Abdominal wall sarcoma, ICD code 10. Soft tissue liposarcoma: highly differentiated, pleomorphic, myxoid

All iLive content is reviewed by medical experts to ensure it is as accurate and factual as possible.

We have strict sourcing guidelines and only cite reputable websites, academic research institutes and, where possible, proven medical research. Note that the numbers in brackets (, etc.) are clickable links to such studies.

If you believe any of our content is inaccurate, outdated, or otherwise questionable, please select it and press Ctrl + Enter.

Sarcoma is a disease that involves malignant neoplasms of various localization. Let's look at the main types of sarcoma, the symptoms of the disease, methods of treatment and prevention.

Sarcoma is a group malignant neoplasms. The disease begins with damage to the primary connective cells. Due to histological and morphological changes, begins to develop malignancy, which contains elements of cells, vessels, muscles, tendons and other things. Among all forms of sarcoma, especially malignant ones account for about 15% of neoplasms.

The main symptomatology of the disease manifests itself in the form of swelling of any part of the body or node. Sarcoma affects: smooth and striated muscle tissue, bones, nervous, adipose and fibrous tissue. Methods of diagnosis and methods of treatment depend on the type of disease. The most common types of sarcoma:

• Sarcoma of the trunk, soft tissues of the extremities.
• Sarcoma of bones, neck and head.
• Retroperitoneal sarcomas, muscle and tendon lesions.

Sarcoma affects connective and soft tissues. In 60% of the disease, the tumor develops on the upper and lower limbs, in 30% of the trunk and only in rare cases, sarcoma affects the tissues of the neck and head. The disease occurs in both adults and children. At the same time, about 15% of the case of sarcoma is cancer diseases. Many oncologists consider sarcoma a rare type of cancer that requires special treatment. There are many names this disease. The names depend on the fabric in which they appear. Bone sarcoma is osteosarcoma, cartilage sarcoma is chondrosarcoma, and smooth muscle tissue damage is leiomyosarcoma.

ICD-10 code

Sarcoma mkb 10 is the classification of the disease according to the tenth revision of the international catalog of diseases.

Code according to the international classification of diseases ICD-10:

• C45 Mesothelioma.
• C46 Kaposi's sarcoma.
• C47 Malignant neoplasm of peripheral nerves and autonomic nervous system.
• C48 Malignant neoplasm of the retroperitoneal space and peritoneum.
• C49 Malignant neoplasm of other types of connective and soft tissues.

Each item has its own classification. Let's look at what each of the categories of the international classification of diseases of sarcoma ICD-10 means:

• Mesothelioma is a malignant neoplasm originating from the mesothelium. Most often affects the pleura, peritoneum and pericardium.
• Kaposi's sarcoma - a tumor that develops from blood vessels. A feature of the neoplasm is the appearance on the skin of red-brown spots with pronounced edges. The disease is malignant, therefore it poses a threat to human life.
• Malignant neoplasm of peripheral nerves and autonomic nervous system - this category includes lesions and diseases of peripheral nerves, lower extremities, head, neck, face, chest, hip area.
• Malignant neoplasm of the retroperitoneal space and peritoneum - soft tissue sarcomas that affect the peritoneum and retroperitoneal space, cause thickening of parts of the abdominal cavity.
• A malignant neoplasm of other types of connective and soft tissues - sarcoma affects soft tissues on any part of the body, provoking the appearance of a cancerous tumor.

ICD-10 code

C45-C49 Malignant neoplasms of mesothelial and soft tissues

Causes of sarcoma

The causes of sarcoma are varied. The disease may occur due to the influence of factors environment, injuries, genetic factors and much more. Specifying the cause of the development of sarcoma is simply impossible. But, there are several risk factors and causes that most often provoke the development of the disease.

• Hereditary predisposition and genetic syndromes (retinoblastoma, Gardner's syndrome, Werner's syndrome, neurofibromatosis, pigmented basal cell multiple skin cancer syndrome).
• Influence of ionizing radiation - tissues exposed to radiation are subject to infection. The risk of developing a malignant tumor increases by 50%.
• The herpes virus is one of the factors in the development of Kaposi's sarcoma.
• Lymphostasis of the upper extremities (chronic form), which develops against the background of radial mastectomy.
• Injuries, wounds, suppuration, impact foreign bodies(fragments, chips, etc.).
• Polychemotherapy and immunosuppressive therapy. Sarcomas appear in 10% of patients who have undergone this type of therapy, as well as in 75% after organ transplant operations.

, , , , , , ,

Sarcoma symptoms

The symptoms of sarcoma are diverse and depend on the location of the tumor, its biological characteristics and the underlying cells. In most cases, the initial symptom of sarcoma is a neoplasm that gradually increases in size. So, if a patient has bone sarcoma, that is, osteosarcoma, then the first sign of the disease is terrible pain in the area of ​​\u200b\u200bbones that occur at night and are not stopped by analgesics. In the process of tumor growth, neighboring organs and tissues are involved in the pathological process, which leads to a variety of painful symptoms.

• Some types of sarcomas (bone sarcoma, parosteal sarcoma) develop very slowly and are asymptomatic for many years.
• But rhabdomyosarcoma is characterized by rapid growth, spread of the tumor to neighboring tissues and early metastasis, which occurs by the hematogenous route.
• Liposarcoma and other types of sarcomas are primary-multiple in nature, sequentially or simultaneously manifesting themselves in different places, which complicates the issue of metastasis.
• Soft tissue sarcoma affects surrounding tissues and organs (bones, skin, blood vessels). The first sign of soft tissue sarcoma is a tumor without limited outlines, causing pain on palpation.
• With lymphoid sarcoma, there is a tumor in the form of a node and a small swelling in the area of ​​​​the lymph node. The neoplasm has an oval or round shape and does not cause pain. The size of the tumor can be from 2 to 30 centimeters.

Depending on the type of sarcoma, it may appear fever. If the neoplasm progresses rapidly, then subcutaneous veins appear on the surface of the skin, the tumor acquires a cyanotic color, and manifestations may appear on the skin. On palpation of the sarcoma, the mobility of the tumor is limited. If the sarcoma appears on the limbs, then this can lead to their deformation.

Sarcoma in children

Sarcoma in children is a series of malignant tumors that affect the organs and systems of the child's body. Most often, children are diagnosed with acute leukemia, that is, a malignant lesion of the bone marrow and circulatory system. In second place in terms of the frequency of diseases are lymphosarcomas and lymphogranulomatosis, tumors in the central nervous system, osteosarcomas, soft tissue sarcomas, tumors of the liver, stomach, esophagus and other organs.

Sarcomas in pediatric patients occur for several reasons. First of all, it is a genetic predisposition and heredity. In second place are mutations in the child's body, injuries and injuries, previous diseases and a weakened immune system. Sarcomas are diagnosed in children, as well as in adults. To do this, they resort to the methods of computer and magnetic resonance imaging, ultrasound, biopsy, cytological and histological examination.

Treatment of sarcoma in children depends on the location of the neoplasm, the stage of the tumor, its size, the presence of metastases, the age of the child and the general condition of the body. For treatment, surgical methods of removing tumors, chemotherapy and radiation exposure are used.

• Malignant diseases of the lymph nodes

Malignant diseases of the lymph nodes are the third most common disease that occurs in both children and adults. Most often, oncologists diagnose lymphogranulomatosis, lymphomas, lymphosarcomas. All these diseases are similar in their malignancy and the substrate of the lesion. But there are some differences between them, clinical course disease, treatment and prognosis.

• Lymphogranulomatosis

Tumors affecting the neck The lymph nodes, in 90% of cases. Most often, this disease affects children under the age of 10 years. This is due to the fact that at this age in the lymphatic system there are serious changes at the physiological level. Lymph nodes become very vulnerable to irritants and viruses that cause certain diseases. With a tumor disease, the lymph nodes increase in size, but are absolutely painless on palpation, the skin over the tumor does not change color.

For the diagnosis of lymphogranulomatosis, a puncture is used and tissues are sent to cytological examination. Lymph node cancer is treated with radiation and chemotherapy.

• Lymphosarcoma

A malignant disease that occurs in the lymphatic tissues. In its course, symptoms and tumor growth rate, lymphosarcoma is similar to acute leukemia. Most often, the neoplasm appears in the abdominal cavity, mediastinum, that is, the chest cavity, in the nasopharynx and peripheral lymph nodes (cervical, inguinal, axillary). Less commonly, the disease affects the bones, soft tissues, skin, and internal organs.

Symptoms of lymphosarcoma resemble those of a viral or inflammatory disease. The patient develops a cough, fever and general ailments. With the progression of sarcoma, the patient complains of swelling of the face, shortness of breath. The disease is diagnosed by X-ray or ultrasound. Treatment can be surgical, chemotherapy and radiation.

• Tumors of the kidneys

Kidney tumors are malignant neoplasms that, as a rule, are congenital in nature and appear in patients during early age. The true causes of kidney tumors are unknown. Sarcomas, leiomyosarcomas, myxosarcomas appear on the kidneys. Tumors can be round cell carcinomas, lymphomas, or myosarcomas. Most often, the kidneys affect fusiform, round cell and mixed types of sarcomas. Wherein mixed type, is considered the most malignant. In adult patients, kidney tumors rarely metastasize, but can be large. And in pediatric patients, tumors metastasize, affecting surrounding tissues.

