Diseases, endocrinologists. MRI
Site search

Sarcoma of the anterior abdominal wall. Abdominal sarcoma. Laboratory research methods

The detection of abdominal sarcoma indicates the development of a serious pathology of a malignant nature, requiring immediate treatment. The disease is not very common, occurs more in women over the age of 50 years.

Causes

Abdominal sarcoma is a soft tissue tumor. The reason for its development may be a long-term effect of adverse factors on the human body. The exact mechanism of malignant transformation is unknown.

The following phenomena are capable of provoking the development of pathology:

  1. Living in an area with poor environmental conditions.
  2. hereditary predisposition.
  3. Frequent contact of the body with harmful substances: poisons, carcinogens, chemicals.
  4. Improper nutrition, characterized by the consumption of food containing a lot of carcinogens and other negative components.
  5. Bad habits: smoking and alcohol abuse.
  6. Poor functioning of the immune system.
  7. Frequent infectious and viral pathologies.
  8. Work in a hazardous industry.
  9. Surgical interventions in the abdominal cavity.

Doctors noticed that the disease is most often affected by people who are in old age, live in megacities and have undergone complex operations on the organs of the peritoneum.

Symptoms

Sarcoma of the retroperitoneal space in the initial stages does not manifest itself as severe symptoms. Therefore, it is quite difficult to identify it at the beginning of development. This helps only preventive examination.

As the tumor grows, patients begin to worry about clinical manifestations. Patients complain of the following signs of pathology:

  • Abdominal pain.
  • Chair disorders.
  • Problems with urination.
  • Weight loss.
  • Loss of appetite.
  • Weakness all over the body.
  • Poor performance.
  • Increase in body temperature.
  • Enlargement of the peritoneum.
  • Discharge of blood during bowel movements.

From the third stage, the process of metastasis to the lymph nodes begins, at the fourth stage of development, secondary foci already affect the internal organs. As a result, patients are concerned about additional symptoms that are characteristic of a dysfunction of the affected area.

Varieties of the tumor

Retroperitoneal sarcoma is of several types:

  1. Angiosarcoma. Formed from lymphatic and blood vessels.
  2. Leimiosarcoma. Formed from muscle tissue.
  3. Fibrosarcoma. Affects connective tissues.
  4. Liposarcoma. Develops their adipose tissue cells.

There is also an embryonic sarcoma, which is formed in the fetus during fetal development.

Also, all malignant tumors are divided into highly differentiated, moderately differentiated and undifferentiated. The latter variety is considered the most dangerous, as it differs from other species in its rapid growth and early metastasis.

Survey

It is possible to identify peritoneal sarcoma using various diagnostic methods. First of all, the doctor himself examines the patient, feels the affected area, studies the clinical picture. After that, the patient donates blood and urine for analysis and is sent for instrumental examination.

The following diagnostic methods are used:

  • Ultrasonography.
  • Radiography.
  • Computed and magnetic resonance imaging.
  • Biopsy followed by histology of affected cells.

Based on the results of the examination, a suitable method for the treatment of retroperitoneal sarcoma is selected.

Therapeutic measures

When a sarcoma of the retroperitoneal wall or abdominal organs is detected, treatment is prescribed depending on the size of the tumor, its type, location. If the neoplasm is small and there are no metastases in other organs, surgery is performed to remove the lesion.

If there are metastases, only chemotherapy can prolong the patient's life. It is carried out with the help of cytotoxic drugs that kill malignant cells. Means affect the entire body at once, so this method allows you to destroy metastases in different organs.

You can also treat pathology with radiation therapy. Unlike chemical treatment, it acts directly on the pathological focus. Usually, all these methods are combined depending on the stage of tumor development.

The prognosis of life with abdominal cavity sarcoma is favorable in the early stages, that is, with 1-2 degrees. Subsequent stages are accompanied by metastases, so it is very difficult to get rid of malignant cells. You can only extend the life for a small number of years. That is why it is so important to identify sarcoma in a timely manner and begin its treatment.

Abdominal sarcoma is rare. It accounts for 1-3% of cases among tumors of the abdominal cavity.

Sarcoma of the abdominal cavity is in the form of a dense node, soldered to the organs, the anterior abdominal wall. In appearance, there are nodal (20%) and diffuse (80%), the so-called muff-like forms.

The narrowing of the intestinal lumen with sarcoma is observed less often than cancer, and more often there is an expansion of the intestine, the so-called "aneurysmal sarcomas of the intestines." The mucosa over the tumor is often ulcerated or there are polyposis growths on it.

Sarcoma can develop from the submucosal layer of the intestine and spread along the intestine, the peritoneum germinates late, but can spread to the entire intestinal wall.

The tumor consists of young immature connective tissue cells. In the colon, all types of sarcoma formations are observed, but round cell ones predominate. On section, the tumor resembles "fish meat" and often has foci of necrosis of various sizes. Sarcomas of the abdominal cavity are characterized by rapid development. The duration of the disease is up to a year. In young people, the disease is even more malignant.

