Sarcoma of the abdominal wall, ICD code 10. Soft tissue sarcoma. Get treatment in Korea, Israel, Germany, USA

RCHR (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 the trunk, unspecified localization (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 soft tissue tumors account for 0.2-2.6% of the total structure malignant neoplasms person. Almost 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 identified diseases in 1993 was 235, in 2002 - 192.

Most often, these tumors are observed between the ages of 20 and 50 years. In children, sarcomas account for 10-11% of the cancer incidence rate. The predominant localization of soft tissue sarcomas is the extremities (up to 60%), with approximately 46% in the lower extremities and about 13% in the upper extremities. These tumors are localized on the body 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- C 49 (malignant tumors of soft tissues).

Abbreviations:

ECG - electrocardiography.

Ultrasound - ultrasound examination.

MTS - metastasis.

ESR - erythrocyte sedimentation rate.

RW - Wasserman reaction.

HIV is the human immunodeficiency virus.

PCT - polychemotherapy.

PET - positron emission tomography.

Date of development of the protocol: September 2011.

Protocol users: district oncologist, oncologist at the dispensary clinic, oncologist at the dispensary hospital.

Indication of no conflict of interest

We have no financial or other interest in the topic of the document under discussion. 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 Ewing sarcoma.

6. Primitive neuroectodermal tumor (PNET).

7. Fibrosarcoma.

8. Leiomyosarcoma.

9. Liposarcoma.

10. Malignant fibrous histiocytoma.

11. Malignant hemangiopericytoma.

12. Malignant mesenchymoma.

13. Malignant tumor arising from the peripheral nerve sheaths.

14. Rhabdomyosarcoma.

15. Synovial sarcoma.

16. Sarcoma not otherwise specified (NOS).

Histological types of tumors not included in the TNM classification: angiosarcoma, Kaposi's sarcoma, dermatofibrosarcoma, fibromatosis (desmoid tumor), sarcoma arising 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 location of the primary tumor. Involvement of regional lymph nodes is rare and when their status cannot be determined clinically or pathologically, they are classified as N0 instead of NX or pNX.

TNM classification

Classification rules. There must be histological confirmation of the diagnosis, allowing one to determine the histological type of tumor and the degree of malignancy.

Anatomical areas:

1. Connective tissue, subcutaneous and others soft fabrics(C 49), peripheral nerves (C 47).

2. Retroperitoneal space (C 48.0).

3. Mediastinum: anterior (C 38.1); rear (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 - the 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 in soft tissues. The appearance and increase of pain syndrome. Impaired movement in the limb.

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

Laboratory research: increased ESR, leukocytosis (if the process is widespread).

Instrumental studies:

1. Ultrasound examination of the affected area.

2. X-ray examination of organs chest.

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

List of basic and additional diagnostic measures:

Careful history taking;

Physical examination;

Blood type, Rh factor;

Wasserman reaction;

Complete blood test;

General analysis urine;

Biochemical blood test (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 the ilium (for Ewing's sarcoma);

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

For small or deep-lying tumors, trephine biopsy is performed under ultrasonographic or radiographic control;

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

With a knife biopsy, the incision should not complicate the subsequent choice of surgical option;
- cytological examination(does not replace histological verification of diagnosis):

Scraping smears from the surface of an ulcerated tumor;

Smear impressions of material taken using a knife or trepanobiopsy;
- ultrasound examination of organs abdominal cavity;

Arteriography (performed when the tumor is localized in the area of ​​passage of large great vessels);

PET - according to indications;

Skeletal scintigraphy according to indications.

Differential diagnosis

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Treatment

Goal 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 well-differentiated tumors (T1a), provided that radical surgery is possible. In other cases, treatment is combined or complex, the leading and decisive component of which is surgical removal of the tumor.

The treatment program is based on the histological degree of malignancy, the spread of the process, the size and location of the tumor.

Features of anesthesia support:

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

Trephine biopsy is performed under local anesthesia.

Non-drug treatment

Principles of surgical interventions:

The site of the previous biopsy is removed along with the tumor;

The sarcoma is removed without exposing the tumor;

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

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

Main types of operations for soft tissue sarcomas

Simple excision- used exclusively as a stage in the morphological diagnosis of 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 performed for deep-lying sarcomas of a high degree of malignancy. It involves the removal of the tumor and the normal tissues surrounding it, including in a single block the fascia and unchanged surrounding muscles, which are completely removed with cutting off at the attachment site. If necessary, resection of vessels, nerves, and bones is performed, simultaneously resorting to appropriate reconstructive plastic surgery on vessels, nerves, bones, and joints.

Organ-preserving and functionally sparing surgical interventions for locally advanced malignant tumors of the soft tissues of the extremities are performed exclusively as part of combined and complex treatment. Control of the radicality of surgical intervention is carried out by urgent intraoperative histological examination the edges of cutting off the tumor from normal tissues.

Amputations and disarticulations. Amputation and disarticulation of the limb are indicated in cases where radical saving surgery is not possible due to massive lesions (involvement of joints, bones, great vessels and nerves in the tumor process over a long distance) and/or when courses of neoadjuvant treatment are ineffective.

Radiation therapy

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

To uniformly deliver the radiation dose to the entire affected area, multifield irradiation techniques are used using devices to form optimal dose fields. The boundaries of the irradiation fields should exceed the size of the tumor by 3-4 cm. For large tumor sizes and/or a high degree of malignancy, the irradiation field should include up to 10 cm of tissue proximal and distal to the tumor boundaries. In this case, after reaching an SOD of 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 circumference of the limb 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 to preoperative radiation therapy are:

Lack of morphological confirmation of the diagnosis;

Disintegration of the tumor with the threat of bleeding;

General contraindications to radiation therapy.

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

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 surgery), the surrounding tissues with a 2 cm indentation from the cutting edges, and the postoperative scar (SD 60 Gy). If there is a residual tumor, which must be marked with titanium staples during surgery, this area is additionally locally irradiated to a dose of at least 70 Gy.

If the tumor is unresectable, radiation therapy is carried out according to a radical program with a dose of 70 Gy in the classical fractionation mode.

Treatment by stages

1. Stage IA (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 degree of malignancy): surgical removal of the tumor (T2a - wide excision, T2b - radical surgery) + 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 organ-conserving surgery, the treatment system includes methods of regional chemotherapy (intra-arterial administration of chemotherapy drugs).