For the treatment of kidney tumors, as a rule, surgical methods of treatment are used. Let's look at some of them.

• Radical nephrectomy - the doctor makes an incision in the abdominal cavity and removes the affected kidney and the fatty tissues surrounding it, the adrenal glands that are adjacent to the affected kidney, and regional lymph nodes. The operation is carried out under general anesthesia. The main indications for nephrectomy: a large size of a malignant tumor, metastasis to regional lymph nodes.
• Laparoscopic surgery - the advantages of this treatment method are obvious: minimally invasiveness, a short recovery period after surgery, less pronounced postoperative pain syndrome and a better aesthetic result. During the operation, several small punctures are made in the skin of the abdomen, through which a video camera is inserted, thin surgical instruments are inserted and the abdominal cavity is pumped with air to remove blood and excess tissues from the operation area.
• Ablation and thermal ablation is the most gentle method of removing kidney tumors. The neoplasm is affected by low or high temperatures, which leads to the destruction of the tumor of the kidney. Main types given treatment: thermal (laser, microwave, ultrasonic), chemical (ethanol injections, electrochemical lysis).

Types of sarcoma

Types of sarcoma depend on the location of the disease. Depending on the type of tumor, certain diagnostic and therapeutic techniques are used. Let's look at the main types of sarcoma:

1. Sarcoma of the head, neck, bones.
2. Retroperitoneal neoplasms.
3. Sarcoma of the uterus and mammary glands.
4. Gastrointestinal stromal tumors.
5. Damage to the soft tissues of the limbs and trunk.
6. Desmoid fibromatosis.

Sarcomas arising from hard bone tissue:

• Ewing's sarcoma.
• Sarcoma parosteal.
• Osteosarcoma.
• Chondrosarcoma.
• Reticulosarcoma.

Sarcomas arising from muscle, adipose and soft tissue:

• Kaposi's sarcoma.
• Fibrosarcoma and skin sarcoma.
• Liposarcoma.
• Soft tissue and fibrous histiocytoma.
• Synovial sarcoma and dermatofibrosarcoma.
• Neurogenic sarcoma, neurofibrosarcoma, rhabdomyosarcoma.
• Lymphangiosarcoma.
• Sarcomas of internal organs.

The group of sarcomas consists of more than 70 different varieties of the disease. Sarcoma is also distinguished by malignancy:

• G1 - low degree.
• G2 - average degree.
• G3 - high and extremely high degree.

Let's take a closer look at certain types of sarcoma that require special attention:

• Alveolar sarcoma - most often occurs in children and adolescents. It rarely metastasizes and is a rare type of tumor.
• Angiosarcoma - affects the vessels of the skin and develops from blood vessels. Occurs in the internal organs, often after exposure.
• Dermatofibrosarcoma is a type of histiocytoma. It is a malignant tumor that arises from the connective tissue. Most often affects the trunk, grows very slowly.
• Extracellular chondrosarcoma is a rare tumor that arises from cartilage, localizes in cartilage and grows into bone.
• Hemangiopericytoma is a malignant tumor of the blood vessels. It has the appearance of nodes and most often affects patients under 20 years of age.
• Mesenchymoma is a malignant tumor that grows from vascular and adipose tissue. Affects the abdominal cavity.
• Fibrous histiocytoma is a malignant tumor localized on the extremities and closer to the trunk.
• Schwannoma is a malignant tumor that affects the sheaths of the nerves. It develops independently, rarely gives metastases, affects deep tissues.
• Neurofibrosarcoma - develops from Schwann tumors around the processes of neurons.
• Leiomyosarcoma - appears from the rudiments of smooth muscle tissue. It spreads rapidly throughout the body and is an aggressive tumor.
• Liposarcoma - arises from adipose tissue, localized on the trunk and lower extremities.
• Lymphangiosarcoma - affects the lymphatic vessels, most often occurs in women who have undergone a mastectomy.
• Rhabdomyosarcoma - arises from striated muscles, develops in both adults and children.

• Kaposi's sarcoma is usually caused by the herpes virus. Often occurs in patients taking immunosuppressants and HIV-infected. The tumor develops from the dura mater, hollow and parenchymal internal organs.
• Fibrosarcoma - occurs on the ligaments and muscle tendons. Very often it affects the feet, less often the head. The tumor is accompanied by ulcers and actively metastasizes.
• Epithelioid sarcoma - affects the peripheral parts of the limbs, in young patients. The disease actively metastasizes.
• Synovial sarcoma - occurs in the articular cartilage and near the joints. It can develop from the synovial membranes of the muscles of the vagina, and spread to the bone tissue. Due to this type of sarcoma, the patient has reduced motor activity. Most often occurs in patients aged 15-50 years.

Stromal sarcoma

Stromal sarcoma is a malignant tumor that affects the internal organs. Typically, stromal sarcoma affects the uterus, but this disease a rare event found in 3-5% of women. The only difference between sarcoma and uterine cancer is the course of the disease, the process of metastasis and treatment. A suggestive sign of the appearance of sarcoma is the passage of a course radiotherapy for the treatment of pathologies in the pelvic area.

Stromal sarcoma is predominantly diagnosed in patients aged 40-50 years, while during menopause, sarcoma occurs in 30% of women. The main symptoms of the disease appear as spotting from the genital tract. Sarcoma causes pain due to an increase in the uterus and squeezing of its neighboring organs. In rare cases, stromal sarcoma is asymptomatic and can only be recognized after a visit to a gynecologist.

Spindle cell sarcoma

Spindle cell sarcoma is made up of spindle cells. In some cases, during histological examination, this type of sarcoma is confused with fibroma. Tumor nodes have a dense texture, when cut, a fibrous structure of a white-gray color is visible. Spindle cell sarcoma appears on the mucous membranes, skin, serous integument and fascia.

Tumor cells grow randomly alone or in bunches. They are located in various directions relative to each other, intertwining and forming a ball. The sizes and localization of a sarcoma are various. With early diagnosis and fast treatment has a positive outlook.

Malignant sarcoma

Malignant sarcoma is a soft tissue tumor, that is, a pathological formation. There are several clinical features that unite malignant sarcomas:

• Localization deep in the muscles and subcutaneous tissues.
• Frequent relapses of the disease and metastasis to the lymph nodes.
• Asymptomatic tumor growth for several months.
• The location of the sarcoma in the pseudocapsule and frequent germination beyond it.

Malignant sarcoma recurs in 40% of cases. Metastases occur in 30% of patients and most commonly affect the liver, lungs, and brain. Let's look at the main types of malignant sarcoma:

• Malignant fibrous histiocytoma is a soft tissue tumor localized in the trunk and extremities. When conducting an ultrasound examination, the tumor does not have clear contours, it can be adjacent to the bone or cover the vessels and tendons of the muscles.
• Fibrosarcoma is a malignant formation of connective fibrous tissue. As a rule, it is localized in the area of ​​the shoulder and thigh, in the thickness of the soft tissues. Sarcoma develops from intermuscular fascial formations. Metastasizes to the lungs and occurs most often in women.
• Liposarcoma is a malignant adipose tissue sarcoma with many varieties. It occurs in patients of all ages, but most often in men. It affects the limbs, thigh tissues, buttocks, retroperitoneal space, uterus, stomach, spermatic cord, mammary glands. Liposarcoma can be single or multiple, simultaneously developing on several parts of the body. The tumor grows slowly, but can reach very large sizes. The peculiarity of this malignant sarcoma is that it does not grow into the bones and skin, but can recur. The tumor metastasizes to the spleen, liver, brain, lungs and heart.
• Angiosarcoma is a malignant sarcoma of vascular origin. It occurs in both men and women, aged 40-50 years. Localized on the lower extremities. The tumor contains blood cysts, which become the focus of necrosis and hemorrhage. The sarcoma grows very rapidly and is prone to ulceration, and can metastasize to regional lymph nodes.
• Rhabdomyosarcoma is a malignant sarcoma that develops from striated muscles and ranks 3rd among malignant soft tissue lesions. As a rule, it affects the limbs, develops in the thickness of the muscles in the form of a knot. On palpation, soft with a dense texture. In some cases, it causes hemorrhages and necrosis. Sarcoma is quite painful, metastasizes to the lymph nodes and lungs.
• Synovial sarcoma is a malignant soft tissue tumor that occurs in patients of all ages. As a rule, it is localized on the lower and upper limbs, in the area of knee joints, feet, thighs, legs. The tumor has the shape of a round node, limited from the surrounding tissues. Inside the formation are cysts of different sizes. Sarcoma recurs and can metastasize even after a course of treatment.
• Malignant neuroma is a malignant neoplasm that occurs in men and in patients suffering from Recklinghausen's disease. The tumor is localized on the lower and upper limbs, head and neck. Metastasizes rarely, can give metastases to the lungs and lymph nodes.

Pleomorphic sarcoma

Pleomorphic sarcoma is a malignant tumor that affects the lower extremities, trunk, and other places. On the early stages development, the tumor is difficult to diagnose, therefore it is detected upon reaching 10 or more centimeters in diameter. The formation is a lobed, dense knot, reddish-gray in color. The node contains a zone of hemorrhage and necrosis.

