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)
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 of human malignant neoplasms. 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.
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.
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 other soft tissues (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
| T |
Primary tumor Tx - primary tumor cannot be assessed T1a - superficial tumor* T1b - deep tumor* T11a - superficial tumor* T11b - deep tumor* |
| N |
Regional lymph nodes: Nx - regional lymph nodes cannot be assessed N0 - no metastases in regional lymph nodes N1 - there are metastases in regional lymph nodes |
| M |
Distant metastases: M0 - no distant metastases. M1 - there are distant metastases. |
| G |
Histological grade of malignancy: G1 - low G2 - medium G3 - high |
| Note: Extraskeletal Ewing sarcoma and primitive neuroectodermal tumor are classified as high-grade tumors. If the grade of malignancy cannot be assessed, a low grade of malignancy is established. |
| Stage IA | T1a | N0 | M0 | |
| Т1b | N0 | M0 | Low grade | |
| Stage 1B | T2a | N0 | M0 | Low grade |
| Т2b | N0 | M0 | Low grade | |
| Stage IIA | T1a | N0 | M0 | |
| Т1b | N0 | M0 | High grade of malignancy | |
| Stage IIB | T2a | N0 | M0 | High grade of malignancy |
| Stage III | T2b | N0 | M0 | High grade of malignancy |
| Any T | N1 | M0 | ||
| Stage IV | Any T | Any N | M1 | Any degree of malignancy |
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.
Resume
Diagnostic criteria(description reliable signs diseases depending on the severity of the process)
Complaints: emergence and gradual growth tumor formation soft tissues. Emergence and growth 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;
Computed tomography;
Magnetic resonance imaging of the affected area;
Bone marrow biopsy from 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 the diagnosis):
Scraping smears from the surface of an ulcerated tumor;
Smear impressions of material taken using a knife or trepanobiopsy;
- ultrasound examination of the abdominal organs;
Arteriography (performed when the tumor is localized in the area of large major vessels);
PET - according to indications;
Skeletal scintigraphy according to indications.
| № | Benign bone tumors / aggressive course | Malignant bone tumors |
| 1. | Fibroma | Fibrosarcoma |
| 2. | Lipoma | Liposarcoma |
| 3. | Neurofibromatosis | Rhabdomyosarcoma |
| 4. | Hemangioma | Malignant mesenchymoma |
| 5. | Malignant histiocytoma |
Treatment abroad
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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);
Trepanobiopsy 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 for 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 within the framework of combined and complex treatment. Control of the radicality of surgical intervention is carried out by urgent intraoperative histological examination of the edges of tumor cutting from normal tissues.
Amputations and disarticulations. Amputation and disarticulation of a 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 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 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) + 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 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 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 lack of 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 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
Port system installation
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;
Liposarcoma is a type of malignant tumor that is classified as mesenchymal. The shape is similar to an irregularly shaped knot, loose. At timely diagnosis cancer condition and treatment can increase the patient's life expectancy.
Liposarcoma is inferior in frequency to fibrolipoma. The neoplasm is modified from mesenchymal fat cells, containing huge amount vessels have an incompletely formed capsule. Some subtypes of formations resemble a wen. The most common localization is the intermuscular space, less common in adipose tissue. The structure of the compaction resembles fish meat. Colour: yellow or grey. The disease occurs in adults, with a maximum incidence of 50 years. The ICD 10 code for mesenchymal tumors is C45-C49.
Cancers do not have specific causes.
Risk factors:
Sarcoma and neurofibromatosis are genetically similar and have a similar mechanism of occurrence. If there are patients in the family cancer diseases the risk of developing sarcoma increases. Soft tissue liposarcoma – pathological formation, capable of forming alone or having a multiple character.
The formation can be found in the area of anatomical structures: hips, shoulders, groin, back, buttocks, legs, retroperitoneum, abdominal cavity(including the diaphragm), knee. The tumor practically does not occur on the scalp, neck, mammary glands and hands. The formation can be found deep in the fat layer and intermuscular space. The degree of differentiation of liposarcoma plays an important role in determining the type.
