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Causes, symptoms, stages and treatment of sarcoma

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What is sarcoma?

Sarcoma is one of the varieties of malignant tumors that originate cellular elements of connective tissue. Since, in the human body no body and anatomical segment that does not contain connective tissue, sarcoma does not have a strong localization. Such malignant transformation is exposed to any areas of the human body. In practice, it is fraught with conflicting statistics that only 5% of all malignant neoplasms accounted for sarcomas. But their peculiarity is that the occurrence of such tumors is associated with high mortality. Another feature of sarcomas is preferentially occur in a young age in the period of active growth of the organism (age more than 35% of patients are less than 30 years).


General characteristics of sarcoma

  • A high degree of malignancy;
  • Invasive type of growth with invasion of surrounding tissues;
  • Growing up to large sizes;
  • Frequent and early metastasis to lymph nodes and internal organs (liver, lungs);
  • Frequent recurrence after tumor removal.

Each of the types of sarcomas has its favorite place of growth, age limits, the relationship with specific floors, and other factors. They differ macroscopically and histologically, the degree of malignancy, a different propensity to metastasis and recurrence, depth of germination and the prevalence. The vast majority of sarcomas grow in the form of knots of different sizes and shapes do not have clear boundaries and context resemble fish meat pale-grey with areas of necrosis and varying number of vessels. Some sarcomas are characterized by rapid growth (weeks, months), but there are tumors with a slow growth (years, decades). Tumors of this type are always well krovosnabjaemah.

The most frequent localization of sarcoma

The main derivatives of the connective tissue in the body are the bones, blood vessels, muscles, ligaments, tendons, fascia, connective tissue sheath and the capsule of the internal organs and nerves, connective tissue constrictions of fatty tissue and cellular spaces.

Inbased on this and the localization of tumor growth the most susceptible:

  1. The bones of the extremities;

  2. The soft tissue of the extremities (together with sarcomas of bone comprise 60% of all sarcomas);

  3. Soft tissue and bone of the trunk;

  4. Soft tissue, cellular spaces of space and the bones of the head and neck;

  5. Connective tissue elements of the breast and uterus;

  6. Fiber retroperitoneal space;

  7. Other rare localization (internal organs, abdominal and pleural cavity, mediastinum, brain and peripheral nerves).


Dr. Handelman about the treatment of sarcoma in Israel

I meet in Israel a lot of patients who came from post-Soviet countries for the treatment of sarcomas. These tumors often affect the bone tissue, so patients do not only recover, but also to avoid amputation of the limb.

Israeli encourtered conduct organ-preserving surgery for sarcomas, widely using techniques of arthroplasty. Depending on the location of the tumor, in the treatment of sarcoma in Israel apply various modern techniques of radiation therapy:

  • IMRT
  • IGRT
  • Brachytherapy.

Israeli oncologists implement the principles of personalized medicine, selecting patients for individual treatment protocols. Along with the usual chemotherapy (cytotoxic and cytostatic medicines) and applied immunotherapy.

Is particularly true of the diagnosis of sarcoma in Israel. In our country high-technology methods of medical imaging – CT and MRI. To detect possible metastases performed PET-CT.

If you want to get advice about treatment options a specific type of sarcoma in Israel, I will answer all your questions. To contact me you can on my personal website: https://gandelman.ru/onkologiya/sarkoma


Histological classification and types of sarcomas

Among all malignant tumors sarcoma has the largest variety of histological types. To the category of sarcomas include:

Osteosarcoma

Type of sarcoma

The structure and description of tumor

Is formed from the cellular components of bone tissue

Chondrosarcoma

Presented cartilage tissue

Parietalna sarcoma

Formed from the periosteum and surrounding bone tissue

Reticulosarcoma

Tumor growth of the elements of the bone marrow

Ewing's Sarcoma

Kind of osteosarcomas, affecting mostly the departments end of the long bones of the limbs