Pleomorphic fibrosarcoma recurs in 25% of patients, metastasizes to the lungs in 30% of patients. Due to the progression of the disease, the tumor often causes death, within a year from the date of detection of the formation. The survival rate of patients after the detection of this formation is 10%.

Polymorphic cell sarcoma

Polymorphic cell sarcoma is a rather rare autonomous type of primary skin sarcoma. The tumor develops, as a rule, along the periphery of the soft tissues, and not in depth, surrounded by an erythematous corolla. During the growth period, it ulcerates and becomes similar to gummy syphilis. It metastasizes to the lymph nodes, causes an increase in the spleen, and when squeezing the soft tissues causes severe pain.

According to the results of histology, it has an alveolar structure, even with reticular carcinoma. The connective tissue network contains round and spindle-shaped cells of the embryonic type, similar to megakaryocytes and myelocytes. In this case, the blood vessels are devoid of elastic tissue and thinned. Treatment of polymorphic cell sarcoma is only surgical.

Undifferentiated sarcoma

An undifferentiated sarcoma is a tumor that is difficult or impossible to classify based on histological findings. This type of sarcoma is not associated with specific cells, but is usually treated as a rhabdomyosarcoma. So, malignant tumors of indefinite differentiation include:

• Epithelioid and alveolar soft tissue sarcoma.
• Clear cell tumor of soft tissues.
• Intimal sarcoma and malignant mesenchymoma.
• Round cell desmoplastic sarcoma.
• Tumor with perivascular epithelioid cell differentiation (myomelanocytic sarcoma).
• Extrarenal rhabdoid neoplasm.
• Extraskeletal Ewing tumor and extraskeletal myxoid chondrosarcoma.
• Neuroectodermal neoplasm.

Histiocytic sarcoma

Histiocytic sarcoma is a rare malignant neoplasm of an aggressive nature. The tumor consists of polymorphic cells, in some cases it has giant cells with a polymorphic nucleus and pale cytoplasm. Histiocytic sarcoma cells are positive when tested for non-specific esterase. The prognosis of the disease is unfavorable, since generalization occurs quickly.

Histiocytic sarcoma is characterized by a rather aggressive course and a poor response to therapeutic treatment. This type of sarcoma causes extranodal lesions. This pathology is exposed to the gastrointestinal tract, soft tissues and skin. In some cases, histiocytic sarcoma affects the spleen, central nervous system, liver, bones, and bone marrow. During the diagnosis of the disease, immunohistological examination is used.

round cell sarcoma

Round cell sarcoma is a rare malignant tumor consisting of round cellular elements. The cells contain hyperchromic nuclei. Sarcoma corresponds to the immature state of the connective tissue. The tumor progresses rapidly, therefore it is extremely malignant. There are two types of round cell sarcoma: small cell and large cell (the type depends on the size of the cells that make up its composition).

According to the results of histological examination, the neoplasm consists of round cells with poorly developed protoplasm and a large nucleus. Cells are located close to each other, do not have a specific order. There are adjoining cells and cells separated from each other by thin fibers and a pale-colored amorphous mass. Blood vessels are located in connective tissue layers and tumor cells that are adjacent to its walls. The tumor affects the skin and soft tissues. Sometimes, with the lumen of the vessels, it is possible to see tumor cells that have invaded healthy tissues. The tumor metastasizes, recurs and causes necrosis of the affected tissues.

Fibromyxoid sarcoma

Fibromyxoid sarcoma is a neoplasm with a low degree of malignancy. The disease affects both adults and children. Most often, the sarcoma is localized in the trunk, shoulders and hips. The tumor rarely metastasizes and grows very slowly. The reasons for the appearance of fibromyxoid sarcoma include hereditary predisposition, soft tissue injuries, exposure to large doses of ionizing radiation and chemical substances with carcinogenic effects. The main symptoms of fibromyxoid sarcoma:

• There are painful seals and tumors in the soft tissues of the trunk and limbs.
• In the area of ​​​​the neoplasm, painful sensations appear, and sensitivity is disturbed.
• The skin becomes bluish-brown in color, and with an increase in the neoplasm, compression of the vessels and ischemia of the extremities occur.
• If the neoplasm is localized in the abdominal cavity, then the patient has pathological symptoms from the gastrointestinal tract (dyspeptic disorders, constipation).

The general symptomatology of fibromyxoid sarcoma is manifested in the form of unmotivated weakness, weight loss and lack of appetite, which lead to anorexia, as well as frequent fatigue.

, , , , , ,

Lymphoid sarcoma

Lymphoid sarcomas are tumors immune system. Clinical picture disease is polymorphic. So, in some patients, lymphoid sarcoma manifests itself in the form of enlarged lymph nodes. Sometimes the symptoms of the tumor manifest themselves in the form of autoimmune hemolytic anemia, eczema-like rashes on the skin and poisoning. Sarcoma begins with a syndrome of compression of the lymphatic and venous vessels, which leads to dysfunction of the organs. Rarely, sarcoma causes necrotic lesions.

Lymphoid sarcoma has several forms: localized and local, widespread and generalized. From a morphological point of view, lymphoid sarcoma is divided into: large cell and small cell, that is, lymphoblastic and lymphocytic. The tumor affects the lymph nodes of the neck, retroperitoneal, mesenteric, less often - axillary and inguinal. A neoplasm can also occur in organs that contain lymphoreticular tissue (kidneys, stomach, tonsils, intestines).

To date, there is no single classification of lymphoid sarcomas. In practice, the international clinical classification is used, which was adopted for Hodgkin's disease:

1. Local stage - affected lymph nodes in one area, have an extranodal localized lesion.
2. Regional stage - affected lymph nodes in two or more areas of the body.
3. Generalized stage - the lesion has arisen on both sides of the diaphragm or spleen, the extranodal organ is affected.
4. Disseminated stage - sarcoma progresses to two or more extranodal organs and lymph nodes.

Lymphoid sarcoma has four stages of development, each of which causes new, more painful symptoms and requires long-term chemotherapy for treatment.

Epithelioid sarcoma

Epithelioid sarcoma is a malignant tumor affecting the distal extremities. The disease most often occurs in young patients. Clinical manifestations indicate that epithelioid sarcoma is a type of synovial sarcoma. That is, the origin of the neoplasm is a controversial issue among many oncologists.

The disease got its name because of the rounded cells, a large epithelioid shape, which resemble granulomatous inflammatory process or squamous cell carcinoma. The neoplasm appears as a subcutaneous or intradermal nodule or multinodular mass. The tumor appears on the surface of the palms, forearms, hands, fingers, feet. Epithelioid sarcoma is the most common soft tissue tumor of the upper extremities.

Sarcoma is treated by surgical excision. Such treatment is explained by the fact that the tumor spreads along the fascia, blood vessels, nerves and tendons. Sarcoma can metastasize - nodules and plaques along the forearm, metastases in the lungs and lymph nodes.

, , , , , , , , ,

myeloid sarcoma

Myeloid sarcoma is a local neoplasm consisting of leukemic myeloblasts. In some cases, before myeloid sarcoma, patients have acute myeloid leukemia. Sarcoma can act as a chronic manifestation of myeloid leukemia and other myeloproliferative lesions. The tumor is localized in the bones of the skull, internal organs, lymph nodes, tissues of the mammary glands, ovaries, gastrointestinal tract, tubular and spongy bones.

Myeloid sarcoma is treated with chemotherapy and local radiation therapy. The tumor is amenable to anti-leukemic treatment. The tumor rapidly progresses and grows, which determines its malignancy. Sarcoma metastasizes and causes disturbances in the functioning of vital organs. If the sarcoma develops in the blood vessels, then patients experience disturbances in the hematopoietic system and anemia develops.

Clear cell sarcoma

Clear cell sarcoma is a malignant fasciogenic tumor. Neoplasm, as a rule, is localized on the head, neck, torso and affects soft tissues. The tumor is a dense round nodules, 3 to 6 centimeters in diameter. During histological examination, it was determined that the tumor nodes have a gray-white color and anatomical connection. Sarcoma develops slowly and is characterized by a long-term long-term course.

Sometimes, clear cell sarcoma appears around or inside tendons. The tumor often recurs and metastasizes to the bones, lungs, and regional lymph nodes. Sarcoma is difficult to diagnose, it is very important to differentiate it from a primary malignant melanoma. Treatment can be performed with surgical methods and radiation therapy methods.

, , , , , , , , , , ,

Neurogenic sarcoma

Neurogenic sarcoma is a malignant neoplasm of neuroectodermal origin. The tumor develops from the Schwann sheath of peripheral nerve elements. The disease is extremely rare, in patients aged 30-50 years, usually on the limbs. According to the results of histological examination, the tumor is round, coarse and encapsulated. The sarcoma consists of spindle-shaped cells, the nuclei are arranged in the form of a palisade, the cells are in the form of spirals, nests and bundles.

Sarcoma develops slowly, causes pain on palpation, but is well limited by surrounding tissues. Sarcoma is located along nerve trunks. Treatment of the tumor is only surgical. In severe cases, excision or amputation is possible. Methods of chemotherapy and radiation therapy are ineffective in the treatment of neurogenic sarcoma. The disease often recurs, but has a positive prognosis, the survival rate among patients is 80%.