Symptoms of abdominal sarcoma

At first, the sarcoma is asymptomatic, therefore it is clinically manifested only when the pathological process has gone far. Patients lose their appetite, sometimes diarrhea appears, and then constipation, often the disease proceeds, simulating chronic. Pain is either absent or minor. In the presence of lesions of the lymph nodes of the retroperitoneal space, pain can be given to the lower back, sacrum and lower limbs. Body temperature often remains normal, but with a decaying tumor, it can reach up to 39 °.

In the final stage of the process appear: metastases, cachexia, disorders of the heart and kidneys. and cachexia in sarcomatous lesions are less common than cancer. The most common complications: germination in the abdominal organs (small intestine, bladder, uterus). perforation into the free cavity, in the later stages, intestinal obstruction. It often happens, especially with multiple sarcoma. Intestinal bleeding is rare.

The diagnosis of sarcoma is difficult. However, the presence of a rapidly growing, large-tuberous, low-painful, often mobile tumor, in the absence of bowel stenosis, should suggest the possibility of sarcomatous formation, especially in young people.

X-ray in the tumor-affected section of the abdominal cavity, in addition to violations of the mucosal relief, there is also a lack of intestinal peristalsis. Differential diagnosis can be difficult. Even with the study, it is not always possible to recognize a sarcomatous neoplasm and distinguish it from inflammatory "tumors", tuberculosis, syphilis and actinomycosis, especially if there is a simultaneous intestinal lesion with sarcoma and tuberculosis. There are cases when the sarcoma of the cecum was mistaken for and an appendectomy was performed. Macroscopically, it is very difficult to distinguish fibrosarcoma from soft fibroma. Therefore, each received

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

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

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

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

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

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

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

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

ICD-10 code

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

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

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

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

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

ICD-10 code

C45-C49 Malignant neoplasms of mesothelial and soft tissues

Causes of sarcoma

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

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

, , , , , , ,

Sarcoma symptoms

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

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

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

Sarcoma in children

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

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

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

  • Malignant diseases of the lymph nodes

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

  • Lymphogranulomatosis

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

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

  • Lymphosarcoma

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

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

  • Tumors of the kidneys

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

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

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

Types of sarcoma

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

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

Sarcomas arising from hard bone tissue:

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

Sarcomas arising from muscle, adipose and soft tissue:

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

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

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

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

  • Alveolar sarcoma - most often occurs in children and adolescents. It rarely metastasizes and is a rare type of tumor.
  • Angiosarcoma - affects the vessels of the skin and develops from blood vessels. Occurs in the internal organs, often after exposure.
  • Dermatofibrosarcoma is a type of histiocytoma. It is a malignant tumor that arises from the connective tissue. Most often affects the trunk, grows very slowly.
  • Extracellular chondrosarcoma is a rare tumor that arises from cartilage, localizes in cartilage and grows into bone.
  • Hemangiopericytoma is a malignant tumor of the blood vessels. It has the appearance of nodes and most often affects patients under 20 years of age.
  • Mesenchymoma is a malignant tumor that grows from vascular and adipose tissue. Affects the abdominal cavity.
  • Fibrous histiocytoma is a malignant tumor localized on the extremities and closer to the trunk.
  • Schwannoma is a malignant tumor that affects the sheaths of the nerves. It develops independently, rarely gives metastases, affects deep tissues.
  • Neurofibrosarcoma - develops from Schwann tumors around the processes of neurons.
  • Leiomyosarcoma - appears from the rudiments of smooth muscle tissue. It spreads rapidly throughout the body and is an aggressive tumor.
  • Liposarcoma - arises from adipose tissue, localized on the trunk and lower extremities.
  • Lymphangiosarcoma - affects the lymphatic vessels, most often occurs in women who have undergone a mastectomy.
  • Rhabdomyosarcoma - arises from striated muscles, develops in both adults and children.
  • Kaposi's sarcoma is usually caused by the herpes virus. Often occurs in patients taking immunosuppressants and HIV-infected. The tumor develops from the dura mater, hollow and parenchymal internal organs.
  • Fibrosarcoma - occurs on the ligaments and muscle tendons. Very often it affects the feet, less often the head. The tumor is accompanied by ulcers and actively metastasizes.
  • Epithelioid sarcoma - affects the peripheral parts of the limbs, in young patients. The disease actively metastasizes.
  • Synovial sarcoma - occurs in the articular cartilage and near the joints. It can develop from the synovial membranes of the muscles of the vagina, and spread to the bone tissue. Due to this type of sarcoma, the patient has reduced motor activity. Most often occurs in patients aged 15-50 years.

Stromal sarcoma

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

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

Spindle cell sarcoma

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

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

Malignant sarcoma

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

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

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

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

Pleomorphic sarcoma

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

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

Polymorphic cell sarcoma

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

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

Undifferentiated sarcoma

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

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

Histiocytic sarcoma

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

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

round cell sarcoma

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

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

Fibromyxoid sarcoma

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

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

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

, , , , , ,

Lymphoid sarcoma

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

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

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

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

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

Epithelioid sarcoma

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

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

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

, , , , , , , , ,

myeloid sarcoma

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

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

Clear cell sarcoma

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

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

, , , , , , , , , , ,

Neurogenic sarcoma

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

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

Bone sarcoma

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

Muscle sarcoma

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

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

Skin sarcoma

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

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

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

Sarcoma of the lymph nodes

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

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

Vascular sarcoma

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

  • Angiosarcoma

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

  • endothelioma

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

  • Peritelioma

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

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

Metastases in sarcoma

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

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

Diagnosis of sarcoma

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

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

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

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

Sarcoma prognosis

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

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

Survival in sarcoma

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

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

It is important to know!