Fibrosarcoma

A tumor of connective tissue cells and fibers of the fibrous type

Angiosarcoma

Basis tumors are growths of vascular elements

Stromal sarcoma of the gastrointestinal tract and other internal organs

Originate from the connective tissue component of the stroma of any organ

Liposarcoma

The tumor, growing from the adipose tissue

Rhabdomyosarcoma

The predominance of elements of striated muscle

Kaposi's Sarcoma

Multiple tumor growths of skin blood vessels and lymphoid tissue on the background of immunodeficiency

Lymphangiosarcoma

The tumor growth of the components of the lymphatic vessels

Dermatofibrosarcoma

The swelling of the structures of the skin with connective tissue-based

Synovial sarcoma

The growth of tumors of the synovial membranes of the joints

Lymphosarcoma

The growth of tumors of lymphoid tissue

Neurofibrosarcoma

Grows out of the membranes of nerves

Fibrous histiocytoma

Contains in its composition of different types of connective tissue cells and fibers

Spindle cell sarcoma

Affects the mucous membranes and consists of large spindle-shaped cells

Mesothelioma

The substrate of the tumor may become the mesothelium of the pericardium, peritoneum and pleura

The degree of differentiation of the sarcoma

Not always, even under the microscope can clearly distinguish the structure of sarcoma and its histological type. The most important thing that must be established is the fact of origin of a tumor of the connective tissue and the degree of differentiation.

Depending on this release:

  1. Poorly differentiated sarcoma. Tumors of this type have the lowest degree of malignancy, since its structure is similar to those of the tissue from which they grow. They practically do not metastasize, grow slowly, have a large size, removal rarely causes a relapse;

  2. Vysokomehanizirovannoe sarcoma. Are the absolute opposite of nizkoeffektivnyj. In structure they are similar to those of the tissue from which originate, high malignant, grow rapidly, before Metastasio could not be treated surgical treatment;

  3. Moderately differentiated sarcoma. Occupy an intermediate position between the previous types.

All malignant tumors of the human organism globally divided into epithelial – cancer, glandular adenocarcinoma, and connective tissue – sarcoma. The latter type of tumor is rarer than the others, but is characterized by the large variety of histological types and the ability to defeat any of the organs, tissues and anatomical segments.


The symptoms of sarcoma

The clinical picture of sarcoma depends on the location and characteristics of its malignancy. The main symptoms of the disease are given in the table.

A group of symptoms

Symptoms

Pain syndrome

  • Intense or moderate pain at the site of tumor growth. More typical of highly malignant sarcomas;
  • Discomfort, mild swelling and feeling of a foreign body in the affected area. Characterizes slow-growing sarcoma with a low degree of differentiation;
  • Increased pain during exercise and palpation of the tumor.

The appearance of the tumor
  • Visual definition of the tumor on the skin surface;
  • Palpation definition of tumor masses, located at different depths from the skin surface;
  • Deformation and swelling of the affected limb;
  • The wound surface in place of tumor growth due toher breakup;
  • The disintegrating tumor is always accompanied by profuse and foul-smelling discharge from the surface of the decay.

Impaired function of the affected organ or segment
  • The inability to perform movements or walk for tumors of the soft tissues or bones of the limbs;
  • With the growth of tumors of the internal organs increases their size with the dysfunction and organ failure.

The germination of the surrounding tissues

  • During germination or compression of the blood vessels – circulation with gangrene of the limbs and profuse bleeding;
  • During germination or compression of the nerve – severe pain and weakness of the limbs;
  • During germination of the retroperitoneal space – the violation of the outflow of urine and hydronephrosis;
  • When compression of the mediastinum and neck – disorders of swallowing and breathing;
  • Swollen lymph nodes near the tumor focus.

Diagnosis sarcoma

The presence of any symptoms of sarcoma is a direct indication for its confirmation or exclusion in the shortest possible time.

This may help in such diagnostic methods.