5. If an extensive wound defect forms after surgical removal of a tumor, which cannot be eliminated by bringing the edges of the wound together, one of the types of primary plastic surgery is performed:

Free skin flap;

Local tissues;

Combined skin grafting;

Plastic surgery with displaced island 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.

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

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

When an extensive wound defect forms after surgical removal of a tumor, which cannot be eliminated by bringing the edges of the wound together, one of the types of primary plastic surgery is performed.

IIB stage(T2a N0, NX M0 - high degree of malignancy).

Stage III(T2b N0, NX M0 - high degree of malignancy):

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 sparing surgery) + 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 (iv or intra-arterial administration of chemotherapy);

When an extensive wound defect is formed, which cannot be eliminated by bringing the edges of the wound together, one of the types of primary plastic surgery is performed;

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

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

Complex 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 includes, in addition to intervention on the primary tumor (organ-preserving or organ-sapping surgery), a typical regional lymph node dissection, which is performed simultaneously with surgery on the primary tumor (single-block or stage-by-stage) 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 provided according to individual programs including polychemotherapy and/or radiation therapy;

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

Drug treatment

Polychemotherapy regimens

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2. SARO:

Cisplatin 100 mg/m2 IV, 1 day;

Doxorubicin 30 mg/m2 IV, days 2, 3, 4;

Vincristine 1.5 mg/m2 IV, day 5;

Cyclophosphamide 600 mg/m2 IV, day 6.

3. CyVADIC:

Dacarbazine 250 mg/m2 IV, days 1-5.

4. CyVADakt:

Cyclophosphamide 500 mg/m2 IV, day 2;

Vincristine 1 mg/m2 IV, days 1, 8, 15;

Doxorubicin 50 mg/m2 IV, 1 day;

Dactinomycin 0.3 mg/m2 IV, days 3/4/5.

Vincristine 1.5 mg/m2 IV, days 1, 8;

Doxorubicin 50 mg/m2 IV, 1 day;

Dacarbazine 250 mg/m2 IV, from 1 to 5 days.

Doxorubicin 60 mg/m2, 1 day;

Dacarbazine 250 mg/m2, days 1-5.

7.VAC-II:

Vincristine 1.5 mg IV, 1.8 days;

Sarcoma is a name that combines a large group of cancer tumors. Different types connective tissue under certain conditions they begin to undergo histological and morphological changes. Then the primary connective cells begin to grow rapidly, especially in children. From such a cell a tumor develops: benign or malignant with elements of muscles, tendons, and blood vessels.

Connective tissue cells divide uncontrollably, the tumor grows and, without clear boundaries, moves into the territory of healthy tissue. 15% of neoplasms whose cells are carried by blood throughout the body become malignant. As a result of metastasis, secondary growing oncological processes are formed, therefore it is believed that sarcoma is a disease characterized by frequent relapses. In terms of lethal outcomes, it ranks second among all cancers.

Is sarcoma cancer or not?

Some signs of sarcoma are the same as those of cancer. For example, it also grows infiltratively, destroys neighboring tissues, recurs after surgery, metastasizes early and spreads in organ tissues.

How does cancer differ from sarcoma?

• The cancer tumor has the appearance of a lumpy conglomerate that grows rapidly without symptoms in the first stages. Sarcoma is pinkish in color, reminiscent of fish flesh;
• a cancer tumor affects epithelial tissue, sarcoma affects muscle connective tissue;
• Cancer development occurs gradually in any specific organ in people after 40 years of age. Sarcoma is a disease of young people and children; it instantly affects their bodies, but is not tied to any one organ;
• Cancer is easier to diagnose, which increases cure rates. Sarcoma is more often detected at stages 3-4, so its mortality rate is 50% higher.

Is sarcoma contagious?

No, she is not contagious. An infectious disease develops from a real substrate that carries infection by airborne droplets or through the blood. Then a disease, such as influenza, can develop in the body of a new host. You can get sarcoma as a result of changes genetic code, chromosomal changes. Therefore, patients with sarcoma often have close relatives who have already been treated for any of its 100 types.

Sarcoma in HIV is a multiple hemorrhagic sarcomatosis called "angiosarcoma" or "". It is recognized by ulcerations of the skin and mucous membranes. A person becomes ill as a result of herpes infection type 8 through the lymph, blood, secretions of the patient’s skin and saliva, as well as through sexual contact. Even with antiviral therapy, Kaposi's tumor often recurs.

The development of sarcoma against the background of HIV is possible with sharp decline immunity. In this case, patients may be diagnosed with AIDS or a disease such as lymphosarcoma, leukemia, lymphogranulomatosis or myeloma.

Causes of sarcomas

Despite the variety of types, sarcoma is rare, accounting for only 1% of all cancers. The causes of sarcoma are varied. Among established reasons emit: exposure to ultraviolet (ionizing) radiation, radiation. Viruses and chemicals, precursors of the disease, also become risk factors. benign neoplasms, turning into oncological ones.

The causes of Ewing's sarcoma may be the rate of bone growth and hormonal levels. Risk factors such as smoking, working in chemical plants, and contact with chemicals are also important.

Most often, this type of oncology is diagnosed due to the following risk factors:

• hereditary predisposition and genetic syndromes: Werner, Gardner, multiple pigmented skin cancer of basal cells, neurofibromatosis or retinoblastoma;
• herpes virus;
• lymphostasis of the legs chronic form, whose recurrence occurred after radial mastectomy;
• injuries, wounds with suppuration, exposure to cutting and piercing objects (shards of glass, metal, wood chips, etc.);
• immunosuppressive and polychemotherapy (10%);
• organ transplant operations (in 75% of cases).

Informative video

General symptoms and signs of the disease

Signs of sarcoma appear depending on its location in the vital important organs. Affects the nature of symptoms biological features the root cause of the cell and the tumor itself. Early signs of sarcoma are the noticeable size of the formation, as it grows rapidly. Pain in joints and bones appears early (especially at night), which is not relieved by analgesics.

For example, due to the growth of rhabdominosarcoma, the oncological process spreads to the tissues of healthy organs and is manifested by various pain symptoms and hematogenous metastasis. If sarcoma develops slowly, signs of the disease may not appear for several years.

Symptoms of lymphoid sarcoma are reduced to the formation of oval or round nodes and small swellings in the lymph node. But even with sizes of 2-30 cm, a person may not feel pain at all.