Consists of mature and affects mostly older people. Well-differentiated liposarcoma rarely metastasizes, but is capable of recurrence. Soft tissues are a favorite place for new growth.
Composition: young and mature cells surrounded by a jelly-like capsule and numerous vessels. The limbs are affected. Older people may experience myxoid liposarcoma.
A rare species. Pleomorphic liposarcoma observed in areas not characteristic of the disease: head, neck, torso. It consists of huge cells with an unusual shape; there are small cells that are inclusions. It differs in location. The tumor does not need to be looked for deep in the tissues; the location is subcutaneous. Metastasis is observed.
The name itself speaks about the shape of the cells that make up the sarcoma. Liposarcoma has a poorly differentiated growth and consists of a small number of blood vessels. Looking at the round cell formation, you can find similarities with fat cells.
High and low degrees of differentiation are intertwined. It metastasizes quickly. The prognosis is unfavorable. Early detection of a tumor is important for the possibility of its cure. Refusal of medical therapy and use folk remedies for cancer treatment is often fatal.
In the first stage of liposarcoma, no symptoms of the condition are felt. Man lives full life. As you grow, discomfort increases. The seal can be felt. There are formations up to 20 cm and more, often solitary. It is a dense or soft formation that does not have a clear contour. The tumor compresses the blood vessels, nerve plexuses. Severe pain occurs at the location of the tumors.
Reaching large sizes, bone deformation and disruption of blood vessels are possible. Thrombosis, phlebitis and edema are a common result of the condition. Having penetrated into large nerve plexuses, paralysis of the limbs and loss of sensitivity are observed. Liposarcoma located in the retroperitoneal space does not cause discomfort. Rare manifestations: heaviness, distension in the abdomen. As it progresses, symptoms increase. The tumor compresses the organs, and complaints arise depending on the organ that was affected.
Liposarcoma can metastasize through the lymphogenous or hematogenous route.
A common route of spread is blood. Target cells: brain, lungs, liver and bones.
Severe symptoms that appeared in the last stage:
The combination of two cancer conditions at the same time is rare. It mainly affects the lower part of the body, including the groin, and can metastasize. Prostate cancer and liposarcoma have similar cells in their structure - lipoblasts. The combination of tumors aggravates the course of the underlying disease and increases the risk fatal outcome. Symptoms of carcinoma can blur the clinical picture of sarcoma; correct diagnosis is important.
The initial stage of examination of any disease begins with collecting anamnesis. Clinical analysis blood is of significant importance in making a diagnosis. Magnetic resonance imaging of liposarcoma is considered relatively new, the most informative and of high quality. Based on the data, you can find out about the state of the body and give a conclusion. To clarify, a biopsy is used, sending a tumor sample for histology. If the presence of metastases is suspected, additional instrumental diagnostic methods are used: ultrasound, CT, MRI, angiography, x-ray.
CT is a relatively new, high-quality type of instrumental diagnostics. It is possible to differentiate liposarcoma from other soft tissue tumors.
By using ultrasound diagnostics determine formations located in tissues. The seals are highly echogenic. Lipomas, like sarcomas, have a similar property; it is impossible to distinguish them on the screen. By using this study, changes in surrounding organs affected by liposarcoma can be observed.
Vascular examination makes it possible to study and treat the disease. In the area of compaction, the vessels are dilated, large veins are usually displaced by the compaction.
Put final diagnosis biopsy and morphological studies help. Conduct differential diagnosis with other diseases.
Removal is carried out surgically, using a combination of chemotherapy and radiation.
There are statistical data indicating the prognosis of the course of the disease. However, the human body is an individual mechanism. Depending on the type of liposarcoma, the five-year survival rate is:
Ten year survival rate:
The location of liposarcoma plays an important role.
The retroperitoneal space is the most dangerous and sensitive. Survival rates are reduced when the kidneys and spinal cord are affected.
Oncology is not a death sentence. Science does not stand still; by learning about the problem in a timely manner and starting treatment, you can say goodbye to a terrible diagnosis.