Kaposi's pseudosarcoma is a chronic vascular disease of the skin of the lower extremities, clinically very similar to Kaposi's sarcoma. developing due to venous insufficiency (Mali type) or insufficiency of arteriovenous anastomoses (Blufarb-Stewart type).

Sarcoma of the abdominal cavity is a neoplasm that occurs in the soft tissues of the retroperitoneal space and grows rapidly with the course of an oncological disease, posing a great threat to human life. Retroperitoneal sarcoma accounts for approximately 13% of the total number of tumors that can occur in soft tissues. Although this disease is not very common among oncological diseases, nevertheless, it entails serious consequences.

Retroperitoneal sarcoma is a type of tumor that is formed from cells of the muscle, fat or connective tissue of the human body. In the international classification of neoplasms, it falls into the ICD-10 section.

Groups of people who are at maximum risk of the disease:
  • people about 50 years old;
  • women in the age group from 20 to 50 years;
  • in some cases, children under 10 years of age.

One of the most unpleasant properties of this disease is that in the course of development at the initial stage, the tumor grows, practically without manifesting itself. As a rule, a retroperitoneal tumor is detected already when it is significantly enlarged, due to which the organs and blood vessels surrounding it are compressed, and it is the pain caused by this condition that causes the patient's anxiety. There have also been cases when the disease is detected by chance during a general examination. The tumor provokes dangerous metastases that are not easy to notice at the initial stages. The occurrence of metastases in the nearest lymph nodes indicates an extremely unfavorable course of this disease.

Very often, the cause of a patient's death is metastasis of a malignant neoplasm to the liver, lungs or even bones - this can lead to complications up to osteosarcoma. The approximate life span of a patient with onset of metastases rarely exceeds more than a year.

In children, tumors of this type are observed very rarely, with the exception of only embryonic types of liver sarcomas. Mostly this type of tumor is formed in young children. Diagnosis of an embryonic liver tumor usually does not cause difficulties: not only can an increase in the tummy be noticeable in a photo or with the naked eye, the disease can also be detected by probing through the anterior abdominal wall, because in a child, neoplasms are relatively large, so they simply cannot go unnoticed.

Usually, the pain syndrome does not manifest itself clearly, however, older children may already complain of a sharp pain in the hypochondrium, on the right side. This may be due to the pressure of the growing tumor on nearby organs and tissues.

Most often, children with this diagnosis complain of:

  • sudden loss of appetite;
  • lethargy, weakness;
  • nausea up to vomiting;
  • causeless increase in body temperature;
  • exhaustion;
  • sometimes there are signs of anemia.

Unfortunately, the clinical prognosis is pessimistic. These tumors are very resistant to chemotherapy and radiation treatments, and some types of surgery may not be possible in children.

Retroperitoneal sarcoma begins to form from muscle, fat or connective tissues of the body. Thus, the disease has some varieties depending on the tissues on which it began to develop. Although the clinical manifestations of these diseases are similar and difficult to distinguish from each other, several types of this disease can be distinguished.

It is worth considering in more detail these types of retroperitoneal sarcoma:

Among other things, such a disease can develop from different sides. The tumor growing on the right in the abdominal cavity is characterized by early venous congestion. Also, the localization of a malignant tumor to the left or right of the epigastric region can provoke heaviness and dull pain, regardless of the meal or the nature of the meal itself. A neoplasm in the lower left side of the retroperitoneum causes symptoms such as intestinal obstruction, as well as multiple pains in the lower back or in the groin, sometimes in the limbs.

This variety is quite difficult for early diagnosis of a tumor. To determine the presence of sarcoma, you can use palpation through the rectum or vagina. Often, retroperitoneal sarcoma, localized in the pelvic region, also causes inguinal and perineal hernias.

It is difficult to detect the first symptoms of retroperitoneal sarcoma, unlike, for example, synovial sarcoma, which is localized mainly on the extremities, where it is easier to notice. As a rule, the clinical picture of various types of retroperitoneal sarcomas are similar, differing only in location and histological features.

A malignant tumor basically does not manifest itself in the initial stages of its growth, but with an increase, compression of nearby organs and vessels is observed, and subsequently the formation of some symptoms:
  • pain in the area of ​​​​the intestines or stomach, as well as the spine;
  • violation of a healthy defecation and urination regimen: constipation, diarrhea;
  • discomfort in the stomach;
  • increase in body temperature;
  • sudden weight loss or weight gain;
  • increased fatigue;
  • visual observation of the tumor site is possible.

All these symptoms may indicate a long-term or advanced process of development of sarcoma. However, even with the already large size of the tumor, the patient can feel normal for a long time, unaware of the developing dangerous disease.