With other types of tumors with rapid growth and progression, the following may appear: fever, veins under the skin, and cyanotic ulcerations on them. Palpation of the formation reveals that it has limited mobility. The first signs of sarcoma are sometimes characterized by deformation of the joints of the limbs.

Liposarcoma, along with other types, can be of a primary multiple nature with sequential or simultaneous manifestation in different areas of the body. This significantly complicates the search for the primary tumor that produces metastases.

Symptoms of sarcoma located in soft tissues are expressed in painful sensations upon palpation. Such a tumor has no outline, and it quickly penetrates into nearby tissues.

With a pulmonary oncological process, the patient suffers from shortness of breath, which causes oxygen starvation of the brain, pneumonia, pleurisy, dysphagia may begin, and the right side of the heart may enlarge.

Cells of the nerve sheaths degenerate into neurofibrosarcoma, cells and connective tissue fibers into. Spindle cell sarcoma, consisting of large cells, affects the mucous membranes. Mesothelioma grows from the mesothelium of the pleura, peritoneum and pericardium.

Types of sarcomas by location

Types of sarcoma are distinguished depending on their location.

AND Of the 100 types, sarcomas most often develop in the area:

• peritoneum and retroperitoneal space;
• neck, head and bones;
• mammary glands and uterus;
• stomach and intestines (stromal tumors);
• fatty and soft tissues of the limbs and trunk, including desmoid fibromatosis.

New ones are especially often diagnosed in fatty and soft tissues:

• , developing from adipose tissue;
• , which refers to fibroblastic/myofibroblastic formations;
• fibrohistiocytic tumors of soft tissues: plexiform and giant cell;
• – from smooth muscle tissue;
• glomus tumor (pericytic or perivascular);
• from the muscles of the skeleton;
• and epithelioid hemangioepithelioma, which refers to vascular formations of soft tissue;
• mesenchymal chondrosarcoma, extraskeletal osteosarcoma – osteochondral tumors;
• malignant gastrointestinal tract tumor (stromal tumor of the gastrointestinal tract);
• tumors of the nerve trunk: peripheral nerve trunk, triton tumor, granulocelloma, ectomesenchymoma;
• sarcomas of unclear differentiation: synovial, epithelioid, alveolar, clear cell, Ewing, desmoplastic round cell, intimal, PEComu;
• undifferentiated/unclassified sarcoma: spindle cell, pleomorphic, round cell, epithelioid.

According to the WHO classification (ICD-10), the following tumors are often found among bone tumors:

• cartilage tissue - chondrosarcoma: central, primary or secondary, peripheral (periosteal), clear cell, dedifferentiated and mesenchymal;
• bone tissue - osteosarcoma, a common tumor: chondroblastic, fibroblastic, osteoblastic, as well as telangiectatic, small cell, central low-grade, secondary and paraosteal, periosteal and superficial high-grade;
• fibrous tumors – fibrosarcoma;
• fibrohistiocytic formations - malignant fibrous histiocytoma;
• / PNET;
• hematopoietic tissue - plasmacytoma (myeloma), malignant lymphoma;
• giant cell: malignant giant cell;
• tumor formation of the notochord - “Dedifferentiated” (sarcomatoid);
• vascular tumors – angiosarcoma;
• smooth muscle tumors – leiomyosarcoma;
• tumors from adipose tissue - liposarcoma.

The maturity of all types of sarcomas can be low-, medium- and highly differentiated. The lower the differentiation, the more aggressive the sarcoma. Treatment and survival prognosis depend on the maturity and stage of formation.

Stages and degrees of malignant process

There are three degrees of malignancy of sarcoma:

1. Poorly differentiated degree, in which the tumor consists of more mature cells and the process of their division occurs slowly. It is dominated by stroma - normal connective tissue with a small percentage of oncoelements. The tumor rarely metastasizes and recurs little, but can grow to large sizes.
2. A highly differentiated degree in which tumor cells divide rapidly and uncontrollably. With rapid growth, a sarcoma forms a dense vascular network with a large number of high-grade cancer cells, and metastases spread early. Surgery high degree of education may be ineffective.
3. Moderately differentiated degree, in which the tumor has intermediate development, and with adequate treatment a positive prognosis is possible.

The stages of sarcoma do not depend on its histological type, but on its location. It determines the stage based on the condition of the organ where the tumor began to develop.

The initial stage of sarcoma is characterized by small size. It does not spread beyond the organs or segments where it originally appeared. There are no disturbances in the working functions of organs, compression, or metastasis. There is virtually no pain. If a well-differentiated stage 1 sarcoma is detected, complex treatment achieve positive results.

Signs initial stage sarcomas, depending on the location in a specific organ, for example, the following:

• in the oral cavity and on the tongue - a small node up to 1 cm in size appears in the submucosal layer or mucous membrane and has clear boundaries;
• on the lips – the knot is felt in the submucosal layer or inside the lip tissue;
• in the cellular spaces and soft tissues of the neck - the size of the node reaches 2 cm, it is located in the fascia that limits its location and does not extend beyond its limits;
• in the area of ​​the larynx - the mucous membrane or other layers of the larynx limit the node, up to 1 cm in size. It is located in the fascial sheath, does not extend beyond it and does not interfere with phonation and breathing;
• V thyroid glands e – a node, up to 1 cm in size, is located inside its tissues, capsule germination does not occur;
• in the mammary gland - a node up to 2-3 cm grows in the lobule and does not extend beyond its limits;
• in the area of ​​the esophagus - an oncological node up to 1-2 cm is located in its wall, without disturbing the passage of food;
• in the lung - manifested by damage to one of the segments of the bronchi, without going beyond its limits and without disrupting the working function of the lung;
• in the testicle - a small node develops without involving the tunica albuginea;
• in the soft tissues of the extremities - the tumor reaches 5 cm, but is located within the fascial sheaths.

Stage 2 sarcoma is located inside the organ, grows into all layers, disrupts the functional functioning of the organ as it increases in size, but there is no metastasis.

The oncological process manifests itself like this:

• in the oral cavity and on the tongue - noticeable growth in the thickness of the tissues, germination of all membranes, mucosa and fascia;
• on the lips - by germination of the skin and mucous membranes;
• in the cellular spaces and soft tissues of the neck - up to 3-5 cm in height, extending beyond the fascia;
• in the area of ​​the larynx – the growth of the node is more than 1 cm, the germination of all layers, which disrupts phonation and breathing;
• in the thyroid gland – the growth of the node is more than 2 cm and the involvement of the capsule in the oncological process;
• in the mammary gland – node growth up to 5 cm and germination of several segments;
• in the esophagus - by germination of the entire thickness of the wall, including the mucous and serous layers, involvement of the fascia, severe dysphagia (difficulty swallowing);
• in the lungs - compression of the bronchi or spread to the nearest pulmonary segments;
• in the testicle - by germination of the tunica albuginea;
• in the soft tissues of the extremities - by the germination of fascia limiting the anatomical segment: muscle, cellular space.