Bone sarcoma

Bone sarcoma is a rare malignant tumor of various localization. Most often, the disease appears in the knee and shoulder joints and in the region of the pelvic bones. The cause of the disease may be an injury. Exostoses, fibrous dysplasia, and Paget's disease are another cause of bone sarcoma. Treatment involves chemotherapy and radiation therapy.

Muscle sarcoma

Muscle sarcoma is very rare and most often affects younger patients. In the early stages of development, sarcoma does not manifest itself and does not cause painful symptoms. But the tumor gradually grows, causing swelling and pain. In 30% of cases of muscle sarcoma, patients experience abdominal pain, attributing this to problems with the gastrointestinal tract or menstrual pain. But soon, painful sensations begin to be accompanied by bleeding. If muscle sarcoma has arisen on the limbs and begins to increase in size, then it is easiest to diagnose.

Treatment depends entirely on the stage of development of the sarcoma, size, metastasis and extent of spread. For treatment, surgical methods and radiation exposure are used. The surgeon removes the sarcoma and some of the healthy tissue around it. Radiation is used both before and after surgery to shrink the tumor and kill any remaining cancer cells.

Skin sarcoma

Skin sarcoma is a malignant lesion, the source of which is the connective tissue. As a rule, the disease occurs in patients aged 30-50 years. The tumor is localized on the trunk and lower extremities. The causes of sarcoma are chronic dermatitis, trauma, prolonged lupus, scars on the skin.

Skin sarcoma most often manifests itself in the form of solitary neoplasms. The tumor can appear both on intact dermis and on scarred skin. The disease begins with a small hard nodule, which gradually increases, acquiring irregular outlines. The neoplasm grows towards the epidermis, growing through it, causing ulceration and inflammation.

This type of sarcoma metastasizes much less frequently than other malignant tumors. But with the defeat of the lymph nodes, the death of the patient occurs in 1-2 years. Treatment of skin sarcoma involves the use of chemotherapy methods, but surgery considered more efficient.

Sarcoma of the lymph nodes

Lymph node sarcoma is a malignant neoplasm that is characterized by destructive growth and arises from lymphoreticular cells. Sarcoma has two forms: local or localized, generalized or widespread. From a morphological point of view, lymph node sarcoma is: lymphoblastic and lymphocytic. Sarcoma affects the lymph nodes of the mediastinum, neck and peritoneum.

The symptomatology of sarcoma is that the disease is growing rapidly and increasing in size. The tumor is easily palpated, the tumor nodes are mobile. But due to pathological growth, they can acquire limited mobility. Symptoms of lymph node sarcoma depend on the degree of damage, stage of development, localization and general condition of the body. Diagnose the disease with the help of ultrasound and x-ray therapy. In the treatment of sarcoma of the lymph nodes, methods of chemotherapy, radiation exposure and surgical treatment are used.

Vascular sarcoma

Vascular sarcoma has several varieties that differ in nature of origin. Let's look at the main types of sarcomas and malignant tumors that affect the vessels.

• Angiosarcoma

It is a malignant tumor that consists of a collection of blood vessels and sarcomatous cells. The tumor progresses rapidly, is capable of disintegration and profuse bleeding. The neoplasm is a dense, painful knot of dark red color. In the initial stages, angiosarcoma can be mistaken for a hemangioma. Most often, this type of vascular sarcoma occurs in children older than five years.

• endothelioma

Sarcoma originating from the inner walls of a blood vessel. A malignant neoplasm has several layers of cells that can close the lumen of blood vessels, which complicates the diagnostic process. But the final diagnosis is made with the help of histological examination.

• Peritelioma

Hemangiopericytoma originating from the outer choroid. The peculiarity of this type of sarcoma is that sarcomatous cells grow around the vascular lumen. The tumor may consist of one or more nodes of different sizes. The skin over the tumor acquires a blue color.

Treatment of vascular sarcoma involves surgical intervention. After the operation, the patient undergoes a course of chemotherapy and radiation exposure to prevent recurrence of the disease. The prognosis for vascular sarcomas depends on the type of sarcoma, its stage and method of treatment.

Metastases in sarcoma

Metastases in sarcoma are secondary foci of tumor growth. Metastases are formed as a result of the detachment of malignant cells and their penetration into the blood or lymphatic vessels. With the blood flow, the affected cells travel throughout the body, stopping anywhere and forming metastases, that is, secondary tumors.

The symptomatology of metastases depends entirely on the location of the neoplasm. Most often, metastases occur in nearby lymph nodes. Metastases progress, affecting organs. The most common sites for metastasis are the bones, lungs, brain, and liver. For the treatment of metastases, it is necessary to remove the primary tumor and tissues of regional lymph nodes. After that, the patient undergoes a course of chemotherapy and radiation exposure. If metastases reach a large size, then they are removed surgically.

Diagnosis of sarcoma

Diagnosis of sarcoma is extremely important, as it helps to establish the location of the malignant neoplasm, the presence of metastases, and sometimes the cause of the tumor. Diagnosis of sarcoma is a complex various methods and techniques. Simplest diagnostic method- This is a visual examination, which includes determining the depth of the tumor, its mobility, size, consistency. Also, the doctor should examine the regional lymph nodes for metastases. In addition to visual examination, for the diagnosis of sarcoma use:

• Computed and magnetic resonance imaging - these methods provide important information about the size of the tumor and its relationship with other organs, nerves and great vessels. Such diagnostics is carried out for tumors of the small pelvis and extremities, as well as sarcomas located in the sternum and abdominal cavity.
• Ultrasound procedure.
• Radiography.
• Neurovascular examination.
• Radionuclide diagnostics.
• Biopsy - taking sarcoma tissue for histological and cytological studies.
• Morphological study - is carried out to determine the stage of sarcoma, the choice of treatment tactics. Allows you to predict the course of the disease.

Do not forget about timely treatment specific inflammatory diseases which can accept chronic form(syphilis, tuberculosis). Hygienic measures are a guarantee of the normal functioning of individual organs and systems. Mandatory is the treatment of benign tumors that can degenerate into sarcomas. And also, warts, ulcers, seals in the mammary gland, tumors and stomach ulcers, erosions and cracks in the cervix.

Prevention of sarcoma should include not only the implementation of the above methods, but also the passage of preventive examinations. Women should visit a gynecologist every 6 months to identify and promptly treat lesions and diseases. Do not forget about the passage of fluorography, which allows you to identify lesions of the lungs and chest. Compliance with all the above methods is an excellent prevention of sarcoma and other malignant tumors.

Sarcoma prognosis

The prognosis of sarcoma depends on the location of the neoplasm, the origin of the tumor, the growth rate, the presence of metastases, the volume of the tumor, and the general condition of the patient's body. The disease is distinguished by the degree of malignancy. The higher the grade of malignancy, the worse the prognosis. Do not forget that the prognosis also depends on the stage of the sarcoma. In the first stages, the disease can be cured without detrimental consequences for the body, but the last stages of malignant tumors have a poor prognosis for the patient's life.

Despite the fact that sarcomas are not the most common oncological diseases that can be treated, sarcomas are prone to metastasis, affecting vital organs and systems. In addition, sarcomas can recur, again and again affecting a weakened body.

Survival in sarcoma

Survival in sarcoma depends on the prognosis of the disease. The better the prognosis, the higher the patient's chances for a healthy future. Very often, sarcomas are diagnosed in the last stages of development, when a malignant tumor has already managed to metastasize and affect all vital organs. In this case, the survival of patients ranges from 1 year to 10-12 years. Survival also depends on the effectiveness of treatment, the more successfully passed medical therapy the more likely the patient will live.

Sarcoma is a malignant tumor that is rightfully considered a cancer of the young. Everyone is susceptible to the disease, both children and adults. The danger of the disease is that at first, the symptoms of sarcoma are insignificant and the patient may not even know that his malignant tumor is progressing. Sarcomas are diverse in origin and histological structure. There are many types of sarcomas, each of which requires a special approach in diagnosis and treatment.

Morbidity. Soft tissue sarcomas account for 1% of all malignant neoplasms in adults. Tumors equally affect men and women, more often at the age of 20-50 years. Possible occurrence in childhood(10-11% sarcomas).

Code according to the international classification of diseases ICD-10:

Histogenesis. The source of growth is the most heterogeneous tissue in structure and origin. Basically, these are derivatives of the mesenchyme: fibrous connective, adipose, synovial and vascular tissues, as well as tissues associated with the mesoderm (striated muscles) and neuroectoderm (nerve sheaths). It should be borne in mind that every third soft tissue tumor cannot be classified with conventional microscopy due to the difficulty in determining histogenesis. In such cases, an immunohistochemical study can provide significant assistance.

Histogenetic classification. Mesenchyme: .. Malignant mesenchymoma.. Myxoma. Fibrous tissue: .. Desmoid (invasive form) .. Fibrosarcoma. Adipose tissue - liposarcoma. Vascular tissue: .. Malignant hemangioendothelioma.. Malignant hemangiopericytoma.. Malignant lymphangiosarcoma. Muscle tissue:.. Striated muscles - rhabdomyosarcoma.. Smooth muscles - leiomyosarcoma. Synovial tissue - synovial sarcoma. Sheaths of nerves: .. Neuroectodermal - malignant neurinoma (schwannoma) .. Connective tissue - perineural fibrosarcoma. Unclassified blastomas.