Only upon reaching a certain degree of the disease, sarcoma with mesenchymal origin can manifest itself with the following symptoms:
  • the patient is unusually quickly satiated;
  • aching incessant pain radiating to the lower back;
  • manifestation of external signs of a tumor: a greatly enlarged abdomen, swollen areas that were not previously observed;
  • bloating, vomiting, problematic bowel movements, and many other abnormalities from normal functioning;
  • violations of venous and lymphatic outflow;
  • shortness of breath, severe fatigue;
  • swelling of the extremities, ascites, enlargement of the veins of the esophagus, venous congestion in the lower extremities, in men there may be an increase in the veins of the spermatic cord.

Metastasis of sarcoma can lead to the manifestation of other signs of tumor development.

A growing neoplasm squeezes blood vessels, nerve endings and nearby organs. This can lead to the manifestation of new symptoms, which will be the result of the development of pathological processes. Although rare, retroperitoneal sarcomas occur in hard-to-reach places, which makes them difficult to detect for modern medicine.

Diagnosis of the disease

Diagnosis of this type of disease is difficult.

For these purposes, specialists usually use the following methods:
  • radiography;
  • Magnetic resonance imaging;
  • puncture or biopsy;
  • histological examination.

With the help of modern tools, it has become possible to determine the presence of sarcoma at an early stage of development. However, patients themselves often lose sight of the early signs of the disease, turning to the oncologist only when the disease is in an advanced stage. Therefore, periodic prophylaxis is necessary, consisting in a complete examination of the body for the presence of various diseases.

Sarcoma treatment

Usually, for the treatment of sarcomas, doctors offer the most effective and widespread methods:
  • surgical operations: laparoscopy or laparotomy;
  • transplantation techniques, including organ autotransplantation;
  • chemotherapy;
  • radioisotope exposure.

Recent studies have shown that chemotherapy or radiation therapy does not have a very high effect on the successful treatment of retroperitoneal sarcomas, so surgery is recognized as the most highly effective method. The introduction of improved transplantation methods into the treatment of cancer patients opens up the possibility of performing organ-preserving operations, which are ways to prolong the life of the patient and improve the quality of life.

Keywords

INORGANIC RETROPERITONEAL TUMORS / LIPOSARCOMA OF THE RETROPERITONEAL SPACE/ RECURRENT / SURGERY / ULTRASONOGRAPHY/ CHEMOTHERAPY / CYTOREDUCTION / THE EXTRA-ORGAN RETROPERITONEAL TUMORS/ RETROPERITONEAL LIPOSARCOMA / RECURRENCE / SURGICAL TREATMENT / ULTRASOUND / CHEMOTHERAPY / CYTOREDUCTION

annotation scientific article on clinical medicine, author of scientific work - Kulikov E.P., Kaminsky Yu.D., Vinogradov I.I., Kholchev M.Yu., Klevtsova S.V.

The article describes a clinical case liposarcomas of the retroperitoneum with a relapsing course and tactics of managing a patient with this pathology on the example of a specific clinical situation. A feature of the clinical example is a long history of liposarcoma with multiple relapsing course. At the time of admission to the Ryazan Regional Clinical Oncology Center, the patient was diagnosed with the fifth recurrence of liposarcoma. For the period from 2009 to 2015. the patient underwent 5 surgeries for the primary tumor and its relapses and 11 courses of chemotherapy. In July 2015, the patient underwent the sixth operation to remove recurrent retroperitoneal tumors and underwent a course of adjuvant intra-abdominal chemotherapy with cisplatin. The patient was discharged from the hospital under dynamic observation. At the next follow-up examination 10 months after the operation, clinically and according to ultrasound data, no recurrence was detected. Patients with relapses belong to the category of patients for whom there is currently no clearly defined management strategy. However, the surgical method remains the leading one in the treatment of patients with recurrent non-organ retroperitoneal tumors(NZO). The case report of the recurrent retroperitoneal liposarcoma is presented as well as the tactics of management patients with this pathology by the example of a certain clinical situation. The peculiarity of the clinical example of the presented patient is a long anamnesis of liposarcoma with the multiple recurrent course. At the time of admission to Ryazan Regional Clinical Oncology Center, the patient had the fifth recurrence of liposarcoma. During the period from 2009 to 2015 the patient underwent five operations on the primary tumor and its recurrences and 11 courses of chemotherapy . In July, 2015 the patient underwent the 6th operation to remove the recurrent tumors of the retroperitoneal space and the course of adjuvant intraperitoneal chemotherapy with cisplatin. The patient was discharged from the hospital to be dynamically observed. The next control examination (10 months later) showed no signs of disease recurrence according to clinical and ultrasound criteria. There is no consensus regarding the management of patients with recurrent retroperitoneal non-organic tumors and they are classified as the patients for whom today clearly defined treatment has not been established. However, the surgery remains the most successful treatment method of patients with recurrent retroperitoneal tumors and the presented clinical example demonstrates that the complete surgical resection is the only potential curative treatment modality (with maximal degree of cytoreduction ).