At the second stage, when the tumor is removed, the excision area is expanded, so relapses are not frequent.

Stage 3 sarcoma is characterized by invasion of the fascia and nearby organs. Metastasis of the sarcoma to regional lymph nodes occurs.

The third stage appears:

• large in size, expressed pain syndrome, disruption of normal anatomical relationships and chewing in the oral cavity and tongue, metastases in the lymph nodes under the jaw and on the neck;
• large in size, deforming the lip, spreading through the mucous membranes and metastases to the lymph nodes under the jaw and on the neck;
• dysfunction of organs located along the neck: innervation and blood supply, swallowing and respiratory functions are disrupted in soft tissue sarcoma of the neck and cellular spaces. As the tumor grows, it reaches blood vessels, nerves and nearby organs; metastases reach the lymph nodes of the neck and sternum;
• sudden respiratory failure and distortion of the voice, germination into organs, nerves, fascia and vessels in the neighborhood, metastasis from laryngeal cancer to the superficial and deep lymphatic cervical collectors;
• in the mammary gland - large in size, which deforms the mammary gland and metastasizes to the lymph nodes under the armpits or above the collarbone;
• in the esophagus - huge in size, reaching the mediastinal tissue and disrupting the food passage, metastases in the mediastinal lymph nodes;
• in the lungs – compression of the bronchi with large sizes, metastases in the mediastinal and peribronchial lymph nodes;
• in the testicle – deformation of the scrotum and germination of its layers, metastasis to the groin lymph nodes;
• in the soft tissues of the arms and legs - tumor foci measuring 10 centimeters. As well as dysfunction of the limbs and tissue deformation, metastases to regional lymph nodes.

At the third stage, extended surgical interventions are performed, despite this, the frequency of sarcoma relapses increases, and treatment results are ineffective.

Stage 4 sarcoma is very difficult, the prognosis after its treatment is the most unfavorable due to its gigantic size, sharp compression of surrounding tissues and germination into them, the formation of a continuous tumor conglomerate, which is prone to bleeding. Often there is a relapse of sarcoma of soft tissues and other organs after surgery or even complex treatment.

Metastasis reaches regional lymph nodes, liver, lungs, and bone marrow. It stimulates a secondary oncological process - the growth of a new sarcoma.

Metastases in sarcoma

The routes of metastasis of sarcoma can be lymphogenous, hematogenous and mixed. From the pelvic organs, intestines, stomach and esophagus, larynx, sarcoma metastases reach the lungs, liver, skeletal bones and other organs through the lymphatic pathway.

Through the hematogenous route (through venous and arterial vessels), tumor cells or metastases also spread to healthy tissues. But sarcomas, for example, of the mammary and thyroid glands, pulmonary, bronchial, and from the ovaries, spread by lymphogenous and hematogenous routes.

It is impossible to predict the organ where elements of the microvasculature will accumulate and the growth of a new tumor will begin. Dust metastases of sarcoma of the stomach and pelvic organs spread throughout the peritoneum and thoracic region with hemorrhagic effusion - ascites.

The oncological process on the lower lip, tip of the tongue and in the oral cavity metastasizes more to the lymph nodes of the chin and under the jaw. Formations in the root of the tongue, at the bottom of the oral cavity, in the pharynx, larynx, and thyroid gland metastasize to the lymph nodes of the vessels and nerves of the neck.

From the mammary gland, cancer cells spread to the clavicle area, to the lymph nodes on the outside of the sternocleidomastoid muscle. From the peritoneum they reach the inner side of the sternocleidomastoid muscle and can be located behind or between its legs.

Most metastasis occurs in adults, lymphosarcoma, liposarcoma, fibrous histiocytoma, even with a size of up to 1 cm due to the accumulation of calcium in the tumor focus, intense blood flow and active growth of cancer cells. These formations lack a capsule that could limit their growth and reproduction.

The course of the oncological process does not become more complicated, and its treatment in connection with metastases to regional lymph nodes will not be so global. With distant metastases in the internal organs, on the contrary, the tumor grows to a large size, there may be several of them. Treatment becomes more complicated; complex therapy is used: surgery, chemotherapy and radiation. As a rule, single metastases are removed. Excision of multiple metastases is not performed; it will not be effective. Primary foci differ from metastases in a large number of vessels and cell mitoses. Metastases have more necrotic areas. Sometimes they are found earlier than the primary focus.

The consequences of sarcoma are as follows:

• surrounding organs are compressed;
• obstruction or perforation may occur in the intestine, peritonitis - inflammation of the abdominal layers;
• elephantiasis occurs due to impaired lymph outflow due to compression of the lymph nodes;
• limbs are deformed and movement is limited in the presence of large tumors in the area of ​​bones and muscles;
• Internal hemorrhages occur during the disintegration of tumor formation.

Diagnosis of sarcomas

Diagnosis of sarcoma begins in the doctor's office, where it is determined by external diagnostic signs: exhaustion, jaundice, pale skin color and change in its color over the tumor, cyanotic tint of the lips, swelling of the face, congestion of the veins on the surface of the head, plaques and nodules in cutaneous sarcoma.

Diagnosis of high-grade sarcoma is carried out by pronounced symptoms of intoxication of the body: decreased appetite, weakness, elevated body temperature and sweating at night. Cases of cancer in the family are taken into account.

When conducting laboratory tests research:

• biopsy by histological method under a microscope. In the presence of tortuous thin-walled capillaries, multidirectional bundles of atypical cancer cells, altered large-nucleated cells with thin shell, large quantity substances between cells containing cartilaginous or hyaline connective tissue substances, histology diagnoses sarcoma. At the same time, the nodes lack normal cells characteristic of organ tissue.
• abnormalities in the chromosomes of oncological cells using the cytogenetic method.
• There are no specific blood tests for tumor markers, so there is no way to unambiguously determine its type.
• General blood test: for sarcoma will show the following abnormalities:

1. hemoglobin and red blood cell levels will decrease significantly (less than 100 g/l), which indicates anemia;
2. the level of leukocytes will increase slightly (above 9.0x109/l);
3. the platelet count will decrease (less than 150․109/l);
4. ESR will increase (above 15 mm/hour).