Code according to the international classification of 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 muscle- leiomyosarcoma. Synovial tissue - synovial sarcoma. Nerve sheaths: . Neuroectodermal - malignant neuroma (schwannoma). Connective tissue - perineural fibrosarcoma. Unclassified blastomas.
TNM classification ( sarcoma Kaposi, dermatofibrosarcoma, grade I desmoid tumors, sarcomas 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 muscular fascia. Deep - “b” - the tumor reaches or grows into the (most) superficial muscle 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 regional lymph nodes. Distant metastases. M1 - there are distant metastases.
Grouping by stages: . Stage IA - G1 - 2T1a - 1bN0M0 - highly differentiated, small size tumors, regardless of location. Stage IB - G1 - 2T2aN0M0 - well-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, 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 microscopically detected at the edges of the resected tissue malignant cells the muscular-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 anterior mediastinum and retroperitoneal ones 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 often detected in late stages and radical removal is often not possible, surgery 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 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 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.
For complications tumor growth it is possible to perform palliative resections, which can reduce intoxication, blood loss from disintegrating tumors, 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 torso, head, neck. Most important sign- 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. They 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. 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, with 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 — malignant tumor, originating from skeletal (striated) muscle. There are embryonic (develops up to 15 years) and adult types of rhabdomyosarcoma.
Clinical picture. Most often, tumors are localized in three anatomical areas of the body: limbs, head and neck, and pelvis. The tumor grows quickly, without pain or dysfunction of organs. Often they germinate into the skin with the formation of exophytic bleeding formations. Characteristic of early relapse
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 exhibits infiltrating rapid growth and 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 is typical in the area of the hand and foot. 25-30% of patients indicate a history of trauma. Tumors in 20% of cases give regional metastases, in 50-60% - hematogenous metastases in the lungs. 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. Most often located on the lower extremities. Primary tumor multiplicity is characteristic. Recurrence is possible. Treatment is surgical. Prognosis. Main prognostic factors - degree of histological differentiation and tumor size, patients with low-grade neuroma have a less favorable prognosis. Tumor size is an independent prognostic factor. Small (less than 5 cm) completely removed well-differentiated tumors rarely recur and metastasize.
Kaposi's sarcoma(cm. Sarcoma Kaposi).
ICD-10. C45 Mesothelioma. C46 Sarcoma Kaposi. C47 Malignant neoplasm peripheral nerves and the autonomic nervous system. C48 Malignant neoplasm of the retroperitoneum and peritoneum. C49 Malignant neoplasm of other types of connective and soft tissues.
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Sarcoma is a neoplasm that often has a malignant course. There are many different sarcomas, which are classified into a single disease in ICD 10. The danger of the pathology lies in the absence of symptoms initial stages development of the tumor, which complicates diagnosis.
Sarcoma can be localized in various tissue areas. It can affect muscles, epithelium, nerve fibers, and connective tissue. In medicine, there are three main types of sarcomas: conditionally benign, malignant and intermediate, accompanied by metastatic lesions.
The disease is diagnosed 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 appear in the liver, brain, and lungs. As a result, cancerous damage to the injured organs develops.
In medicine, many different sarcomas are distinguished depending on their composition:
The type of sarcoma is determined based on the results of instrumental diagnostic methods by a specialist. This is necessary because many of the formations are capable of transforming into malignant tumors and become the cause metastatic lesions.
Experts were unable to establish the true reasons for the formation of sarcoma. But they have identified a number of factors that can influence and increase the risk of developing the disease.
Of great importance in the formation of tumors of various types is genetic predisposition. It was found that in patients diagnosed with 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 say that carcinogenic, chemical, toxic and poisonous substances also have an effect on the body.
Unfavorable environmental conditions in the patient’s areas of residence can also significantly increase the risk of sarcoma formation.
Scientists believe that the causes of tumor formation are regular damage to the skin, long-term use steroid drugs and the presence of precancerous diseases.
Despite the fact that in medicine there are many types of sarcomas, 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 tumor grows, the patient experiences a sharp weight loss, constant fatigue, depression and fatigue.
Advanced stage of cancer is characterized 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 distinguished by a yellowish or white 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 and ulcers appear. The size of the tumor can reach 30 centimeters.