Related Topics scientific papers in clinical medicine, author of scientific work - Kulikov E.P., Kaminsky Yu.D., Vinogradov I.I., Kholchev M.Yu., Klevtsova S.V.

  • Results of surgical treatment of non-organ retroperitoneal tumors

    2015 / Afanasiev Sergey Gennadievich, Dobrodeev Alexey Yuryevich, Volkov Maxim Yuryevich
  • Surgical and combined treatment of mesenchymal non-organ retroperitoneal tumors

    2011 / Kharchenko V. P., Chkhikvadze V. D., Sdvizhkov A. M., Chazova N. L., Abdullaeva A. A.
  • Modern possibilities of diagnostics and treatment of patients with malignant non-organ retroperitoneal tumors

    2017 / Suleimanov E.A., Kaprin A.D., Kostin A.A., Moskvicheva L.I.
  • Non-organ retroperitoneal tumors: past and present

    2015 / Rasulov Rodion Ismagilovich, Dvornichenko Victoria Vladimirovna, Muratov Andrey Anatolyevich, Songolov Gennady Ignatievich, Mozgunov Dmitry Viktorovich
  • Reimplantation of the kidney with extended-combined removal of retroperitoneal liposarcoma (clinical observation)

    2017 / Rasulov Rodion Ismagilovich, Muratov Andrey Anatolevich, Dvornichenko Victoria Vladimirovna, Morikov Dmitry Dmitrievich, Teterina Tatiana
  • "Nephro-preserving" operations in the treatment of patients with non-organ retroperitoneal tumors

    2014 / Stilidi Ivan Sokratovich, Nikulin M. P., Davydov M. M., Gubina G. I.
  • 2011 / Kharchenko Vladimir Petrovich, Chkhikvadze Vladimir Davydovich, Abdullaeva Aziza Asralovna, Sdvizhkov A. M.
  • Angioplasty of the iliac vessels and abdominal aorta in a patient with retroperitoneal non-organ liposarcoma

    2016 / Stilidi I.S., Nikulin Maxim Petrovich, Abgaryan M.G., Kalinin A.E., Anurova O.A.
  • Surgical treatment of non-organ retroperitoneal tumors

    2012 / Kit O. I., Kasatkin V. F., Maksimov A. Yu., Moroshan A. N.
  • Immediate results of surgical treatment of non-organ retroperitoneal tumors

    2009 / Zubkov R. A., Rasulov R. I.

The text of the scientific work on the topic "Possibilities of surgical treatment of recurrent retroperitoneal liposarcomas"

doi: 10.18484/2305-0047.2016.5.513 E.P. KULIKOV 1, Yu.D. KAMINSKY 12, I.I. VINOGRADOV 2, M.Yu. KHOLCHEV 2, S.V. KLEVTSOV 1

POSSIBILITIES OF SURGICAL TREATMENT OF RECURRENT LIPOSARCOMAS OF THE RETROPERITONEAL SPACE

SBEE HPE "Ryazan State Medical University named after I.I. acad. I.P. Pavlov ", GBU "Ryazan Regional Clinical Oncological Dispensary" 2, Russian Federation

The article describes a clinical case of retroperitoneal liposarcoma with a recurrent course and the tactics of managing a patient with this pathology using a specific clinical situation as an example.

A feature of the clinical example is a long history of liposarcoma with multiple relapsing course. At the time of admission to the Ryazan Regional Clinical Oncology Center, the patient was diagnosed with the fifth recurrence of liposarcoma. For the period from 2009 to 2015. the patient underwent 5 operations for the primary tumor and its relapses and 11 courses of chemotherapy. In July 2015, the patient underwent the sixth operation to remove recurrent retroperitoneal tumors and underwent a course of adjuvant intra-abdominal chemotherapy with cisplatin. The patient was discharged from the hospital under dynamic observation. At the next follow-up examination 10 months after the operation, clinically and according to ultrasound data, no recurrence was detected.

Patients with recurrent non-organ retroperitoneal tumors belong to the category of patients for whom there is currently no clearly defined management strategy. However, the surgical method remains the leading one in the treatment of patients with recurrent non-organ retroperitoneal tumors (NRTs). The presented clinical example demonstrates that in the presence of recurrence of NZO, the only way to prolong life is active surgical tactics with the maximum degree of cytoreduction.

Keywords: non-organ retroperitoneal tumors, retroperitoneal liposarcoma, recurrence, surgical treatment, ultrasound, chemotherapy, cytoreduction

The case report of the recurrent retroperitoneal liposarcoma is presented as well as the tactics of management patients with this pathology by the example of a certain clinical situation.The peculiarity of the clinical example of the presented patient is a long anamnesis of liposarcoma with the multiple recurrent course. At the time of admission to Ryazan Regional Clinical Oncology Center, the patient had the fifth recurrence of liposarcoma. During the period from 2009 to 2015 the patient underwent five operations on the primary tumor and its recurrences and 11 courses of chemotherapy. In July, 2015 the patient underwent the 6th operation to remove the recurrent tumors of the retroperitoneal space and the course of adjuvant intraperitoneal chemotherapy with cisplatin. The patient was discharged from the hospital to be dynamically observed. The next control examination (10 months later) showed no signs of disease recurrence according to clinical and ultrasound criteria.