• biochemical blood test, it determines increased level lactate dehydrogenase. If the enzyme concentration is above 250 U/l, then we can talk about the aggressiveness of the disease.

Diagnosis of sarcoma is complemented by a chest x-ray. The method can detect a tumor and its metastases in the sternum and bones.

Radiological signs of sarcoma are as follows:

• the tumor is round or irregular in shape;
• the size of the formation in the mediastinum ranges from 2-3 mm to 10 cm or more;
• the structure of the sarcoma will be heterogeneous.

X-ray is necessary to detect pathology in lymph nodes: one or more. In this case, the lymph nodes on the x-ray will be darkened.

If sarcoma is diagnosed by ultrasound, it will be characteristic, for example:

• heterogeneous structure, uneven scalloped edges and lymph node damage - with lymphosarcoma in the peritoneum;
• absence of a capsule, compression and expansion of surrounding tissues, foci of necrosis inside the tumor - with sarcoma in the organs and soft tissues of the abdominal cavity. Nodes will be noticeable in the uterus and kidneys (inside) or in the muscles;
• formations of different sizes without borders and with foci of decay inside them - with skin sarcoma;
• multiple formations, heterogeneous structure and metastases of the primary tumor - with fatty sarcoma;
• heterogeneous structure and cysts inside, filled with mucus or blood, unclear edges, effusion in the cavity of the joint capsule - with sarcoma of the joints.

Tumor markers for sarcoma are determined in each specific organ, as in cancer. For example, for ovarian cancer - for sarcoma of the breast - for gastrointestinal tract - CA 19-9 or lung - ProGRP (precursor of gastrin releasing peptide), etc.

Computed tomography is performed with the introduction of an x-ray contrast agent to determine the location, boundaries of the tumor and its shape, damage to surrounding tissues, vessels, lymph nodes and their fusion into conglomerates.

Magnetic resonance imaging is performed to identify the exact size, metastases, destruction of the skin, bones, tissues, fibering of the periosteum, thickening of the joints, etc.

The diagnosis is confirmed by a biopsy and malignancy is determined by histological examination:

• bundles of tangled spindle-shaped cells;
• hemorrhagic exudate - fluid coming out of the walls of blood vessels;
• hemosiderin - a pigment formed during the breakdown of hemoglobin;
• giant atypical cells;
• mucus and blood in the sample, etc.

A lumbar puncture (spinal puncture) test will indicate sarcoma, where there may be traces of blood and many atypical cells of different sizes and shapes.

Do not remove:

• after 75 years;
• for severe diseases of the heart, kidneys and liver;
• with a large tumor in vital organs that cannot be removed.

The following treatment tactics are also used:

1. For low- and moderately differentiated sarcomas at stages 1-2, operations and regional lymph node dissection are performed. After – polychemotherapy (1-2 courses) or external beam radiation therapy for sarcoma.
2. For highly differentiated sarcomas at stages 1-2, surgical treatment and extended lymph node dissection. is performed before and after surgery, and in case of complex treatment it is added.
3. At the third stage of the oncological process, combined treatment is carried out: before surgery - radiation and chemotherapy to reduce the size of the tumor. During the operation, all growing tissues and regional lymphatic drainage collectors are removed. Restore important damaged structures: nerves and blood vessels.
4. Amputation is often required for sarcoma, especially osteosarcoma. Resection of the bone area is performed for poorly differentiated superficial osteosarcomas in older people. Next, prosthetics are performed.
5. At stage 4, symptomatic treatment is used: correction of anemia, detoxification and analgesic therapy. For comprehensive, complete treatment at the last stage, access to the tumor formation is required for the purpose of its removal, small size, location in the superficial layers of tissue, and single metastases.

Modern methods include external beam radiation therapy with linear accelerators using special programs that plan irradiation fields and calculate the power and dose of exposure to the oncological process area. Radiotherapy is carried out under full computerized control and automatic verification of the correctness of the settings specified on the accelerator control panel to eliminate human error. used for sarcomas different localization. She irradiates the tumor with precision high dose radiation without damaging healthy tissue. The source is inserted into it by remote control. Brachytherapy can replace surgery and external radiation in some cases.

Traditional medicine for sarcoma

Treatment of sarcoma folk remedies included in complex therapy. For each type of sarcoma there is its own medicinal herb, mushrooms, resins, and food products. Diet during oncological process has great importance, since fortified foods and the presence of micro- and macroelements increase immunity, give strength to fight cancer cells, and prevent metastasis.

For malignant sarcomas, treatment is carried out:

• infusions;
• alcohol tinctures;
• decoctions;
• poultices.

Herbs used:

• black henbane;
• spotted hemlock;
• oxwort;
• cocklebur;
• white water lily;
• clematis;
• poppy samosa;
• sun milk,
• red fly agaric;
• knotty burrow;
• white mistletoe;
• incense pikulnik;
• evasive peony;
• European wormwood;
• common hop;
• common blackhead;
• greater celandine;
• saffron sativa;
• tall ash.

For old sarcoma ulcers on the skin and mucous membranes, treat with auranium officinalis, cutaneous sarcoma with wolfberry, sweet clover, cocklebur, kirkazon and clematis, milkweed and bittersweet nightshade, common tansy and European dodder, common hops and medicinal garlic ym.

During an oncological process in organs, the following drugs will be needed:

• in the stomach - from wolfberry, black crow, datura and cocklebur, peony, greater celandine and wormwood;
• V duodenum– from aconites, swamp whiteweed;
• in the esophagus - from spotted hemlock;
• in the spleen - from wormwood;
• in the prostate gland - from spotted hemlock;
• in the mammary gland - from spotted hemlock, leftwort and common hop;
• in the uterus - from evasive peony, wormwood, Lobel's hellebore and saffron;
• in the lungs - from the magnificent crocus and cocklebur.

Osteogenic sarcoma is treated with a tincture: crushed St. John's wort (50 g) is poured with grape vodka (0.5 l) and infused for two weeks with daily shaking of the container. Before meals, take 30 drops 3-4 times.

