ml, the norm for women who smoke is slightly higher than 0-5.5. A negative result does not exclude cancer.

Diagnosis of soft tissue sarcoma

Detailed information about the disease and the general state of a person’s health allows us to choose the most appropriate treatment for each individual patient.

Highly qualified oncology specialists carry out a complete diagnosis of soft tissue sarcomas using the most modern equipment - quickly, efficiently, without queues and loss of time.

The examination begins with a general examination and a survey about symptoms and family diseases, after which the doctor prescribes a series of tests.

The first of these may be conventional radiography.

. With its help, immediately after diagnosis, doctors can see whether the tumor has spread to the lungs.

– computed tomography: X-rays create detailed images of internal organs and tissues. The method is used to detect tumors in the chest or abdominal cavity, as well as to detect damage to the lungs, liver and other organs.

MRI

– Magnetic resonance imaging: radio waves and strong magnets often work better than CT scans. MRI can provide information about the size of the sarcoma, its location, and sometimes even the type of tissue it consists of. In addition, this study is prescribed to study the condition of the brain and spinal cord.

Ultrasound

Using ultrasound waves, a specialist can distinguish seals from ordinary cysts - fluid-filled sacs.

PAT

– positron emission tomography: before performing this study, the patient receives radioactive sugar, which accumulates in dangerous tumors. A scan is then performed to show whether changes in the tissue are cancerous.

If a soft tissue sarcoma is suspected, the doctor prescribes a biopsy

, during which a small piece of the tumor is removed and sent to a laboratory where its cells are carefully studied.

In children

Among all tumors in children, 5% are bone sarcomas : 3% osteosarcoma and 2% Ewing sarcoma . The lack of alertness among doctors and the atypical nature of the course greatly complicate early diagnosis. Osteosarcoma in children in half of the cases is localized in the knee joint (the femur and tibia are affected). In second place is damage to the metadiaphysis of the humerus. The most common symptom of osteosarcoma is constant, dull pain with a gradual increase. Most children have a soft tissue component - swelling and an increase in the volume of the limb. Weight loss and fever at the onset of the disease are rare.

Ewing's sarcoma occurs before the age of 17 years, but the peak incidence occurs between 12 and 15 years of age. Boys are most often affected. Ewing's sarcoma, unlike osteosarcoma, does not synthesize osteoid . Often this tumor is located in the scapula, ribs, vertebrae, pelvic bones, as well as the femur, fibula, and humerus. Unlike osteosarcoma, flat bones are affected. Signs of Ewing's sarcoma are increasing pain, tissue swelling, dysfunction, and fever. The soft tissue component is more pronounced than bone destruction. An increase in local temperature due to the appearance of hemorrhages and necrosis in the tumor complicates diagnosis, since osteomyelitis is suspected. Neurological symptoms are associated with nerve damage.

Combined treatment for Ewing's sarcoma:

• Chemotherapy courses (standard drugs for this pathology).
• Surgery (similar to osteosarcoma).
• Radiation therapy. It is carried out on the area of ​​the primary tumor and on distant metastases.

Treatment of soft tissue sarcoma

The fight against oncology is not an easy task, and requires the participation of not just one doctor, but a whole team of professionals.

The oncology department carries out all types of diagnostics and therapy of sarcomas using the most modern equipment. Our world-class specialists fully guide the patient and provide him with all the necessary assistance - quickly, efficiently, without queues or waste of time.

Each situation is unique and requires an individual approach and a combination of different techniques:

Surgery

, with which some tumors can be completely cured. The purpose of the operation is to remove the entire tumor and a certain amount of healthy tissue surrounding it - at least 1-2 centimeters, so that no altered cells remain in the human body. In the past, many patients with sarcomas on the arms and legs were treated with limb amputation. Today this method is rarely used, and only in some cases it is not possible to avoid this traumatic procedure. In addition, there are situations when, along with oncology, it is necessary to remove important nerves, muscles, bones and blood vessels.