  • X-ray diffraction study for suspected osteosarcoma and other tumors of the bone tissue;
  • Ultrasonic examination of soft tissues or internal organs;
  • Imaging. When bone tumors more feasible execution of the computed tomography. Soft tissue tumors are better seen at MRI;
  • Radioisotope methods of diagnosis. Their diagnostic value increases with deep tumors in the cavities and cellular spaces spaces;
  • Biopsy of the tumor. When superficial tumors is not difficult. Deep seated tumours can be examined only under ultrasound or CT control;
  • Angiography. Introduced in the artery, a contrast agent defines a local cluster of vessels at the place of growth of the tumor and the nature of the violation of blood circulation below the growth of sarcoma.

The causes of sarcoma

All types of sarcomas, like all malignant tumors, are polyetiological diseases whicharise under the influence of many causal factors. To determine their possible in rare cases.

The main responsible of neoplastic transformation of the connective tissue can become:

  • Burdened by family history and genetic predisposition;
  • Damaging effect of ionizing radiation on DNA cells;
  • The impact of oncogenic viruses on the cells, triggering mechanisms uncontrolled division;
  • Violation of lymphatic drainage after surgery and pathological processes;

  • Congenital and acquired immunodeficiencies, HIV infection;

  • Courses of chemotherapy and treatment with immunosuppressive drugs;
  • Transplantation of internal organs;

  • Traumatic injuries, extensive and nonhealing wounds, unexploded foreign body soft tissues.

The implementation of the oncogenic action of the causal factors in the development of sarcomas most often occur in the growing organism. This is because it is much easier to cause damage in those cells that are undergoing active division. The pattern is that the deeper DNA damage, the more malignant sarcoma be!


Stages of sarcoma

The basis of the division of sarcoma at the stage are:

  • The size of the primary tumor;
  • Spread outside the capsule of an organ or the fascia of the anatomical structures from which the sarcoma grows (muscles, bones, tendons, etc.);
  • Involvement in the process and the germination of the surrounding tissue;
  • The presence of metastasis in regional lymph nodes;
  • The presence of metastasis in distant organs.

Histological type of tumor does not affect the staging of sarcomas, in contrast to the primary localization of the tumor in the body. That is what started her on the growth of sarcoma, the greater impact on the definition stage of the process.

Sarcoma stage 1

Such sarcomas are small, do not extend beyond the organ or segment, which began its growth not disrupt its function, not squeezing vital structures, virtually painless, does not metastasize. The identification of even high-grade sarcoma in the first stage allows to achieve good results of treatment.

Signs of the first stage of sarcoma depending on the particular localizationsuch:

  • Sarcoma of the oral cavity and tongue – the tumor is about 1 centimeter comes from the mucosa or submucosal layer in the form of a small node with clear boundaries;
  • Sarcoma of the lip is located within the submucosal layer of the mucous membrane or in the thickness of the lip;
  • Sarcoma cellular spaces and soft tissues of the neck – can be sized up to 2 cm beyond the fascia, which limits the area of its location;
  • Sarcoma laryngeal – node up to 1 cm bounded by the mucous membrane, or other layers of the larynx, within its fascial sheath, does not cause pronounced disorders of phonation and respiration;
  • Sarcoma of the thyroid – a tumor of up to 1 inch with organ, located deep in the tissues. Capsule not grow;
  • Sarcoma of the breast is determined as a node to 2-3 cm, within a lobule, from which its growth began;
  • Sarcoma of the esophagus – the tumor size 1-2 cm, located in the thickness of the body wall. Passage of food through the esophagus is not broken;
  • Sarcoma of the lung – striking one of the segmental bronchi. Does not go beyond the segment and does not violate the functions of the lung;
  • Sarcoma of the testicle has the form of small node, and does not involve in the process of the tunica albuginea;
  • Soft tissue sarcoma of the extremities – the site can reach 5 cm, but not beyond the fascial sheaths.

Sarcoma 2 stage

General characteristics of sarcomas of the second stage: blood location with invasion of all layers, increasing the size of the tumor, impaired organ function, the absence of metastasis.