The reasons

Risk factors. Ionizing radiation. Exposure to chemicals (such as asbestos or wood preservatives) . Genetic disorders. For example, 10% of patients with von Recklinghausen's disease develop neurofibrosarcoma. Previous bone disease. Osteosarcomas develop in 0.2% of patients with Paget's disease (ostosis deformans).

Symptoms (signs)

clinical picture. Sarcomas can develop anywhere on the trunk or limbs and usually appear as a painless tumor of varying consistency and density. Sarcomas that occur in the deep parts of the thigh, retroperitoneal space, by the time of diagnosis, usually reach large sizes. Patients usually note a decrease in body weight and complain of pain of uncertain localization. In the distal extremities, even a small tumor attracts attention early. Bleeding is the most common manifestation of sarcomas of the gastrointestinal tract and female genital organs.

Diagnostics

Diagnostics. Fast growth, the location of the tumor below or at the level of the deep fascia, signs of infiltrative growth, fixation to other anatomical structures, raise serious suspicions of the malignant nature of the process. Biopsy. Fine needle aspiration biopsy does not give an idea of ​​the histological structure and degree of differentiation, but only confirms the presence of a malignant tumor. Trepan - a biopsy or excisional biopsy makes it possible to establish a final diagnosis for choosing a treatment method. When choosing a site for a biopsy, one should take into account the possible subsequent reconstructive (plastic) operations. Radiological examination: radiography, bone scintigraphy, MRI, CT . precise definition boundaries between tumors and soft tissues .. CT and bone scintigraphy are preferable for detecting bone lesions .. If there are signs of liver dysfunction in sarcomas of internal organs or extremities, ultrasound and CT are performed (to detect metastases) .. Contrast angiography is indicated if vascular germination is suspected.

Classification

TNM classification (Kaposi's sarcoma, dermatofibrosarcoma, grade I desmoid tumors, sarcomas of the dura mater, brain, parenchymal organs or visceral membranes are not classified) .. Primary focus. The depth of location in the classification is taken into account as follows: ... Superficial - "a" - the tumor does not involve the (most) superficial muscle fascia ... Deep - "b" - the tumor reaches or grows (most) superficial muscle fascia. This includes all visceral tumors and / or tumors, sprouting large vessels, intrathoracic lesions. Most tumors of the head and neck are also considered deep... T1 - Tumor up to 5 cm in greatest dimension... T2 - Tumor more than 5 cm in greatest dimension. Regional lymph nodes (N) ... N1 - Metastases in regional lymph nodes nodes .. Distant metastases ... M1 - there are distant metastases.

Grouping by stages: .. Stage IA - G1 - 2T1a - 1bN0M0 - highly differentiated, small tumors, regardless of location .. Stage IB - G1 - 2T2aN0M0 - highly differentiated, large tumors located superficially .. Stage IIA - G1 - 2T2bN0M0 - well-differentiated, large tumors located deep.. Stage IIB - G3 - 4T1a - 1bN0M0 - poorly differentiated, small tumors, regardless of location. Stage IIC - G3 - 4T2aN0M0 - poorly differentiated, large tumors located superficially. Stage III - G3 - 4T2bN0M0 - poorly differentiated, large tumors located deep. Stage IV - the presence of any metastases - G1 - 4T1a - 2bN1M0, G1 - 4T1a - 2bN0M1.

Treatment

Treatment, general principles

When choosing treatment regimens, it is recommended to adhere to the age of 16 years and older as a criterion for an adult, but the choice of treatment method must be chosen by a council. For example, rhabdomyosarcoma is successfully treated according to pediatric schemes up to the age of 25, however, low-grade fibrosarcoma at the age of 14 should be treated as in an adult - with a surgical method.

Tumors of the extremities and superficially located tumors of the trunk are subject to surgical removal using the principles of "case". Possible skin deficiency is not an obstacle to intervention. When the tumor is presented to the bone, it is removed along with the periosteum, and during germination, a planar or segmental resection of the bone is performed. With microscopic detection of malignant cells in the edges of the resected tissue, the muscular-fascial case is re-excised. Radiation therapy is indicated when the edge of the tumor is located less than 2-4 cm from the resection line or when the wound is seeded with tumor cells.

Tumors of the posterior mediastinum, retroperitoneal in the pelvis and paravertebral, are more likely to be unremovable. Small tumors anterior mediastinum and retroperitoneal in the left half of the body can be removed surgically. In doubtfully operable tumors, radiation or thermoradiotherapy, regional chemotherapy, and chemoembolization of the vessels feeding the tumor are performed preoperatively. Since tumors of these localizations are more often detected in the later stages and radical removal often fails, the operation is supplemented with radiation therapy. With the development of relapses, repeated interventions are indicated.

Recurrence is a characteristic biological feature of sarcomas, therefore methods of combined and complex treatment are being developed.

Features of therapeutic measures depend on the histological structure of the tumor. Neurogenic sarcoma and fibrosarcomas are insensitive to radiation and chemotherapy, treatment (including relapses) is only surgical. Angiosarcoma, liposarcoma are relatively sensitive to radiation therapy (preoperative telegamma therapy is required). Myogenic and synovial sarcomas require neoadjuvant chemotherapy and radiotherapy.

Solitary metastases of sarcomas in the lungs are subject to surgical removal (wedge resection), followed by chemotherapy. Most often, such metastases occur within 2 to 5 years after the primary operation.

With complications of tumor growth, it is possible to perform palliative resections, which can reduce intoxication, blood loss in decaying tumors, eliminate symptoms of compression of other organs (obstruction of the ureter, intestinal collapse with symptoms intestinal obstruction etc.).

TYPES OF SOFT TISSUE SARCOMAS

fibrosarcoma accounts for 20% of malignant soft tissue lesions. It occurs more often in women 30-40 years old. The tumor consists of atypical fibroblasts with a different amount of collagen and reticular fibers. Clinical picture .. Localization - soft tissues of the limbs (thigh, shoulder girdle), less often the trunk, head, neck .. Most important feature- no skin lesions over the tumor. Metastases in regional lymph nodes are noted in 5-8% of patients. Hematogenous metastases (most often in the lungs) - in 15-20%. Treatment is excision of the tumor in compliance with zoning and sheathing. Forecast. With adequate treatment, the 5-year survival rate is 77%.

Liposarcoma registered in 15% of cases of soft tissue tumors. Occur more often at the age of 40-60 years The tumor consists of anaplastic fat cells and areas of myxoid tissue. Clinical picture.. Most often, the tumor is located on the lower extremities and in the retroperitoneal space.. Extremely rarely, liposarcomas develop from single and multiple lipomas.. Typically, early hematogenous metastasis to the lungs (30-40%). Treatment is wide excision; for large tumors, preoperative radiation therapy is justified. Forecast. In patients with differentiated tumors, the 5-year survival rate is 70%, with poorly differentiated tumors - 20%.

Rhabdomyosarcoma- a malignant tumor originating from the skeletal (striated) muscle. There are embryonic (develops up to 15 years) and adult types of rhabdomyosarcoma. Frequency. It occupies the 3rd place among malignant soft tissue neoplasms. It is registered at any age, but more often in adolescents and in the middle age group. Women get sick 2 times more often.

Pathomorphology. The tumor consists of spindle-shaped or rounded cells, in the cytoplasm of which longitudinal and transverse striations are determined. Genetic aspects. In the development of rhabdomyosarcomas, the participation of several genes located on the ridge is assumed. 1, 2, 11, 13 and 22; the possible role of genomic imprinting or duplication of individual genes (for example, the gene for insulin-like growth factor - 2 IGF2, PAX3 and PAX7 genes) is considered.

Clinical picture .. Most often, tumors are localized in three anatomical regions of the body: limbs, head and neck, small pelvis .. The tumor grows rapidly, without pain and organ dysfunction .. Often, the skin sprouts with the formation of exophytic bleeding formations. Treatment- surgical, for large tumors, preoperative radiation therapy is advisable. When carrying out combined (surgical, chemotherapy) treatment of localized forms of embryonic rhabdomyosarcoma, an increase in 5-year survival rate up to 70% is noted. In the presence of metastases, the 5-year survival rate is 40%. With pleomorphic rhabdomyosarcoma (a tumor of adults), the 5-year survival rate is 30%.

Synonyms. Rhabdosarcoma. Rhabdomyoblastoma. Malignant rhabdomyoma

Angiosarcoma accounts for about 12% of all soft tissue neoplasms. The tumor is more often observed in young people (up to 40 years). Morphology.. Hemangioendothelioma is formed from many atypical capillaries with proliferation of atypical endothelial cells that fill the lumen of the vessels.. Hemangiopericytoma, which develops from modified cells of the outer shell of capillaries. Clinical picture.. The tumor has infiltrating rapid growth, is prone to early ulceration and fusion with surrounding tissues. Early metastasis to the lungs and bones, dissemination to the soft tissues of the body is quite common. Treatment - surgery is combined with radiation therapy.

Lymphangiosarcoma(Stuart-Treevs syndrome) - a specific tumor that develops in the area of ​​​​permanent lymphatic edema (upper limb in women with postmastectomy syndrome, especially after a course of radiation therapy). The prognosis is unfavorable.