For sarcoma, folk remedies are used according to the method of M.A. Ilves (from the book “The Red Book of the White Land”):

1. To increase immunity: mix in equal weight fractions: tartar (flowers or leaves), calendula flowers, tricolor and field violet, cocklebur, chamomile flowers and speedwell, celandine and immortelle flowers, mistletoe and young burdock root. Brew 2 tbsp. l. collect 0.5-1 liters of boiling water and leave for 1 hour. Drink during the day.
2. Divide the herbs from the list into 2 groups (5 and 6 names each) and drink each collection for 8 days.

Important! When collected, plants such as celandine, violet, cocklebur and mistletoe are poisonous. Therefore, the dose cannot be exceeded.

To eliminate cancer cells, treatment of sarcoma with folk remedies includes the following Ilves recipes:

• grind the celandine in a meat grinder and squeeze out the juice, mix with vodka in equal parts (preserve) and store at room temperature. Drink 3 times a day, 1 tsp. with water (1 glass);
• Grind 100 g of marina root (peony evasive) and add vodka (1 l) or alcohol (75%), leave for 3 weeks. Take 0.5-1 tsp. 3 times with water;
• Grind the mistletoe, place it in a jar (1 liter) 1/3 full, fill it to the top with vodka and let it brew for 30 days. Separate the grounds and squeeze, drink 1 tsp. 3 times with water;
• chop meadowsweet root – 100 g and add vodka – 1 liter. Leave for 3 weeks. Drink 2-4 tsp. 3 times a day with water.

The first three tinctures should be alternated every 1-2 weeks. Meadowsweet tincture is used as a reserve. All tinctures are taken for the last time before the evening dinner. Course - 3 months, in the break between the monthly course (2 weeks) - drink meadowsweet. At the end of the 3-month course, drink meadowsweet or one of the tinctures once a day for another 30 days.

Nutrition for sarcoma

The diet for sarcoma should consist of the following products: vegetables, herbs, fruits, fermented milk rich in bifido- and lactobacteria, boiled (steamed, stewed) meat, cereals as a source complex carbohydrates, nuts, seeds, dried fruits, bran and sprouted cereals, wholemeal bread, cold-pressed vegetable oils.

To block metastases, the diet includes:

• fat sea ​​fish: saury, mackerel, herring, sardine, salmon, trout, cod;
• green vegetables and yellow color: zucchini, cabbage, asparagus, green peas, carrots and pumpkin;
• garlic.

You should not eat confectionery products, since they are stimulators of cancer cell division, as well as sources of glucose. Also products with tannin: persimmon, coffee, tea, bird cherry. Tannin, as a hemostatic agent, promotes thrombus formation. Smoked meats are excluded as sources of carcinogens. You should not drink alcohol or beer, the yeast of which feeds cancer cells with simple carbohydrates. Avoid acidic berries: lemons, lingonberries and cranberries, since cancer cells actively develop in an acidic environment.

Prognosis for life with sarcoma

The five-year survival rate for sarcoma of soft tissues and extremities can reach 75%, and up to 60% for cancer on the body.
In fact, even the most experienced doctor. According to research, life expectancy for sarcoma is influenced by the forms and types, stages of the oncological process, and the general condition of the patient. With adequate treatment, a positive prognosis is possible for the most hopeless cases.

Disease prevention

Primary prevention of sarcoma involves active identification of patients at increased risk of developing the disease, including those infected with herpes virus VIII (HHV-8). It is especially necessary to carefully monitor patients receiving. Prevention involves eliminating and treating the conditions and diseases that cause sarcoma.

Secondary prevention is carried out for patients in remission to prevent relapse of sarcoma and complications after a course of treatment. As a preventive measure, instead of tea, you should drink brewed herbs according to the Ilves method (step 1) for 3 months, take a break of 5-10 days and repeat the intake. You can add sugar or honey to tea.

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

Code by international classification diseases ICD-10:

Histogenesis. The source of growth is the most heterogeneous tissues in structure and origin. Basically, these are derivatives of mesenchyme: fibrous connective, adipose, synovial and vascular tissues, as well as tissues associated with mesoderm (striated muscles) and neuroectoderm (nerve sheaths). It should be taken into account that every third soft tissue tumor cannot be classified using conventional microscopy due to the difficulty of determining histogenesis. In such cases, immunohistochemical examination 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: .. Transversely striated muscles - rhabdomyosarcoma.. Smooth muscles - leiomyosarcoma. Synovial tissue - synovial sarcoma. Nerve sheaths: .. Neuroectodermal - malignant neuroma (schwannoma).. Connective tissue - perineural fibrosarcoma. Unclassified blastomas.

Causes

Risk factors. Ionizing radiation. Action chemical substances(eg asbestos or wood preservatives). Genetic disorders. For example, 10% of patients with von Recklinghausen disease develop neurofibrosarcoma. Pre-existing bone diseases. Osteosarcoma develops in 0.2% of patients with Paget's disease (ostosis deformans).

Symptoms (signs)

Clinical picture. Sarcomas can develop anywhere on the trunk or extremities and usually appear as a painless tumor of varying consistency and density. Sarcomas that arise in the deep parts of the thigh and retroperitoneum usually reach large sizes by the time of diagnosis. Patients usually note a decrease in body weight and complain of pain of unknown localization. In the distal parts of the 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. Rapid growth, 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 about 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.. Trephine biopsy or excisional biopsy makes it possible to establish final diagnosis to select a treatment method. When choosing a biopsy site, you should consider possible holding subsequently reconstructive (plastic) surgery. Radiological examination: radiography, bone scintigraphy, MRI, CT.. For some types of sarcomas and when planning organ-conserving surgery, MRI is preferable - diagnostics that provide a more accurate determination of the boundary between tumors and soft tissues.. CT and bone scintigraphy are preferable for detecting bone lesions.. For signs of liver dysfunction in sarcomas internal organs or extremities, ultrasound and CT are performed (to identify metastases).. If vascular proliferation is suspected, contrast angiography is indicated.

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 lesion. The depth of location in the classification is taken into account as follows: ... Superficial - “a” - the tumor does not involve the (most) superficial muscular fascia... Deep - “b” - the tumor reaches or grows into the (most) superficial muscular fascia. This includes all visceral tumors and/or tumors, invading large vessels, and intrathoracic lesions. Most head and neck tumors 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 - there are metastases in the regional lymph nodes nodes.. Distant metastases... M1 - there are distant metastases.

