Malignant tumors. Sarcoma: types of tumors, signs, causes, treatment and prognosis. Is sarcoma cancer or not?

The nature of malignant tumors is very diverse. There are many various forms cancers, differing in structure, degree of malignancy, origin and type of cells. One of dangerous formations is considered a sarcoma, which will be discussed.

What is this disease?

Sarcoma is a malignant tumor process that originates from the connective tissue cell structures.

According to statistics, the prevalence of sarcomas among all is about 5%. Such tumors are characterized by a very high lethality, since they are characterized by an aggressive course.

Localization

This type of tumor does not have any strict localization, since connective tissue elements are present in all structures of the body, so sarcomas can occur in any organ.

In the photo you can see what the initial stage of Kaposi's sarcoma looks like

The danger of sarcoma is also that this tumor in a third of cases affects young patients under 30 years old.

Classification

Sarcoma has many classifications. According to the origin of the sarcoma are divided:

On tumors originating from hard tissues;
From soft tissue structures.

According to the degree of malignancy, sarcomas are divided into:

Highly malignant– rapid division and growth of tumor cell structures, few stroma, widely developed vascular system tumors;
with low malignancy- cell division occurs with low activity, they are perfectly differentiated, the content of tumor cells is relatively low, there are few vessels in the tumor, and stroma, on the contrary, there are many.

Depending on the type of tissue, sarcomas are classified into, reticulosarcomas, cystosarcomas, etc.

According to the degree of differentiation, sarcomas are divided into several varieties:

GX - it is impossible to determine the differentiation of cellular structures;
G1 - highly differentiated sarcoma;
G2 - moderately differentiated sarcoma;
G3 - poorly differentiated sarcoma;
G4 - undifferentiated sarcomas.

Cell differentiation involves determining the type of cells, the type of tissue to which they belong, etc. With a decrease in cell differentiation, the malignancy of the sarcoma increases.

With the growth of malignancy, the tumor begins to grow intensively, which leads to increased infiltrativity and even more rapid development tumor process.

Causes of the disease

The exact causes of sarcomas have not been established, however, scientists have established a connection between certain factors and the tumor.

Heredity, genetic conditioning, the presence of chromosomal pathologies;
Ionizing radiation;
Carcinogenic influence like cobalt, nickel or asbestos;
Abuse of ultraviolet radiation with frequent visits to the solarium or prolonged exposure to the scorching sun;
Viruses like herpesvirus, HIV or Epstein-Barr virus;
Harmful industries associated with the chemical industry or oil refining;
immune failures, causing development autoimmune pathologies;
The presence of precancerous or;
Long ;
Hormonal disruptions in the process of puberty, which lead to intensive growth of bone structures.

Such factors cause uncontrolled division of cellular connective tissue structures. As a result, abnormal cells form a tumor and grow into neighboring organs and destroy them.

Symptoms of sarcoma of various organs

Clinical manifestations of sarcoma differ in accordance with the specific form of the tumor, its localization, and the degree of development of the sarcoma.

At the first stage of the tumor process, a rapidly progressive formation is usually detected, but with its development, neighboring structures are also involved in the tumor process.

Abdomen

Sarcomas that form in the abdominal cavity have symptoms similar to cancer. Abdominal sarcomas develop in the tissues of various organs.

Liver. It is rare, manifested by painful symptoms in the right hypochondrium. Patients noticeably lose weight, the skin becomes icteric, in the evenings hyperthermia worries;
Stomach. It is characterized by a long asymptomatic onset. Often discovered by chance. Patients note the appearance of dyspeptic disorders such as nausea, heaviness, flatulence and bloating, rumbling, etc. Gradually, signs of exhaustion increase, the patient constantly feels tired, weakened, depressed and irritable;
Intestine. Such sarcomas are accompanied by pain in the abdomen, weight loss, nausea and belching, lack of appetite, frequent diarrhea, bloody-mucous discharge from the intestine, frequent bowel movements, rapid depletion of the body;
Kidneys. Sarcoma in the renal tissues is characterized by pronounced hematuria, soreness in the area where the formation is located, and the tumor can be felt on palpation. Blood in the urine does not cause any other discomfort or urinary disorders;
Retroperitoneal space. Most often, the sarcoma grows to a significant size, squeezing neighboring tissues. The tumor can destroy nerve roots, vertebral elements, which is accompanied by intense pain in the corresponding areas. Sometimes such a sarcoma causes paralysis or paresis. When the tumor occludes blood vessels, edema of the extremities and abdominal walls. If the hepatic circulation is disturbed, then it develops, etc.
Spleen. In the early stages of development, the sarcoma does not manifest itself in any way, but with the growth of education, the organ increases, then the tumor begins to disintegrate, which is accompanied by an intoxication clinic like subfebrile temperature, anemia and progressive weakness. Also, splenic sarcoma is characterized by a constant feeling of thirst, lack of appetite, apathy, nausea and vomiting, frequent urges to urination, pain, etc .;
Pancreas. Such a sarcoma tumor is characterized by pain, hyperthermia, weight loss, loss of appetite, diarrhea or constipation, jaundice, malaise and general weakness, nausea and vomiting symptoms, belching, etc.