Leiomyosarcoma accounts for 2% of all sarcomas. The tumor consists of atypical elongated cells with rod-shaped nuclei. Clinical picture.. On the limbs, the tumor is located in the projection of the vascular bundle.. The tumor is always solitary. Treatment is surgical.

synovial sarcoma occupies the 3rd-4th place in frequency among soft tissue sarcomas (8%). Register mainly in persons younger than 50 years The tumor consists of succulent spindle-shaped and rounded cells. Clinical picture. Localization in the area of ​​the hand and foot is typical. Treatment is surgical, with regional lymph node dissection.

Malignant neuromas- a fairly rare pathology (about 7% of soft tissue lesions .. The tumor consists of elongated cells with elongated nuclei. Clinical picture .. They are most often located on the lower extremities .. The primary multiplicity of the tumor is characteristic .. Recurrence is possible. Treatment - surgical. Prognosis. The main prognostic factors are the degree of histological differentiation and tumor size, in patients with poorly differentiated neurinoma, the prognosis is less favorable.

Kaposi's sarcoma(see Kaposi's sarcoma).

ICD-10. C45 Mesothelioma. C46 Kaposi's sarcoma. C47 Malignant neoplasm of peripheral nerves and autonomic nervous system. C48 Malignant neoplasm of the retroperitoneal space and peritoneum. C49 Malignant neoplasm of other types of connective and soft tissues.

Sarcoma is a neoplasm more often of a malignant nature of the course. There are many different sarcomas, which in ICD 10 are singled out as a single disease. The danger of pathology lies in the absence of symptoms in the initial stages of neoplasm development, which makes diagnosis difficult.

General concept of sarcoma

Sarcoma can be localized in different parts of the tissues. It can affect muscles, epithelium, nerve fibers, connective tissue. In medicine, there are three main types of sarcomas: conditionally benign, malignant and intermediate, accompanied by metastatic lesions.

The disease is established in both men and women. But in male patients, sarcoma is diagnosed more often.

The main feature of the formations, which distinguishes them from other types of tumors, is that they form in soft tissues. Metastases in this case appear in the liver, brain, and lungs. As a result, a cancerous lesion of the injured organs develops.

In medicine, a wide variety of sarcomas are distinguished depending on their composition:

• epithelioid;
• angiosarcoma;
• fibrous histiocytoma;
• fibrosarcoma;
• leiomyosarcoma;
• rhabdomyosarcoma;
• liposarcoma;
• schwannoma;
• spindle cell sarcoma;
• hemangiopericytoma;
• mesenchymoma;
• synovial sarcoma;
• alveolar.

The type of sarcoma is determined based on the results of instrumental diagnostic methods by a specialist. This is necessary for the fact that many of the formations are able to transform into malignant tumors and become the cause of metastatic lesions.

The reasons

Experts failed to establish the true causes of the formation of sarcoma. But they brought out a number of factors that can influence and increase the risk of developing the disease.

Genetic predisposition is of great importance in the formation of tumors of various types. It was found that in patients with diagnosed fibrosarcoma or another type of formation, close relatives suffered from a similar pathology.

One of the factors causing sarcomas is the herpes virus. Experts also argue that carcinogenic, chemical, toxic and poisonous substances also have an effect on the body.

The unfavorable environmental situation in the areas where the patient lives can also significantly increase the risk of sarcoma formation.

Scientists believe that the causes of the formation of tumors are regular damage to the skin, prolonged use of steroid drugs and the presence of precancerous diseases.

Clinical picture

Despite the fact that many types of sarcomas are distinguished in medicine, they have similar symptoms, as a result of which they were combined into one group.

At the initial stages of tumor development, signs of the disease do not appear. As the neoplasm grows, the patient notices a sharp weight loss, constant fatigue, depression and fatigue.

The advanced stage of cancer is characterized by painful sensations in the affected area, impaired performance of the affected organ. The skin at the location of the tumor changes its color, and ulcers appear on its surface.

The tumor is most often defined as a small nodule. It is yellowish or white in color. In the initial stages of its development, it is not accompanied by painful sensations.

The surface of the formation is smooth, but as it develops, it becomes bumpy, ulcers appear. The size of the tumor can reach 30 centimeters.

The danger of sarcoma is that it does not manifest itself for a long time and the patient is not aware of its presence. If the sarcoma affects the muscle tissue, it becomes noticeable already in the later stages of development, when there is no chance for a successful recovery.

Diagnostics

The establishment of the disease in the presence of sarcoma in some cases is difficult, due to the absence of symptoms in the initial stages. A preliminary diagnosis is established on the basis of patient complaints and external examination. In order to confirm it and determine the characteristics of the course of the pathology, the specialist prescribes a number of instrumental and laboratory methods diagnostics:

Ultrasound procedure

The technique is quite informative and allows you to establish the presence of a tumor that is localized in the soft tissues of the thigh, limbs and other parts of the body.

Ultrasound helps to determine the size, location, structure of the neoplasm. Sarcoma has certain features that distinguish it from other types of diseases. First of all, it does not have a capsule and a homogeneous structure. Also, foci of necrosis in sarcoma are located inside the formation.

CT scan

CT is often performed with a contrast agent if sarcoma is suspected. This allows you to determine the area of ​​circulatory disorders. When performing computed tomography, sarcoma is determined by irregular shape, fuzzy contours, heterogeneous structure. Neighboring tissues are also damaged and compressed. The contrast agent accumulates in tortuous vessels.

Magnetic resonance imaging

Appointed when it is impossible to conduct computed tomography. The methodology is quite informative. The main advantage of MRI is the possibility of layer-by-layer visualization of the neoplasm, determination of its structure, size, and area of ​​location.

Radiography

X-ray examination is carried out in order to determine metastatic lesions not only in neighboring organs or tissues, but also in distant ones.

The disadvantage of the technique is that it is impossible to determine the type of neoplasm using an X-ray machine.

Biopsy

A fine-needle biopsy for sarcoma is prescribed to confirm the type of formation and the presence of cancer cells. The sampling procedure is carried out using a special apparatus, in which a specialist extracts a sample of sarcoma tissue.

The obtained samples are sent to the laboratory for histological examination. The patient can find out the results of the diagnosis from his attending physician after 7-10 days. In some cases, the study is done on an emergency basis. The result is ready after 20-30 minutes.

Patients should also take a general and biochemical blood test. The specialist may prescribe a plasma test for tumor markers. As additional methods studies show angiography and positron emission tomography.

Based on the results of the studies, the final diagnosis is established and a course of therapy is prescribed.

Treatment

When diagnosing sarcoma, treatment is carried out on an individual basis. Before prescribing a course of therapy, a consultation of doctors is held.

Despite the fact that there is no single system of treatment for embryonic rhabdomyosarcoma, fibrosarcoma and other types of sarcomas, surgery is often prescribed. The goals of the operation are to remove the neoplasm, alleviate the patient's condition and increase his life expectancy.

Surgical intervention in some cases can be supplemented with radiation or chemotherapy. Techniques can slow down tumor growth, but have a number of side effects. Among the undesirable effects are hair loss, brittle nails, weakness, nausea and weight loss. That is why they are not prescribed for the treatment of patients over the age of 60 years.

In cases where the sarcoma is large enough, affects many nerve endings and great vessels, it is removed along with the affected organ.

Chemotherapy is carried out in two or three courses. The interval between them is at least 3 weeks. Radiation therapy in some cases is prescribed as the main method of therapy. It is performed if the patient has contraindications for surgical intervention or the neoplasm is small.

After undergoing treatment, the patient becomes a dispensary record. The patient should visit his/her doctor on a regular basis for preventive purposes.

Complications

Soft tissue sarcoma of peripheral nerves, connective or muscle tissue, if left untreated, causes the development serious complications. Among them are noted:

1. Spreading metastatic lesions.
2. squeezing surrounding tissues and organs, which causes significant pain.
3. Development obstruction intestines and organ perforation.
4. Defeat lymphatic system resulting in a malfunction.
5. The appearance of internal bleeding.

Against the background of the development of various types of sarcoma, there is a decrease in hearing, vision, memory impairment and concentration. The sensitivity of the skin is also impaired. That is why treatment should not be postponed when diagnosing sarcoma.

Forecast

The prognosis in the case of the development of sarcoma is influenced by many different factors. First of all, this is the stage of the disease. Also great importance have the localization of the tumor process, age, condition of the patient, the presence of metastatic lesions, concomitant diseases.

The prognosis is more favorable in the case when treatment was started at stage 1 or 2 of the development of the pathology. The survival rate of patients is about 70-50%. But the sarcoma is dangerous because after removal, the neoplasm is formed again.

Most often, the prognosis for sarcoma is unfavorable, due to the fact that it is quite difficult to determine the presence of pathology at the initial stages of development. The survival rate of patients at stages 3 or 4 is no more than 15%.

Prevention measures

Since the true causes of the development of sarcoma have not been established, there are no special preventive measures. Doctors recommend following the basic rules:

1. visit in time doctor. Regular examinations and x-ray examinations will help to establish the onset of the development of the tumor process in a timely manner.
2. News active Lifestyle. If the work involves a long stay in one position, it is recommended to do exercises every hour, do morning exercises every day.
3. More walk outdoors. For these purposes, a park is suitable. You should dress according to the weather so as not to catch a cold.
4. Treat in a timely manner colds diseases.
5. Correctly eat. Eating foods fast food and fast food has a negative impact on immunity. You need to include more fruits and vegetables in your diet. Vitamin complexes should be taken in autumn and spring. This will help maintain immunity at the right level.
6. Eliminate exposure chemical, toxic and poisonous substances skin covering and the body as a whole. When working in hazardous industries, you must use personal protective equipment, and do housework only with gloves.
7. Avoid injury skin cover.