The danger of sarcoma is that it does not appear for a long time and the patient is not aware of its presence. If sarcoma affects muscle tissue, it becomes noticeable in the later stages of development, when there is no chance of successful recovery.
Establishing the disease in the presence of sarcoma is difficult in some cases, due to the absence of symptoms in the initial stages. A preliminary diagnosis is established based on the patient's 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:
The technique is quite informative and allows you to determine the presence of a tumor that is localized in the soft tissues of the thigh, limbs and other parts of the body.
Ultrasound helps determine the size, location, and structure of the tumor. Sarcoma has certain characteristics 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.
If sarcoma is suspected, CT is often performed using a contrast agent. This allows you to determine the area of circulatory disturbance. When performing a computed tomography scan, sarcoma is determined by its irregular shape, unclear contours, and heterogeneous structure. Adjacent tissues are also damaged and compressed. The contrast agent accumulates in the tortuous vessels.
Prescribed when it is impossible to conduct a computed tomography scan. The technique is quite informative. The main advantage of MRI is the ability to visualize a tumor layer-by-layer, determine its structure, size, and location.
X-ray examination is carried out to determine metastatic lesions not only in neighboring organs or tissues, but also distant ones.
The disadvantage of the technique is that it is impossible to determine the type of tumor using an x-ray machine.
A fine-needle biopsy for sarcoma is prescribed to confirm the type of formation and presence cancer cells. The collection 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 learn the diagnostic results 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 undergo a general and biochemical blood test. A specialist may order a plasma test for tumor markers. As additional methods Studies show angiography and positron emission tomography.
Based on the research results, a final diagnosis is established and a course of therapy is prescribed.
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 treatment system for embryonal rhabdomyosarcoma, fibrosarcoma and other types of sarcomas, it is often prescribed surgery. The goals of the operation are to remove the tumor, alleviate the patient's condition and increase his life expectancy.
Surgery in some cases may be supplemented with radiation or chemotherapy. Techniques can slow down tumor growth, but have a number of side effects. Among undesirable consequences hair loss, brittle nails, weakness, nausea and weight loss are noted. That is why they are not prescribed for the treatment of patients over 60 years of age.
In cases where sarcoma has enough large sizes, strikes 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 carried out if the patient has contraindications to surgical intervention or the tumor is small in size.
After treatment, the patient is registered at the dispensary. The patient should regularly established deadlines visit your doctor for preventative purposes.
Sarcoma of soft tissues of peripheral nerves, connective or muscle tissue if left untreated, it becomes the cause of development serious complications. Among them are:
With the development of various types of sarcoma, there is a decrease in hearing, vision, memory loss and concentration. The sensitivity of the skin is also impaired. That is why treatment should not be delayed when diagnosing sarcoma.
The prognosis for the development of sarcoma is influenced by many different factors. First of all, this is the stage of the disease. Also great value have the localization of the tumor process, age, condition of the patient, the presence of metastatic lesions, concomitant diseases.
The prognosis is more favorable when treatment was started at stages 1 or 2 of the pathology. The survival rate of patients is about 70-50%. But sarcoma is dangerous because after removal the tumor forms again.
Most often, the prognosis for sarcoma is unfavorable, which is due to the fact that in the initial stages of development it is quite difficult to determine the presence of pathology. The survival rate of patients at stages 3 or 4 is no more than 15%.
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:
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 recovery from the disease.
Soft tissue sarcoma is a common disease among other cancers. 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 diagnosis.
That is why patients are recommended to undergo annual preventive examinations. When sarcoma is diagnosed, treatment should not be delayed, since its absence causes the development serious consequences, including death.
The main clinical sign of osteosarcoma is pain over the affected area. The pain is dull, constant with a gradual increase in intensity. A characteristic symptom is night pain. In 3/4 of patients a soft tissue component may be present. The limb is enlarged and often looks swollen. Pain and increased volume lead to dysfunction. The duration of the anamnesis is on average 3 months.