In the defeat of the bodies, it looks like this:

  • Sarcoma of the oral cavity and tongue, the tumor is clearly visible by visual inspection, located in the thickness of anatomical structures, but grows all its layers, including the mucosa and facies;
  • Sarcoma of lip – a node is in the thickness of the lips, but grows the skin and mucous membranes;
  • Sarcoma cellular spaces and soft tissues of the neck – the tumor is 3 to 5 cm beyond the fascia, limiting its growth space;
  • Sarcoma of the larynx node more than 1 cm spread through all layers of authority, violation of phonation and respiration;
  • Sarcoma of the thyroid gland – the size of a node about 2 cm, the capsule of the organ involved in the pathological process;
  • Sarcoma of the breast – sizesthe tumor about 5 cm, sprouting several segments;
  • Sarcoma of the esophagus – tumor invades the entire thickness of the esophageal wall from the mucous to the serous layer with involvement of the fascia. Significant dysphagia;
  • Sarcoma of the lung – the tumor causes compression of the bronchi or spread to adjacent lung segments;
  • Sarcoma of the testis – germination tumor of the tunica;
  • Soft tissue sarcoma of the extremities – the invasion of tumor fascial formations, which are restricted to an anatomical segment (muscle, cellular spaces space).

The principle of separation of the second stage of sarcoma is that these tumors are within the body, but require an extended resection of the tissues when they are deleted. Results worse, than at the first stage of the process, but relapses occur frequently.

Sarcoma stage 3

The third stage of the sarcoma involves the invasion of tumor fascia and organs located in the immediate vicinity of the tumor or presence of metastasis to regional, to her lymph nodes.

In relation to specific bodies, it looks like this:

  • Sarcoma of the oral cavity and tongue – the primary tumor is of large size, pronounced pain syndrome, disruption of normal anatomic relationships, and chewing. There are metastases in the submandibular and cervical lymph nodes;
  • Sarcoma of the lip – large tumor size, sharply deforming the lip with a possible spread to the surrounding mucosa. Metastases to the submandibular or lymph nodes in the neck;
  • Sarcoma cellular spaces and soft tissues of the neck sharply expressed signs of violations of the functioning of the organs of the neck (swallowing, breathing, disorders of innervation and blood supply). The tumor grows to a large size and grows the blood vessels, nerves and adjacent organs of the neck. There are metastases in the superficial and deep cervical and thoracic lymph nodes;
  • Sarcoma of the larynx sharply disrupts the breath and the voice. Grows vessels, nerves, adjacent fascia. There are metastases in the superficial and deep lymphatic collectors of the neck;
  • Sarcoma of the thyroid gland grows adjacent thyroid tissue. There are metastases in the cervical lymph nodes;
  • Sarcoma breast cancer – large tumor size with sharp deformation of the mammary gland and metastases in the axillary or supraclavicular lymph nodes;
  • Sarcoma of the esophagus the tumor is large, coverstissue of the mediastinum, sharply violates the passage of food. Metastasizes to lymph nodes of the mediastinum;
  • Sarcoma of the lung reaches a large size, causing compression of the bronchi, metastases to peribronchial and mediastinal lymph nodes;
  • Sarcoma of the testicle – is large, distorts the scrotum and grows its layers. There are metastases in the inguinal lymph nodes;
  • Soft tissue sarcoma of the extremities – tumor of about 10 cm, disrupts the function of the limb, dramatically distorts it . There are metastases in regional lymph nodes.

Sarcoma the third stage is characterized by the disappointing results of the treatment requires extended operative intervention and often recurs.

Sarcoma stage 4

The poor prognosis is the detection of sarcoma at stage 4 cancer. The danger of this situation is that these tumors are of gigantic size, sharply compress surrounding tissue or grow into them, forming a solid tumor conglomerate, often accompanied by disintegration and bleeding. Always there are metastases in regional lymph nodes of any localization. Characterized by the presence of distant metastasis in the liver, lungs, brain and bones. There is no need to dwell in detail on the description of stage 4 separate sites of sarcomas, as they are similar with the third stage. Distinguished only by the worsening of the local manifestations and destructive effects of the tumor and presence of distant metastasis.