Even if the disease has spread to distant parts of the body, such as the lungs or other organs, surgery can cure a person or prolong his life.

Radiation therapy

: Targeting the tumor with radioactive beams or particles is a key part of the fight against soft tissue sarcomas.
In most cases, this procedure is prescribed as an adjuvant
treatment after surgery.
With its help, specialists kill the altered cells remaining in the body and prevent the recurrence of the disease. Some patients require neoadjuvant
therapy, used before surgery to shrink the tumor and make it easier to remove. In addition, radiation can be used as a palliative - to relieve the symptoms of an advanced disease that cannot be cured.

There are several types of radiation therapy:

• classic external
  , in which the effect occurs externally, through the skin;
• intraoperative
  , which involves irradiating tissue after tumor removal, before closing the wound;
• brachytherapy - injection of small granules of radioactive material into areas damaged by sarcoma;
• and proton
  therapy, which uses streams of protons instead of X-rays - positively charged particles precisely aimed at the changed cells.

Chemotherapy

– the use of special drugs that kill tumor cells.
They are injected into a vein or taken orally as tablets and reach all areas of the body. Thanks to this property, the method is effective even at advanced stages of the disease. Depending on the type and stage of sarcoma, this procedure can be prescribed as the main or auxiliary treatment - in addition to surgery, and combined with radiation therapy. Some patients require a special type of chemotherapy called isolated limb perfusion
. During this procedure, a specialist separates the blood circulation of the affected arm or leg from the rest of the body, after which it injects the active substances.

Targeted therapy

– drugs that attack only certain components of altered cells and cause minimal harm to healthy ones.

Pazopanib

(
Votrient
): Blocks several enzymes important for cell growth and survival—proteins that initiate or speed up the chemical reactions needed to copy genetic information. It can be used to treat advanced soft tissue sarcomas that have not responded to chemotherapy. It slows tumor progression and eases side effects in patients whose tumors cannot be treated with surgery.

Tazemetostat (Tazverik

): affects a protein that promotes the growth of some cancer cells. It is used for epithelioid sarcomasEpithelioid sarcomas typically damage tissue in the hands, forearms, feet, or legs of adolescents and young adults that cannot be removed by surgery.

Diet

Dietary table No. 1a

• Efficacy: therapeutic effect after a week
• Terms: 3-10 days
• Cost of food: 900-1100 rubles per week

Diet table No. 5a

• Efficacy: therapeutic effect after 5-7 days
• Time frame: 2-6 weeks
• Cost of products: 1300 - 1400 rubles per week

Diet 11 table

• Efficacy: therapeutic effect after a month
• Terms: 2 months or more
• Cost of products: 1800-1900 rubles. in Week

of diarrhea while undergoing chemotherapy . In this regard, a diet excluding spicy, fatty foods containing fiber (vegetables, nuts, seeds, bran bread) is recommended.

Fried foods and simple carbohydrates, which increase intestinal motility, are also excluded. A balanced diet during this period should contain a sufficient amount of protein (boiled meat, fish, steamed cutlets, meat soufflé). Dishes must be pureed - soups, cereals, meat. During this period, you can use Diet No. 1B and No. 5P . When diarrhea disappears and the patient’s condition improves, the patient is transferred to Table No. 11 . The diet of this diet is suitable for the recovery period. The patient’s diet is complete, containing the necessary vitamins, nutrients and minerals. Daily calorie content is increased (3000 kcal). Food is taken in 5 divided doses. The goal of the diet is to normalize weight while reducing it and improve the function of all body systems.

All foods and cooking methods are allowed, but nevertheless, it is better to adhere to a healthy diet, in which preference is given to stewed, boiled or baked dishes, vegetable and cereal soups that are not fried. To replenish vitamins, the diet should contain fresh seasonal vegetables, fruits and berries. It is important to choose healthy and natural products that do not contain dyes, flavor enhancers or preservatives. The diet must contain dairy products: natural milk, kefir, cottage cheese, cheeses, yogurt. Dairy products are important for replenishing calcium, which is constantly lost from bones. The calorie content of the diet should not be increased by fatty foods, cakes, cream pies, semi-finished products, beef and lamb fat, fatty sausages, ready-made sauces, pastes, mayonnaise, and sweet carbonated water.