Grouping by stages: .. Stage IA - G1 - 2T1a - 1bN0M0 - well-differentiated, small-sized tumors, regardless of location.. Stage IB - G1 - 2T2aN0M0 - well-differentiated, large-sized 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 the criterion for an adult, but the choice of treatment method must be chosen by consultation. For example, rhabdomyosarcoma can be successfully treated using pediatric regimens up to the age of 25, but poorly differentiated fibrosarcoma at the age of 14 should be treated as in an adult - surgically.

Tumors of the extremities and superficially located tumors of the torso are subject to surgical removal using the principles of “sheathing”. Possible skin deficiency is not an obstacle to intervention. If the tumor is presenting to the bone, it is removed along with the periosteum, and if it grows, a planar or segmental resection of the bone is performed. When microscopic detection of malignant cells at the edges of the resected tissue, the muscle-fascial sheath is resected. Radiation therapy is indicated when the tumor edge is located less than 2-4 cm from the resection line or when the wound is contaminated with tumor cells.

Tumors of the posterior mediastinum, retroperitoneal in the pelvis and paravertebral, are often unremovable. Small tumors of the anterior mediastinum and retroperitoneal tumors in the left half of the body can be removed surgically. For doubtfully operable tumors, preoperative radiation or thermoradiotherapy, regional chemotherapy, and chemoembolization of the vessels feeding the tumor are performed. Since tumors of these locations are more often detected in late stages and radical removal often fails, the operation is supplemented with radiation therapy. If relapses develop, repeated interventions are indicated.

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

Peculiarities therapeutic measures depends on histological structure tumors. Neurogenic sarcoma and fibrosarcomas are insensitive to radiation and chemotherapy; treatment (including relapses) is only surgical. Angiosarcoma and liposarcoma are relatively sensitive to radiation therapy (preoperative telegammatherapy is required). Myogenic and synovial sarcomas require neoadjuvant chemotherapy and radiation therapy.

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 initial operation.

In case of complications of tumor growth, it is possible to perform palliative resections, which can reduce intoxication, blood loss from disintegrating tumors, and eliminate symptoms of compression of other organs (ureteral obstruction, bowel compression 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 varying amounts of collagen and reticular fibers. Clinical picture.. Localization - soft tissues of the extremities (thigh, shoulder girdle), less often the trunk, head, neck.. The most important sign is the absence of 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, observing zonality and casing. Forecast. With adequate treatment, the 5-year survival rate is 77%.

Liposarcoma is 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 lower limbs and in the retroperitoneal space.. It is extremely rare that liposarcomas develop from single and multiple lipomas.. Typically, early hematogenous metastasis to the lungs (30-40%). Treatment is wide excision; for larger tumors, preoperative radiation therapy is warranted. Forecast. In patients with differentiated tumors, the 5-year survival rate is 70%, with poorly differentiated tumors - 20%.

Rhabdomyosarcoma- a malignant tumor originating from skeletal (striated) muscle. There are embryonic (develops up to 15 years) and adult types of rhabdomyosarcoma. Frequency. It ranks 3rd 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 round cells, in the cytoplasm of which longitudinal and transverse striations are determined. Genetic aspects. The development of rhabdomyosarcomas is expected to involve several genes located on the ridge. 1, 2, 11, 13 and 22; the possible role of genomic imprinting or duplication of individual genes (for example, the insulin-like growth factor 2 gene IGF2, the PAX3 and PAX7 genes) is being considered.

Clinical picture.. Most often, tumors are localized in three anatomical areas of the body: limbs, head and neck, pelvis.. The tumor grows quickly, without pain and dysfunction of organs.. They often invade the skin with the formation of exophytic bleeding formations.. Early recurrence is characteristic. Treatment— surgical; for large tumors, preoperative radiation therapy is advisable. When carrying out combined (surgical, chemotherapy) treatment of localized forms of embryonal rhabdomyosarcoma, an increase in 5-year survival rate of up to 70% is noted. In the presence of metastases, the 5-year survival rate is 40%. For pleomorphic rhabdomyosarcoma (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 (under 40 years of age). Morphology.. Hemangioendothelioma is formed from many atypical capillaries with proliferation of atypical endothelial cells filling the lumen of the vessels.. Hemangiopericytoma, developing from modified cells of the outer lining of the 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(Stewart-Treves syndrome) is a specific tumor that develops in the area of ​​constant lymphatic edema (upper limb in women with postmastectomy syndrome, especially after a course of radiation therapy). The prognosis is unfavorable.

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

Synovial sarcoma It ranks 3rd-4th in frequency among soft tissue sarcomas (8%). It is registered mainly in people under 50 years of age. The tumor consists of juicy spindle-shaped and round 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 rather 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.. Primary tumor multiplicity is characteristic.. Recurrence is possible. Treatment is surgical. Prognosis. The main prognostic factors are the degree of histological differentiation and tumor size; in patients with low-grade neuroma, the prognosis is less favorable. Tumor size is an independent prognostic factor. Small (less than 5 cm) completely removed well-differentiated tumors rarely recur and metastasize.

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

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

Soft tissue sarcomas occur in any part of the body. In about half, the tumor is localized on the lower extremities. The hip is most commonly affected. In 25% of patients, sarcoma is located on upper limbs. The rest are on the torso and occasionally on the head.
Malignant tumor soft tissue has the appearance of a rounded node of whitish or yellowish-gray color with a bumpy or smooth surface. The consistency of the neoplasm depends on the histological structure. It can be dense (fibrosarcomas), soft (liposarcomas and angiosarcomas) and even jelly-like (myxomas). Soft tissue sarcomas do not have a true capsule, however, during the process of growth, the neoplasm compresses the surrounding tissues, the latter become denser, forming a so-called false capsule, which quite clearly outlines the tumor.
A malignant neoplasm usually occurs in the thickness of the deep layers of muscle. As the tumor increases in size, it gradually spreads to the surface of the body. Growth is accelerated under the influence of injury and physical therapy.
The tumor is usually single, but some types of sarcomas are characterized by multiple lesions. Sometimes they occur at a great distance from each other (multiple liposarcoma, malignant neuromas in Recklinhausen disease).
Metastasis of malignant neoplasms of soft tissue occurs predominantly through the hematogenous route (through blood vessels). The favorite location of metastases is the lungs. Less commonly, the liver and bones are affected. Metastases to 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 abdominal wall, intermuscular or embryonal lipomas and fibromas, differentiated fibrosarcomas i).
The leading sign is the appearance of a painless node or swelling of a round or oval shape. The size of the node varies from 2–3 to 25–30. The nature of the surface depends on the type of tumor. The boundaries of the tumor in the presence of a pronounced false capsule are clear, but with a deep tumor, the contours of the swelling are unclear and difficult to define. The skin is usually not changed, but compared to the healthy side above the tumor there is a local increase in temperature, and with massive, rapidly growing formations that reach the surface, 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 serves as one of the most characteristic and important symptoms for diagnosis.
Occasionally, soft tissue sarcomas lead to deformation of the limbs, causing a feeling of heaviness and awkwardness when moving, but the function of the limb is rarely impaired.
“Alarm signals”, in the presence of which a suspicion of soft tissue sarcoma should be raised, are:
- the presence of a gradually increasing tumor formation;
- limitation of the mobility of the existing tumor;
- the appearance of a tumor emanating from the deep layers of soft tissues;
- the appearance of swelling after a period of several weeks to 2–3 years or more after the injury.