There is no consensus regarding the management of patients with recurrent retroperitoneal non-organic tumors and they are classified as the patients for whom today clearly defined treatment has not been established. However, the surgery remains the most successful treatment method of patients with recurrent retroperitoneal tumors and the presented clinical example demonstrates that the complete surgical resection is the only potential curative treatment modality (with maximal degree of cytoreduction).

Keywords: the extra-organ retroperitoneal tumors, retroperitoneal liposarcoma, recurrence, surgical treatment, ultrasound, chemotherapy, cytoreduction.

Possibilities of Surgical Treatment of Recurrent Retroperitoneal Liposarcomas

E.P. Kulikov, Y.D. Kaminsky, I.I. Vinogradov, M. Y. Holchev, S.V. Klevtsova

It is customary to single out their introduction into an independent nosological form. By classification

Non-organic retroperitoneal tumors (NZO) - International Cancer Union NZO

neoplasms that do not have an organ are classified as soft tissue sarcomas, however, due to

belongings developing from soft tissues, due to the rarity of this pathology, objective

located in the retroperitoneal space. statistics related to NZO, in

Clinical course, diagnostic principles are absent in our country.

and treatment of retroperitoneal sarcomas According to various authors, 60-80%

have many features, therefore, the histo-tumors of the retroperitoneal space are

are malignant, and 14-40% are benign. Malignant retroperitoneal tumors are characterized by a high percentage of recurrence after surgical treatment, but the metastatic potential of NZO is relatively low.

To date, no effective options have been proposed for the active detection of NSOs. Most patients come to specialized oncology clinics with massive, locally advanced tumors. The most common reasons for the neglect of the disease are late seeking medical help and errors in primary diagnosis due to the weak oncological alertness of doctors in the general medical network.

The surgical method is the leading one in the treatment of patients with NZO. However, the world is actively searching for new methods of treatment and studying the role of additional methods of exposure. The most important feature of surgical interventions in NZO is non-standard situations in almost all cases. Operations for NSO are among the most traumatic interventions with a high degree of operational risk. It is quite obvious that the greater the number of organs and anatomical structures undergoing resection or removal, the more difficult the stage of reconstruction and the higher the likelihood of complications. According to the Russian Cancer Research Center (RONTS) named after. N.N. Blokhin, the number of combined interventions for NSO in recent years has increased from 40.2% to 55.8% without an increase in postoperative mortality. This figure for combined operations even decreased to 3.08% compared to 4.8% in previous years. A similar trend is observed in operations that are not accompanied by multivisceral resections: 1.72% and 3.3%, respectively. At the same time, the number of radical surgeries increased from 61.9% to 84.33%. An increase in the degree of radicalism with a decrease in postoperative mortality makes it possible to consider aggressive surgical tactics in case of UO as quite justified.

The prognosis of the disease in liposarcomas (LS) largely depends on the histological subtype of the tumor. According to RONTS them. N.N. Blokhin, overall survival rates of 3, 5, 10 years, the median survival for the highly differentiated type was 77.9%, 55.6%, 30.8% and 85.0 months, for the dedifferentiated type - 61.5%, 50, 3%, 16.8% and 74.0 months, with pleomorphic - 28.6%, 14.3%, 0% and 12 months. . In patients with myxoid

the histological type of drugs with a round cell component content of less than 5%, overall survival rates are significantly better than in tumors with a round cell component content of more than 5%. The 3, 5-year survival rates were 84%, 63% versus 60% and 36%, respectively. Thus, the content of the round cell component in the tumor of more than 5% is a significant factor in the poor prognosis in myxoid LS.

Despite extensive combined interventions and the use of the entire range of measures aimed at preventing relapses, the results of surgical and combined treatment are unsatisfactory. The recurrence rate exceeds 50%. In the presence of a recurrence of NZO, an active surgical approach is justified with the maximum possible removal of all available tumor foci. This can significantly prolong the life of the patient.

The purpose of this article is to demonstrate a case of retroperitoneal liposarcoma with a recurrent course, describing the tactics of surgical treatment in a particular patient.

Clinical case

A 53-year-old patient was treated in the 1st surgical department of the Ryazan Regional Clinical Oncological Dispensary (ROKOD) from 06/23/2015 to 07/17/2015 with a diagnosis of retroperitoneal liposarcoma, recurrent course, treatment since 2009, multi-stage surgical treatment, courses chemotherapy, relapse.

The patient was admitted to the surgical department with complaints of a giant tumor in the abdominal cavity, abdominal pain, febrile temperature for 1 month. From the anamnesis it is known that since 2009 the patient has been treated at the Russian Cancer Research Center. N.N. Blokhin for retroperitoneal liposarcoma. Operated for a primary tumor in 2009; in 2010 and 2011 - removal of recurrent tumors, in the period from 2011 to 2013. 11 courses of chemotherapy were carried out. In 2013, another relapse was diagnosed, for which the patient was operated on twice. During the last operation, the right kidney was removed along with the recurrent tumor. Given the resistance of the tumor to chemotherapy and the risk of developing renal failure, adjuvant treatment was not performed. Since May 2015, the condition has worsened: pain in the abdominal cavity, weakness, fever, and an increase in the size of the abdomen have appeared. On examination

clinically and according to CT scan revealed a massive recurrence of the retroperitoneal tumor.