Damage to the metaphyses of long tubular bones is typical. The most common location (approximately 50% of cases) is the knee joint - the distal part of the femur and the proximal part of the tibia. The proximal part is also often affected humerus And femur, And middle third femur. Damage to flat bones, especially the pelvis, occurs in less than 10% of cases in childhood.
Osteosarcoma has a huge tendency to develop hematogenous metastases. By the time of diagnosis, 10%-20% of patients already have macrometastases in the lungs, detected by X-ray. But already about 80% of patients by the time of diagnosis have micrometastases in the lungs, which are not detected x-ray, but are visible on computed tomography. Since bones do not have a developed lymphatic system, early spread of osteosarcoma to regional lymph nodes is rare, but if it occurs, it is a poor prognostic sign. Other areas of metastasis are bones, pleura, pericardium, kidneys, and central nervous system.
Osteosarcoma also has local aggressive growth, can spread to the epiphysis and the adjacent joint (the knee and shoulder joints are most often affected), spreading along intra-articular structures, through articular cartilage, through the pericapsular space, or, directly, due to a pathological fracture, and forming non-adjacent to it foci - satellites - "skip" metastases.
Rare variants of osteosarcoma.
Telangiectatic - radiologically resembles an aneurysmal bone cyst and giant cell tumor, manifested by the presence of lytic foci with mild sclerosis. The course of the disease and response to chemotherapy are practically no different from standard variants of osteosarcoma.
Juxtacortical (paraosseous) - comes from the cortical layer of the bone, tumor tissue can surround the bone on all sides, but, as a rule, does not penetrate the medullary canal. There is no soft tissue component, so radiologically the tumor is difficult to distinguish from osteoid. As a rule, this tumor is of low malignancy, progresses slowly, and almost does not metastasize. However, paraosseous osteosarcoma requires adequate surgical treatment, almost the same as standard tumor variants. Otherwise, this tumor recurs and the tumor component changes to a higher degree of malignancy, which determines the prognosis of the disease.
Periossal - just like paraosseous, it is located on the surface of the bone and has a similar course. The tumor has a soft tissue component, but does not penetrate the medullary canal.
Intraosseous tumors with a low degree of malignancy, well differentiated, with minimal cellular atypia, can be regarded as a benign tumor. But they also tend to recur locally with a change in the tumor component to a more malignant variant.
Multifocal - manifests itself in the form of multiple foci in the bones, similar to each other. It is not entirely clear whether they appear immediately, or whether rapid metastasis occurs from one focus. The prognosis of the disease is fatal.
Extraskeletal osteosarcoma is a rare malignant tumor characterized by the production of osteoid or bone tissue, sometimes together with cartilage tissue - in soft tissues, most often of the lower extremities. But there are also lesions in other areas, such as the larynx, kidneys, esophagus, intestines, liver, heart, bladder and The prognosis of the disease remains poor, sensitivity to chemotherapy is very low. The diagnosis of extraskeletal osteosarcoma can be established only after excluding the presence of bone tumor foci.
Small cell osteosarcoma is a highly malignant tumor; its morphological structure differs from other variants, which determines its name. Most often this tumor is localized in the femur. Small cell osteosarcoma ( differential diagnosis with other small cell tumors) necessarily produces osteoid.
Osteosarcoma of the pelvis - despite improved overall survival for osteosarcoma, has a very poor prognosis. The tumor is characterized by rapid and widespread spread in and along tissues, since, due to anatomical structure pelvis, does not encounter significant fascial and anatomical barriers on its way.
Staging of osteogenic sarcoma (Enneking WF, Spanier SS, Goodman MA, 1980, USA).
Stage IA - Well differentiated tumor. The lesion is limited by a natural barrier that prevents the spread of the tumor. No metastases.
Stage IB - Well-differentiated tumor. The outbreak spreads beyond the natural barrier. No metastases.
Stage IIA - Poorly differentiated tumor. The outbreak is limited by a natural barrier. No metastases.
Stage IIB - Poorly differentiated tumor. The outbreak spreads beyond the natural barrier. No metastases.
Stage III - The presence of regional and distant metastases, regardless of the degree of tumor differentiation.