What kind of pathology

According to the WHO definition, synovial sarcoma is a mesenchymal spindle cell malignancy that may have a tissue component with epithelial differentiation and the presence of a specific chromosomal translocation. This term first appeared back in 1895: it was introduced by the German doctor G. Simon. Since then, several hundred cases have been described, which have brought synovial sarcoma into an independent nosology.

Despite its name, the origin of malignant synovioma from elements of the synovial membrane has not been confirmed. It is believed that this tumor is a variant of primary carcinosarcoma. Some scientists believe that it comes from immature mesenchymal cells. There is even evidence that the tumor may be of neuroectodermal origin (due to the similarity in the expression of many genes of synovial sarcoma with a malignant tumor originating from the sheaths of nerve fibers). Be that as it may, research into the origin of the cells of this neoplasm continues, and in the WHO classification it is in the section of tumors with unclear tissue differentiation.

Prevention

There is no specific prevention. However, you can perform non-specific activities:

• Observation and treatment of precancerous diseases of the skeletal system. Processes that tend to become malignant include chondroma, fibrous degeneration, Paget's disease, and osteochondral exostoses. The malignancy rate of these benign formations reaches 15%. It is important to undergo follow-up examinations annually to detect malignancy at an early stage. Healthy lifestyle. Balanced diet. Avoiding injuries and occupational hazards.

Secondary relapse prevention includes:

• The use of drugs that restore the immune system after chemotherapy and radiation therapy .
• Complete nutrition, rich in vitamins, antioxidants, proteins.
• Rejection of bad habits.
• Prevention of viral infections.
• Preventive examinations with an oncologist. Local relapses can be detected visually. It is important to have examinations during the first 4 years. After this, local relapses are rare.
• Carrying out diagnostic procedures according to the protocol. Metastases develop in 95% of cases within the first 5 years and most often affect the lungs. Bone metastases present with pain, and they appear simultaneously with pulmonary metastases.

Treatment of foot cancer

The following techniques have proven effectiveness in the fight against malignant neoplasms of the lower extremities:

• surgical method - removal of the affected area of ​​the foot or complete amputation of the limb, followed by prosthetics;
• chemotherapy - the introduction of special medications that suppress tumor growth, metastasis, and inflammation;
• Radiation therapy is a non-invasive method of treating cancer with ionizing radiation.

The main goal of therapy is to stop the growth of a malignant tumor without damaging healthy cells of surrounding tissues.

Consequences and complications

The course of the disease itself is complicated by fractures and infection of soft tissues. All bone cancers after treatment have consequences:

• Secondary cancer , which develops as a result of exposure to radiation.
• Chronic heart failure (exposure to doxorubicin ).
• Osteoporosis.
• Hearing and vision impairments.
• Renal dysfunction and renal failure .
• Endocrinopathies.
• Impaired fertility ( infertility ). Reproductive functions suffer after chemotherapy with alkylating drugs ( Ifosfamide , Carboplatin , Cyclophosphamide ) and after the use of radiotherapy in the pelvis, abdomen, and spine. Menstrual irregularities are always observed after high-dose chemotherapy and stem cell transplantation.
• The consequences of the operation are significant - after amputation, limb prosthetics and long-term rehabilitation are necessary.

Diagnostic methods

Since the onset of synovial sarcoma can be disguised as rheumatological diseases of the joints, it is important to conduct a full range of instrumental and laboratory studies. Radiation diagnostic methods (x-ray examination in at least two projections and computed tomography) make it possible to identify foci of tumor formation and the presence of calcifications, which are often located in the central part of the sarcoma during its long-term course. In many cases, X-ray images reveal destructive processes in the adjacent bone tissue.

Angiography shows pathological proliferation of blood vessels in the affected area. Radioisotope research with strontium can help determine the exact location of the neoplasm and its boundaries, which is important for determining the scope of organ-preserving surgery.