Compliance with preventive measures will help to significantly reduce the risk of developing various types of sarcoma. It is important for patients to know that only timely treatment increases the likelihood of a cure for the disease.

Soft tissue sarcoma is a common disease among other cancerous lesions. In medicine, there are many various types formations, but all of them pose a great danger to the health and life of the patient. In the initial stages, sarcoma does not show symptoms, which complicates the diagnosis.

That is why patients are recommended to undergo annual preventive examinations. When establishing a sarcoma, treatment should not be postponed, since its absence causes the development of serious consequences, including death.

RCHD (Republican Center for Health Development of the Ministry of Health of the Republic of Kazakhstan)
Version: Archive - Clinical Protocols of the Ministry of Health of the Republic of Kazakhstan - 2012 (Orders No. 883, No. 165)

Connective and soft tissues of trunk, unspecified (C49.6)

general information

Short description

Clinical protocol"Soft Tissue Sarcomas"

The term "soft tissue sarcomas" refers to a group of malignant tumors that arise in extraskeletal soft and connective tissues. Similar tumors are combined into one group due to the similarity of their histopathological characteristics and clinical manifestations, as well as the course of the tumor process.

Malignant tumors of soft tissues account for 0.2-2.6% in overall structure human malignant neoplasms. Nearly all malignant soft tissue tumors are sarcomas, which account for 0.7% of all human malignant tumors. In the Republic of Kazakhstan, the absolute number of newly diagnosed diseases in 1993 was 235, in 2002 - 192.

Most often, these tumors are observed at the age of 20-50 years. In children, in the structure of oncological morbidity, sarcomas make up 10-11%. The predominant localization of soft tissue sarcomas is the limbs (up to 60%), with approximately 46% of the lower limbs and about 13% of the upper limbs. On the trunk, these tumors are localized in 15-20% of cases, on the head and neck - in 5-10%. The retroperitoneal space accounts for 13-25%.

Protocol"Soft tissue sarcomas".

ICD code- С 49 (malignant tumors of soft tissues).

Abbreviations:

ECG - electrocardiography.

Ultrasound - ultrasonography.

MTS - metastasis.

ESR - erythrocyte sedimentation rate.

RW - Wasserman reaction.

HIV is the human immunodeficiency virus.

PCT - polychemotherapy.

PET - positron emission tomography.

Protocol development date: September 2011

Protocol Users: district oncologist, oncologist of the clinic of the dispensary, oncologist of the hospital of the dispensary.

Indication of no conflict of interest

We have no financial or other interest in the topic of the discussed document. Have not been involved in the sale, production or distribution of drugs, equipment, etc. in the last 4 years.

Classification

Histological types of tumors

According to the ICD-O morphological codes, the following histological types of tumors are classified according to the TNM system:

1. Alveolar soft tissue sarcoma.

2. Epithelioid sarcoma.

3. Extraskeletal chondrosarcoma.

4. Extraskeletal osteosarcoma.

5. Extraskeletal sarcoma of Ewing.

6. Primitive neuroectodermal tumor (PNET).

7. Fibrosarcoma.

8. Leiomyosarcoma.

9. Liposarcoma.

10. Malignant fibrous histiocytoma.

11. Malignant hemangiopericytoma.

12. Malignant mesenchymoma.

13. Malignant tumor originating from the sheaths of the peripheral nerve.

14. Rhabdomyosarcoma.

15. Synovial sarcoma.

16. Sarcoma without further specification (NOS).

Histological types of tumors not included in the TNM classification: angiosarcoma, Kaposi's sarcoma, dermatofibrosarcoma, fibromatosis (desmoid tumor), sarcoma originating from the dura mater, brain, hollow or parenchymal organs (with the exception of breast sarcoma).

Regional lymph nodes

Regional lymph nodes are nodes corresponding to the localization of the primary tumor. Regional lymph nodes are rarely involved, and when their condition cannot be determined clinically or pathologically, they are classified as N0 instead of NX or pNX.

TNM classification

Classification rules. Must be histological confirmation diagnosis, which allows to determine the histological type of the tumor and the degree of malignancy.

Anatomical areas:

1. Connective tissue, subcutaneous and other soft tissues (C 49), peripheral nerves(C 47).

2. Retroperitoneal space (C 48.0).

3. Mediastinum: anterior (C 38.1); posterior (C 38.2); mediastinum, NOS (C 38.3).

Determination of the stage of soft tissue sarcomas according to the T, N, M, G system

R classification

The absence or presence of residual tumor after treatment is described by the symbol R:

RX - presence of residual tumor cannot be assessed.

R0 - no residual tumor.

R1 - microscopic residual tumor.

R2 - macroscopic residual tumor.

Summary

Diagnostics

Diagnostic criteria(description of reliable signs of the disease depending on the severity of the process)

Complaints: the appearance and gradual growth of tumor formation of soft tissues. The appearance and growth of pain syndrome. Disturbance of movements in the limb.

Physical examination: the presence of a soft tissue tumor. Palpation pain. Visible impairment of limb function.

Laboratory research: increased ESR, leukocytosis (with the prevalence of the process).

Instrumental research:

1. Ultrasound examination of the affected area.

2. X-ray examination of the chest.

Indications for consultation with an oncologist: the presence of soft tissue tumors. The presence of radiological (ultrasound, CT) data of tumor lesions of soft tissues.

The list of basic and additional diagnostic measures:

Careful history taking;

Physical examination;

Blood type, Rh factor;

Wasserman reaction;

Detailed blood test;

General analysis urine;

Biochemical analysis blood (total protein, creatinine, urea, bilirubin, transaminases, alkaline phosphatase, ions - Na, K, Ca, Cl, glucose);

Coagulogram;

X-ray of the chest organs;

CT scan;

Magnetic resonance imaging of the affected area;

Bone marrow biopsy from ilium(with Ewing's sarcoma);

Morphological verification of the disease with the establishment of the histological type and degree of tumor differentiation (trepan or open biopsy):

For small or deep-seated tumors, trepanobiopsy is performed under ultrasonographic or radiographic control;

The dimensions of the tissue column should not be less than 4 x 10 mm;

At knife biopsy the incision should not complicate the subsequent choice of the variant of surgical intervention;
- cytological examination (does not replace the histological verification of the diagnosis):

Scraping smears from the surface of an ulcerated tumor;

smears-imprints of material taken with a knife or trephine biopsy;
- ultrasound examination of the abdominal organs;

Arteriography (performed when the tumor is located in the area where large main vessels);

PET - according to indications;

Scintigraphy of the skeleton according to indications.

Differential Diagnosis

Treatment abroad

Get treatment in Korea, Israel, Germany, USA

Get advice on medical tourism

Treatment

Purpose of treatment: removal of the tumor, prevention of distant metastasis and affected lymph nodes (if any).

Treatment tactics

The surgical method as an independent type is used in the treatment of primary highly differentiated tumors (T1a), provided that a radical surgical intervention can be performed. In other cases, the treatment is combined or complex, the leading and decisive component of which is the surgical removal of the tumor.

The treatment program is built taking into account the histological degree of malignancy, the spread of the process, the size and localization of the tumor.

Features of anesthetic support:

Surgical interventions for soft tissue sarcomas are performed under anesthesia or conduction anesthesia (if there are contraindications to anesthesia);

Trepanobiopsy is performed under local anesthesia.

Non-drug treatment

Principles of surgical interventions:

Together with the tumor, the site of the previous biopsy is removed;

Removal of the sarcoma is performed without exposing the tumor;

Regional lymph nodes in the absence of signs of their defeat are not removed;

The boundaries of tissue resection are marked with metal brackets (for planning postoperative radiation therapy and for non-radical removal of the tumor).

The main types of operations for soft tissue sarcomas

Simple excision- used exclusively as a stage morphological diagnostics malignant tumors.

wide excision. During this operation, the tumor is removed within the anatomical zone, in a single block with the pseudocapsule and at a distance of 4-6 cm or more from the visible edge of the tumor. Wide local resection is used for tumors of low grade, superficial, located above the superficial fascia, in the skin, subcutaneous tissue (small fibrosarcomas, liposarcomas, desmoids, dermatofibrosarcomas). This operation is not performed for high-grade sarcomas.

Radical operation. This operation is undertaken for deeply located sarcomas of a high degree of malignancy. It involves the removal of the tumor and the normal tissues surrounding it with the inclusion in a single block of fascia and unchanged surrounding muscles, which are completely removed with cutting off at the attachment site. If necessary, resection of vessels, nerves, bones is performed, resorting simultaneously to the corresponding reconstructive plastic surgery on vessels, nerves, bones, joints.

Organ-preserving and functionally sparing surgical interventions in case of locally advanced malignant tumors of the soft tissues of the extremities, they are performed exclusively as part of combined and complex treatment. The control of the radicalness of the surgical intervention is carried out by urgent intraoperative histological examination of the edges of the cut-off of the tumor from normal tissues.