Soft tissues are all non-epithelial extraskeletal tissues of the body, with the exception of the endothelial system and supporting tissues of internal organs (WHO, 1969). The term “soft tissue sarcomas” is due to the origin of these tumors from connective tissue outside the skeletal boundaries.

Epidemiology.

There are no complete and accurate statistical data on morbidity and mortality from this pathology. The majority of patients (up to 72%) are under the age of 30 years, with 30% occurring in children under 15 years of age. Most often, these tumors affect the extremities. According to world statistics, the proportion of sarcomas in different countries accounts for 1-3% of all malignant neoplasms in adults.

Etiology.

· Action of ionizing radiation.

· Exposure to chemicals (eg asbestos or wood preservatives).

· Genetic disorders. For example, 10% of patients with Recklinghausen disease will develop neurofibrosarcoma during their lifetime.

· Pre-existing bone diseases. In 0.2% of people with the disease Paget(osteitis deformans) osteosarcomas develop.

Pathological anatomy.

Over 30 histological types of soft tissue sarcomas have been described. These are tumors of mesodermal and, less commonly, neuroectodermal origin, which are found in different places bodies where their maternal tissue is located. The morphological classification developed by WHO experts (Geneva, 1974) covers 15 types of tumor process according to histogenesis.

Histogenetic classification

Mesenchyme

Malignant mesenchioma

Fibrous tissue

Desmoid (invasive form)

Fibrosarcoma

Adipose tissue

Liposarcoma

Vascular tissue

Malignant hemangioendothelioma

Malignant hemangiopericytoma

Malignant lymphangiosarcoma

Muscle

Rhabdomyosarcoma - from striated muscles

Leiomyosarcoma - from smooth muscle

Synovial tissue

Synovial sarcoma

Nerve sheaths

Malignant neuroma (schwannoma) - from neuroectodermal membranes

Perineural fibrosarcoma - from connective tissue membranes

Unclassified sarcomas. In terms of frequency, liposarcomas occupy the first place (up to 25% of cases). Further rankings are malignant fibrous histiocytoma, unclassified, synovial and rhabdomyosarcoma (17 to 10%, respectively). Other histological types of soft tissue sarcomas are relatively rare. Soft tissue sarcomas are characterized by pronounced local aggressive infiltrative growth, including invasion into surrounding structures, and the ability for early hematogenous metastasis. Metastases to regional lymph nodes occur rarely (5-6% of patients) and indicate dissemination of the process. Regional lymph nodes are nodes that correspond to specific locations of the primary tumor.

Classification of soft tissue tumors

( code ICD - O S 38.1, 2; WITH 47-49) according to the system TNM(5th edition, 1997).

TNM Clinical classification

T - Primary tumor

T x- insufficient data to evaluate the primary tumor

T 0- the primary tumor is not determined

Tis- carcinoma in situ

T 1 - tumor no larger than 5 cm in greatest dimension

T 1a - superficial tumor *

T 1b - deep tumor *

T 2- tumor larger than 5 cm in greatest dimension

T 2a - superficial tumor *

T 2b - deep tumor *

Note: *Superficial tumor is located exclusively over the superficial fascia without fascial invasion; a deep tumor is located either exclusively under the superficial fascia, or superficial to the fascia with invasion of the fascia, or with penetration through it. Retroperitoneal, mediastinal and pelvic sarcomas are classified as deep tumors

N - Regional lymph nodes.

Nx- there is not enough data to assess the condition of regional lymph nodes

N 0- no signs of damage to regional lymph nodes

N 1- existing metastases in regional lymph nodes

M - Distant metastases

M x- insufficient data to determine distant metastases

M 0 - distant metastases are not detected

M 1- there are distant metastases

Clinic.

Soft tissue sarcomas often accompany:

Overgrowth and swelling of soft tissues

· Pain in the trunk or limbs.

· Retroperitoneal tumors. Patients usually note weight loss and complain of pain of undetermined localization.

Bleeding is the most common manifestation of sarcomas gastrointestinal tract and female genital organs.

Diagnostics.

A tumor that is growing rapidly (or tissue growth that exceeds 5 cm) should arouse the specialist's suspicion, especially if it is hard, adherent to surrounding tissues and deeply located. Such neoplasms require morphological verification.

· Biopsy - Excisional biopsy is used (puncture biopsy is most often ineffective). The site for the biopsy should be chosen carefully, with the expectation of possible subsequent reconstructive (plastic) surgery on the extremities.

· Radiological examination includes radiography, bone scintigraphy, MRI, CT.

Treatment.

The basis of treatment is surgical removal of the tumor; if necessary, radiation or chemotherapy can be added. The approaches used ensure complete recovery in 60% of patients. For tumors of questionable resectability, preoperative intra-arterial chemotherapy and radiation may contribute to the effectiveness of reconstructive surgery on the extremities. It is necessary to exclude the entry of malignant cells into surgical wound and adhere to the principles of ablastics during surgery.

An increase in the recovery rate and an increase in survival of patients with sarcomas of the extremities when using combination chemotherapy based on the use of doxorubicin are reported. The effectiveness of combination chemotherapy has been confirmed, especially when combining doxorubicin and thiophosphamide.

Forecast. The main prognostic factors are histological differentiation and tumor size.

· Histological differentiation depends on the mitotic index, nuclear polymorphism and other signs of cellular atypia, as well as on the prevalence of necrosis. The smaller the differentiated tumor, the worse the prognosis.

· Tumor size- independent prognostic factor. Small (less than 5cm ) well-differentiated tumors rarely recur and metastasize.