During additional examination: CT scan of the abdominal organs dated 17.06.2015. (RNRC named after N.N. Blokhin). In the abdominal cavity, a massive multinodular tumor formation (relapse of liposarcoma) is revealed, represented by large solid nodes up to 19 cm in diameter, mainly localized in the anterior sections of the abdomen, the stomach, body and tail of the pancreas are compressed between the nodes, the duodenum is spread on the back surface of one of the nodes intestine, the tumor is closely adjacent to the visceral surface of the left lobe of the liver. Separate inclusions of the tumor are detected behind the spleen, along the ascending and descending colon, between the loops of the sigmoid colon. The left adrenal gland is closely adjacent to the tumor (Fig. 1).

In June 2015, the patient was hospitalized in the 1st surgical department of the State Budgetary Institution ROKOD for further examination and to decide whether the recurrent tumor could be removed.

Ultrasound of the abdominal cavity dated June 22, 2015. The structure of the liver is heterogeneous, the gallbladder and ducts are without pathology, the pancreas: head 34 mm, body 21 mm, in the epigastric and mesogastal regions of the abdominal cavity, a group of isoechoic formations of a heterogeneous structure 140x90 mm in size, located nearby and forming a conglomerate. The conglomerate extends from the anterior abdominal wall to the aorta and iliac

Fig.1. CT scan of the abdominal organs. 1 - volumetric formations; 2 - spleen; 3 - compressed stomach; 4 - liver.

vessels, the contour of the conglomerate is hilly, relatively clear, the inferior vena cava is poorly visible. The right kidney is not defined, the left kidney without features.

X-ray of the stomach from 22.06.15. Signs of pushing the stomach from the outside with volumetric formations of the abdominal cavity, without signs of wall germination.

X-ray of the lungs dated 06/22/15. Shadows of a focal and infiltrative nature were not detected, the roots were structural, the sinuses were free.

FGS dated June 26, 2015. Signs of gastroduodenitis, submucosal formation of the esophagus.

Irrigoscopy dated June 29, 2015. Signs of displacement of the ascending colon.

On 07/07/2015, under combined anesthesia, the operation was performed: removal of recurrent tumors of the retroperitoneal space with removal of the greater and lesser omentums, B2 celiac retroperitoneal lymph node dissection, removal of tumors of the mesentery of the transverse colon and sigmoid colon.

Operation protocol. During the revision of the abdominal cavity and retroperitoneal space, it was found that a lot of tuberous multinodular tumors with clear boundaries, not associated with the main vessels, come from the retroperitoneal space:

No. 1 - from the lesser omentum, with a maximum diameter of 18 cm, with many tumor germs located along the branches of the celiac trunk and along the lesser curvature of the stomach;

No. 2 - from the root of the cervical organ under the lower edge of the pancreas, with a maximum diameter of more than 20 cm, pushing the stomach cranially, and the colon caudally (Fig. 2);

No. 3 - from the region of the gate of the spleen, with a maximum diameter of 14 cm and 10 cm;

No. 4 - in the greater omentum there are many small tumor germs from 1 cm to 3 cm;

No. 5 - in the mesentery of the sigmoid colon several tumor germs with a diameter of 1 to 6 cm;

No. 6 - in the region of the gate of the liver, several single tumor germs from 1 cm to 4 cm;

No. 7 - focus 5 cm, located between the bladder and rectum;

No. 8 - a tumor 5 cm in diameter, located behind the spleen on the diaphragmatic peritoneum.

All the tumors described above were mobilized and removed with B2 celiac lymph node dissection, removal of the greater and lesser omentums from the cardia to the duodenum with transection and ligation of the left gastric artery at the point of origin from the celiac trunk and preservation of the short gastric arteries (Fig. 3, 4).

Rice. 2. Intraoperative picture during revision of the abdominal organs and retroperitoneal space. 1 - tumor in the area of ​​the lesser omentum; 2 - tumor at the root of mesocolon; 3 - under the tupfer the colon and stomach, squeezed between the tumor nodes.

Rice. 3. View after removal of tumors from the mesocolon root, greater and lesser omentum. 1 - stomach after removal of the greater and lesser omentums; 2 - colon; 3 - pancreas; 4 - spleen; 5 - the bed of the tumor of the lesser omentum; 6 - mesocolon root tumor bed.

Rice. Fig. 4. Tumor elements removed from various 5. Liposarcoma of a differentiated structure of the abdominal cavity. Staining: hematoxylin and eosin. Boost*200.

The postoperative period was uneventful, healing by primary intention.

Histological conclusion No. 30829-38 dated July 10, 2015 - mixed liposarcoma of differentiated and myxoid structure, 0-2 (Fig. 5, 6).

On July 14 - July 16, 2015, a course of intra-abdominal chemotherapy with cisplatin No. 3 50 mg daily (total dose - 150 mg) was carried out. No adverse reactions or complications were noted.