A biopsy of the lesion will help differentiate synovial sarcoma not only from other tumors and non-oncological diseases, but also to determine the histological subtype of cancer.

Forecast

The prognosis of osteosarcoma is determined by many factors: age, size, localization, stage, “purity” of the resection margin, histological conclusion.

How long do people live with bone cancer? With complex treatment of osteosarcoma, 70% of patients live 5 years or more, with Ewing's sarcoma - 60% of patients.

Some authors give a higher survival rate. If there is a localized distal lesion, radical resection is performed and the result of chemotherapy treatment is more than 90% tumor necrosis, then 5-year survival is observed in 80% of cases.

The prognosis is worse in case of proximal localization, large tumor size, poor response to chemotherapy before surgery and metastases. Poor prognosis for tumors in the pelvic bones and weight loss at diagnosis. It is known that the intensity of tumor cell apoptosis also affects prognosis. Also unfavorable factors are early relapse, multiple lesions in the lungs and pleural involvement. If patients have stage 4, how long do they live in this case? When removing metastases in the lungs, 12-20% occur; if metastasectomy is not performed, 2.5% of patients survive. Life spans are calculated in months.

Prognosis and survival of soft tissue sarcoma

Each person's outlook is different and depends on a variety of factors, such as age, general health, type of disease, stage, location of the tumor, and response to treatment.

To present approximate forecasts, doctors use a special term - “five-year survival rate”. This statistic says nothing about a person's chances - it only shows the percentage of people still alive 5 or more years after diagnosis.

For soft tissue sarcoma the numbers look like this:

• at localized stages, before the tumor has time to spread beyond the area in which it first appeared: approximately 81%;
• at regional stages, with damage to nearby tissues or nearby lymph nodes: about 56%;
• when the disease spreads to distant parts of the body such as the lungs: 15%.

List of sources

• Clinical guidelines for bone sarcomas, 2021, 51 p.
• Practical recommendations for the treatment of primary malignant bone tumors (osteosarcoma, Ewing's sarcoma) / Malignant tumors. Russian Society of Clinical Oncology. 2021, pp. 283-295.
• Clinical guidelines for the diagnosis and treatment of patients with primary malignant bone tumors. 2014, 25 p.
• Clinical protocol for diagnosis and treatment. Bone sarcoma, 2015, 19 p.
• Belokon O.V., Nechval V.N. Surgical treatment of children with osteogenic sarcoma with metastases to the lungs/Health of Ukraine, 2012, pp. 34-35.

Resources

The Kristen Ann Carr Fund www.sarcoma.com The Kristen Ann Carr Fund is a resource for people diagnosed with soft tissue sarcoma. Every year the foundation prepares a newsletter “Up-to-date information on the problem of sarcoma.” You can find more information about soft tissue sarcoma on the foundation's website.

Cancer.Net www.cancer.net/cancer-types/sarcoma-soft-tissue/introduction Cancer.Net provides information about soft tissue sarcoma, including videos and blog posts.
This website also has a Spanish version. Visit the website for more information. to come back to the beginning

Genetic causes and inheritance of angiosarcoma

Recent studies have identified associations of AS with chromosomal abnormalities, as well as hereditary genetic diseases. In this condition, there are two possibilities for common chromosomal abnormalities:

• An increase in the normal number of two in each of chromosomes 5, 8 and 20.
• Loss of the number of chromosomes 7 to 22 and the male sex chromosome Y.

For example, in breast AS, an increase in chromosome 8 is more common than any other genetic abnormality.

Genetic diseases believed to be associated with AS include:

• Klippel-Trenaune syndrome
• Von Hippel–Lindau disease
• Neurofibromatosis von Recklinghausen

Reports of several individual case studies also suggest that AS can occur among immediate family members without any identifiable cause. This highlights the possibility of the existence of a rare variant of AS that is hereditary, or of probable but as yet unknown exposure to an agent of this condition among family members.