Amputations and disarticulations. Amputation and disarticulation of a limb are indicated in cases where a radical sparing operation is not possible due to a massive lesion (large involvement of the joints, bones, great vessels and nerves in the tumor process) and/or in case of failure of neoadjuvant treatment courses.

Radiation therapy

Radiation therapy is used as part of combined and complex treatment. Radiation therapy is applied using deep-focus R-therapy, electron beam or Υ-therapy, as a rule, in the form of a preoperative or postoperative course in SOD 50-70 Gy in the classical fractionation mode. The choice of radiation source and energy of the electron beam is determined by the localization and depth of the tumor.

For a uniform summing up of the radiation dose to the entire interested zone, multifield methods of irradiation are used with the use of devices for the formation of optimal dose fields. The boundaries of the irradiation fields should exceed the size of the tumor by 3-4 cm. In case of large tumor sizes and/or a high degree of malignancy, the irradiation field should include up to 10 cm of tissues proximal and distal to the tumor boundaries. In this case, after reaching SOD 45-50 Gy, the irradiation field is reduced to the size of the tumor.

For tumors localized on the extremities, additional oblique fields extending beyond the irradiated tissues are used to reduce the likelihood of developing radiation osteonecrosis. Ideally, in order to reduce the severity of fibrosis, muscle contracture and edema, up to 1/3 of the limb circumference should be excluded from the irradiation field. The minimum width of non-irradiated tissues should be: on the forearm - 2 cm, on the lower leg - 3 cm, on the thigh - 4 cm.

Contraindications for preoperative radiotherapy are:

Lack of morphological confirmation of the diagnosis;

The collapse of the tumor with the threat of bleeding;

General contraindications for radiotherapy.

Postoperative radiation therapy is performed upon receipt of a histological conclusion of a high degree of malignancy and multicentric tumor growth (if no preoperative radiation therapy was performed), as well as conditionally radical or non-radical tumor removal. The beginning of radiation therapy  not later than 4 weeks after the operation.

If preoperative radiation therapy was not performed, the irradiation zone includes the bed of the removed tumor (the boundaries are marked with tantalum clips during the operation), the surrounding tissues with a 2 cm indent from the cut-off edges and postoperative scar(SOD 60 Gy). If there is a residual tumor, which should be marked with titanium staples during the operation, this area is additionally irradiated locally up to SOD of at least 70 Gy.

If the tumor is unresectable, radiation therapy is performed according to a radical program in SOD 70 Gy in the classical fractionation mode.

Treatment by stages

1. IA stage (T1a, T1b N0, NX M0 - low degree of malignancy): wide excision of the tumor within the anatomical zone.

2. Stage IB (T2a, T2b N0, NX M0 - low grade): surgical removal of the tumor (T2a - wide excision, T2b - radical operation) + a course of postoperative radiation therapy (the need is determined by the results of the final histological examination);

4. In order to increase the effectiveness of neoadjuvant treatment, when planning an organ-preserving surgical intervention, methods of regional chemotherapy (intra-arterial administration of chemotherapy drugs) are included in the treatment system.

5. When a large wound defect is formed after surgical removal of the tumor, which cannot be eliminated by reducing the edges of the wound, one of the types of primary plasty is performed:

Free skin flap;

local tissues;

Combined skin plasty;

Plasty with displaced islet flaps on vascular pedicles, autotransplantation of tissue complexes using microsurgical techniques.

6. If it is impossible to perform organ-preserving treatment, due to the local prevalence of the tumor process and the ineffectiveness of neoadjuvant treatment, amputation of the limb is performed.

IIA stage(T1a, T1b N0, NX M0 - high degree of malignancy):

Pre- or postoperative radiation therapy + wide excision of the tumor;

When an extensive wound defect is formed after surgical removal of the tumor, which cannot be eliminated by reducing the edges of the wound, one of the types of primary plasty is performed.

IIB stage(T2a N0, NX M0 - high grade).

III stage(T2b N0, NX M0 - high grade):

Pre- or postoperative radiation therapy (preference should be given to radiation therapy under conditions of local microwave hyperthermia) + surgical removal of the tumor (T2a - wide excision, T2b - radical salvage operation) + 3-4 courses of adjuvant polychemotherapy;

In order to increase the effectiveness of neoadjuvant treatment when planning organ-preserving surgery, the treatment system includes methods of regional chemotherapy (in / venous or intra-arterial administration of chemotherapy drugs);

With the formation of an extensive wound defect that cannot be eliminated by reducing the edges of the wound, one of the types of primary plasty is performed;

If it is impossible to perform organ-preserving treatment due to the local spread of the tumor and the absence of clinical effect after neoadjuvant treatment, limb amputation is performed.

IV stage(any T N1 M0 - any degree of malignancy):

Comprehensive treatment is carried out according to the principles of treatment of soft tissue sarcomas of stages I-III, taking into account the degree of tumor differentiation and local spread of the tumor process;

The surgical component provides, in addition to intervention on the primary focus (organ-preserving or organ-removing surgery), a typical regional lymph node dissection, which is performed simultaneously with the operation on the primary tumor (single block or staged) or in a delayed manner (depending on the affected area and the general condition of the patient).

IV stage(any T and N M1 - any degree of malignancy):

Palliative and symptomatic treatment is carried out according to individual programs with the inclusion of polychemotherapy and / or radiation therapy;

Surgical interventions are performed to reduce the tumor mass or according to sanitary indications (limb amputation).

Medical treatment

Polychemotherapy regimens

Port system installation

2. SARO:

Cisplatin 100 mg/m 2 IV, 1 day;

Doxorubicin 30 mg/m 2 IV, 2, 3, 4 days;

Vincristine 1.5 mg/m 2 IV, day 5;

Cyclophosphamide 600 mg/m 2 IV, day 6.

3. CyVADIC:

Dacarbazine 250 mg/m 2 IV, days 1-5.

4. CyVADakt:

Cyclophosphamide 500 mg/m 2 IV, day 2;

Vincristine 1 mg/m 2 IV, days 1, 8, 15;

Doxorubicin 50 mg/m 2 IV, 1 day;

Dactinomycin 0.3 mg/m 2 IV, 3/4/5 days.

Vincristine 1.5 mg/m 2 IV, 1, 8 days;

Doxorubicin 50 mg/m 2 IV, 1 day;

Dacarbazine 250 mg/m 2 IV, from 1 to 5 days.

Doxorubicin 60 mg/m 2 , 1 day;

Dacarbazine 250 mg/m 2 , days 1-5.

7. VAC II:

Vincristine 1.5 mg IV, 1.8 days;

Soft tissue sarcomas occur anywhere in the body. Approximately half of the tumor is localized on the lower extremities. The hip is most commonly affected. In 25% of patients, sarcoma is located on the upper limbs. The rest are on the trunk and occasionally on the head.
malignant tumor soft tissues has the appearance of a rounded whitish or yellowish-gray node with a bumpy or smooth surface. The consistency of the neoplasm depends on the histological structure. It can be firm (fibrosarcomas), soft (liposarcomas and angiosarcomas), and even jelly-like (myxomas). Soft tissue sarcomas do not have a true capsule, however, in the process of growth, the neoplasm compresses the surrounding tissues, the latter become denser, forming the so-called false capsule, which quite clearly outlines the tumor.
A malignant neoplasm usually occurs in the thickness of the deep layers of the muscles. As the size increases, the tumor gradually spreads to the surface of the body. Growth is accelerated under the influence of trauma and physiotherapy.
The tumor is usually solitary, but some types of sarcomas are characterized by multiple lesions. Sometimes they occur at a great distance from each other (multiple liposarcomas, malignant neuromas in Recklinhausen's disease).
Metastasis of malignant neoplasms of soft tissues occurs mainly by the hematogenous route (through the blood vessels). The favorite localization of metastases are the lungs. The liver and bones are less commonly affected. Metastases to the lymph nodes occur in 8-10% of cases.
A feature of soft tissue neoplasms is the existence of a group of tumors intermediate between benign and malignant. These tumors have locally recurrent infiltrating growth, often recur, but do not metastasize or metastasize extremely rarely (desmoid tumors of the abdominal wall, intermuscular or embryonic lipomas and fibromas, differentiated fibrosarcomas, etc.).
The leading sign is the appearance of a painless node or swelling of a round or oval shape. Node sizes vary from 2-3 to 25-30. The nature of the surface depends on the type of tumor. The boundaries of the neoplasm in the presence of a pronounced false capsule are clear, with a deep tumor, the contours of the swelling are fuzzy and difficult to determine. The skin is usually intact, but compared to the healthy side, there is a local increase in temperature above the tumor, and with massive, rapidly growing and reaching the surface of the formations, a network of dilated saphenous veins appears, cyanotic coloration and infiltration or ulceration of the skin. The mobility of the palpable formation is limited. This is one of the most characteristic and important symptoms for diagnosis.
Occasionally, soft tissue sarcomas lead to deformation of the limbs, cause a feeling of heaviness and awkwardness during movements, but the function of the limb is rarely impaired.
"Warning signals", in the presence of which a suspicion of soft tissue sarcoma should be expressed, are:
- the presence of a gradually increasing tumor formation;
- restriction of the mobility of the existing tumor;
- the appearance of a tumor emanating from the deep layers of soft tissues;
- the occurrence of swelling after a period of several weeks to 2-3 years or more after the injury.