On July 17, 2015, the patient was discharged from the hospital under dynamic observation.

May 10, 2016 The patient came for follow-up examination. Clinically and according to ultrasound data on recurrence was not revealed.

Discussion

Rice. 6. Myxoid liposarcoma. Staining: hematoxylin and eosin. Boost*200.

The article describes the tactics of managing patients with recurrent NZO on the example of

specific clinical case. A feature of the course of the disease in the presented patient is multiple relapses of liposarcoma, requiring repeated surgical intervention and courses of systemic and intracavitary chemotherapy.

Despite the fact that NZOs are characterized by a high probability of local recurrence, at present no one doubts the possibility and justification of surgical treatment of recurrent tumors.

The presented clinical case demonstrates the feasibility of an aggressive surgical approach in the treatment of this category of patients. Only repeated surgery with the maximum degree of cytoreduction made it possible to achieve a sufficiently long remission in this patient.

Conclusion

The presented clinical example demonstrates a rare case of successful treatment of retroperitoneal liposarcoma with multiple recurrent course.

The leading method of treatment that allows to prolong the life of patients with recurrent non-organ retroperitoneal tumors is optimal cytoreductive surgery.

Intra-abdominal chemotherapy should be considered as an additional method of antitumor effects in this category of patients.

The clinical case is presented with the consent of the patient.

LITERATURE

1. Babayan LA. Non-organ retroperitoneal tumors. Selected lectures on clinical oncology. Chissov VI, Daryalova SL, red. Moscow, RF; 2000. 735 p.

2. Klimenkov AA, Gubina GI. Non-organ retroperitoneal tumors: basic principles of diagnosis and surgical tactics. Prakt Oncology. 2004;5(4):285-90.

3. Kulikov EP, Ryazantsev ME, Zubareva TP, Sudakov IB, Kaminsky YUD, Sudakov AI, et al. Dynamics of incidence and mortality from malignant neoplasms in the Ryazan region in 2004-2014. Ros Med-Biol Vestn named after Akad IPPavlov. 2015;(4):109-15.

4. Mytsyk YuO. The role of diffuse-weighted imaging of magnetic resonance imaging in the differential diagnosis of malignant neoplasms of the kidney. Science of the Young. 2014;(4): 12127.

5. Chissov VI, Davydov MI. Oncology: national hands. Moscow, RF: GEOTAR-Media; 2008. 1072 p.

6. Stolyarov VI, Gorzov PP. Combined operations for non-organ tumors of the retroperitoneal space. Question of Oncology. 1996; 42(1):103-105.

7. Stilidi IS, Gubina GI, Nered SN, Klimenkov AA, Selchuk VYu, Tyurin IE, et al. Immediate results of surgical treatment of non-organ retroperitoneal tumors. Ros Oncol Zhurn. 2007;(1):25-28.

8. Nered SN, Stilidi IS, Klimenkov AA, Bolotsky VI, Anurova OA. Clinical and morphological features and results of surgical treatment of retroperitoneal non-organ liposarcomas. Question of Oncology. 2012;58(1):94-100.

9. Barnes L, Tse LLY, Hunt JL, Michaels L. Ch 8: Tumors of the Paraganglionic System: introduction. In: Barnes L, Eveson JW, Reichart P, Sidransky D, eds. Pathology and genetics of head and neck tumors. Lyon: IARC Press; 2005. p. 362-70.

10. Gronchi A, Casali PG, Fiore M, Mariani L, Lo Vullo S, Bertulli R, et al. Retroperitoneal soft tissue sarcomas: patterns of recurrence in 167 patients treated at a single institution. cancer. 2004 Jun 1;100(11):2448-55.

11. Lehnert T, Cardona S, Hinz U, Willeke F, Mechtersheimer G, Treiber M, et al. Primary and locally recurrent retroperitoneal soft-tissue sarcoma: local control and survival. Eur J Surg Oncol. 2009 Sep;35(9):986-93. doi: 10.1016/j.ejso.2008.11.003.

12. van Dalen T, Hoekstra HJ, van Geel AN, van Coevorden F, Albus-Lutter C, Slootweg PJ, et al. Lo-coregional recurrence of retroperitoneal soft tissue sarcoma: second chance of cure for selected patients. Eur JSurg Oncol. 2001 Sep;27(6):564-68.

1. Babaian L.A. Neorgannye zabriushinnye opukholi. Izbrannye lektsii po klinicheskoi onkologii Chissov VI, Dar "ialova SL, red. Moscow, RF; 2000. 735 p.

2. Klimenkov AA, Gubina GI. Neorgannye zabri-ushinnye opukholi: osnovnye printsipy diagnostiki i khirurgicheskoi taktiki. Prakt Onkologia. 2004;5(4):285-90.

3. Kulikov EP, Riazantsev ME, Zubareva TP, Sudakov IB, Kaminskii IuD, Sudakov AI, i dr. Dinamika zabolevaemosti i deathnosti ot zlokachestvennykh no-voobrazovanii v Riazanskoi oblasti v 2004-2014 godakh . Ros Med-Biol Vestn im Akad IP Pavlova. 2015;(4):109-15.