Sarcoma with metastases

Metastasis is cancer cells that lymphatic or venous vessels spread from the primary tumor lesion in healthy tissue (lymph nodes, internal organs). In places where a large number of elements of the microvasculature is their attachment and active tumor growth. Which body will be the target, to predict hard. Most often, metastases are recorded in regional lymph nodes, liver, lungs, brain, spine and flat bones. Each histological type of sarcoma specific localization there are favorite places of metastasis. Most of them are 4 stage causes liver damage.

The metastatic types of sarcomas are Ewing's sarcoma, liposarcoma, fibrous histiocytoma, lymphosarcoma. These tumors can potentially metastasize at a size less than one centimeter. This phenomenon is due to the highthe concentration of calcium in the tumor site, a very intense blood flow and active growth of tumor cells. They have no capsule, which would limit the area of their growth and reproduction.

Metastases of sarcomas in regional lymph nodes do not cause much difficulty in respect of the disease and its treatment. Differently acting distant metastases of internal organs. They are prone to progression in the form of increase in size and number. Very rarely deal with them with the help of surgery, radiation and chemotherapy. Are subject to removal only a single metastasis in a limited area of the liver, lungs or bones. Multiple metastases are not removed, as it will not bring effect.

Histologically, metastases of sarcomas are distinguished from the primary lesions. They are less vascular, cellular mitoses and other features of atypia, a lot of necrosis areas. Sometimes, the initially detected metastases from an unknown focus. Only an experienced histologist in structure metastasis can determine what type of sarcoma it belongs to.


The treatment of sarcoma

Treatment of sarcomas should be performed in accordance with modern principles of cancer care.

The main thrust of the integrated differentiated approach:

  • The use of surgical methods;
  • Chemotherapy (administration of drugs: ifosfamide, doxorubicin, dacarbazine, methotrexate, cyclophosphamide, vincristine);
  • External beam and radioisotope therapy.

The choice of specific therapies and their combination depend on:

  • Type, stage and location of the sarcoma;
  • The age of the patient;
  • The General condition;
  • Preceding treatment.

The removal of sarcoma

The Central method of treatment of sarcoma is considered to be surgical. Only by removing the tumor, you can expect the cure of the disease. The volume of operation and features pre - and postoperative period should be chosen individually in every particular case, of the tumor. Differential treatment strategy might be:

  1. Low and usernotification sarcoma stage 1-2 any location in individuals of all age groups in a satisfactory condition are subject to radical treatment by removal of the tumor by surgery with regional lymph node dissection. In the postoperative period can be used one or two courses of chemotherapy or external beam radiation therapy. The decision on theirfeasibility shall be made after histological examination of the removed product;

  2. Vysokomehanizirovannoe sarcoma stage 1-2. Required to be surgical treatment with extended lymph node dissection and concomitant chemotherapy in the pre - and postoperative period;

  3. Sarcoma stage 3 should be treated by a combination of all methods. In the preoperative period it is advisable to conduct courses of radiation and chemotherapy. With their help, the tumor decreases in size, which facilitates its removal. The operation involves removing the sarcoma with all of the germinating tissue, restoring damaged important structures (vessels, nerves) and excision of regional lymph collectors. In the postoperative period is mandatory chemotherapy;

  4. Most osteosarcomas require combined treatment. Peculiarities of surgical interventions in that the operation requires amputation of the affected limb with further prosthetics. Removed by resection of a bone site can only immature surface osteosarcoma in the elderly;

  5. Sarcoma stage 4. Most of them require a symptomatic treatment (pain relief, detoxification therapy, correction of anemia , etc.). Comprehensive treatment of such sarcomas may be indicated only for tumors of the affordable surgically removed (no germination vital structures, small size, superficial location) in combination with an isolated metastasis in the liver, lungs or bones.


ykov, Yevgeny Pavlovich, doctor-oncologist