As can be seen, sarcomas of organs located in the abdominal cavity are often accompanied by similar symptoms.

The organs of the chest

Tumors of similar localization most often arise as a result of metastasis from other foci. Symptoms vary depending on location.

Rib sarcoma. At first, the patient feels pain in the region of the ribs, chest and surrounding tissues, gradually the pains increase, soon even anesthetics cannot cope with them. A swelling is felt on the ribs, which, when pressed, causes pain. The patient is disturbed by symptoms such as irritability, excessive excitability, anxiety, anemia, fever, local hyperthermia, respiratory disorders.
. Such formations are indicated by a symptom such as excessive fatigue, shortness of breath, dysphagia (with metastasis to the esophagus), nausea and vomiting symptoms, hoarseness, pleurisy, cold symptoms, prolonged pneumonia, etc.
Heart and pericardium. The tumor manifests itself with slight hyperthermia, weight loss, joint pain and general weakness. Then rashes appear on the body and limbs, a clinical picture of heart failure develops. Patients have swelling of the face and upper limbs. If the sarcoma is localized in the pericardium, then the symptoms suggest the presence of hemorrhagic effusion and tamponade.
Esophagus. Symptoms of esophageal sarcoma are based on violations of swallowing processes and pain. Pain symptoms are concentrated behind the sternum, but can radiate to vertebral divisions and scapular region. There is always inflammation of the esophageal walls. As in other cases, sarcoma is accompanied by anemia, weakness and weight loss. Such a pathology ultimately leads to the complete exhaustion of the patient.
Mediastinum. The tumor spreads to all tissues of the mediastinum and squeezing and growing into the organs located in it. When the tumor penetrates into the pleura, exudate appears in its cavities.

Spine

Sarcoma of the spine is a tumor malignant formation in the spinal tissues and adjacent structures. Danger of pathology in compression or damage spinal cord or its roots.

Symptoms of vertebral sarcoma are determined by its localization, for example, in the cervical, thoracic, lumbosacral, or ponytail.

However, all lumbar tumors have common features:

Rapid tumor development (in a year or less);
In the department affected by the tumor, pain is felt, which is characterized by a constant character, which is not eliminated by anesthetics. Although at first it is weakly expressed;
Limited mobility of the affected vertebrae, forcing patients to take a forced position;
Complications of a neurological nature in the form of paresis, paralysis, dysfunction of the pelvis, which are among the first to manifest themselves;
The general condition of the patient with vertebral sarcoma is assessed by doctors as severe.

Brain

The main symptoms of cerebral sarcoma are:

unexplained headaches;
Frequent dizziness with loss of consciousness, movements become uncoordinated, vomiting is often disturbing;
Behavioral disorders, mental disorders;
Frequent cases of epileptic seizures;
Temporary visual disturbances, however, against the background of constantly elevated ICP, there is a high risk of developing optic nerve atrophy;
The development of partial or complete paralysis.

Ovary

Ovarian sarcoma is characterized by large size and fast growth, meager symptoms like pulling-aching pains, heaviness in the lower abdomen, menstrual disorders, sometimes ascites. Sarcoma is often bilateral and has a very rapid development.

Eyes

Primary sarcomas usually form in upper parts eye sockets, and similar shape more common in children.

Such tumors grow rapidly, increasing their size. There is swelling and pain in the eye socket. Eyeball limited in mobility and shifted, exophthalmos develops.

Blood and lymph

The clinical picture of lymphosarcoma depends on primary focus Or rather, its localization. Lymphosarcomas are most often B-cell in nature and are similar in course to acute leukemia.

Diagnostics

Diagnostic measures involve standard procedures such as:

MRI either;
Radiography;
Radionuclide research
Neurovascular or morphological diagnostics;
etc.

How to treat sarcoma?

Treatment of sarcoma is predominantly surgical with additional or. Exactly combined treatment ensures maximum efficiency.

This approach contributes to an increase in survival up to 70% of cases. Since the tumor is sensitive to radiation, such a technique necessarily complements surgical removal.

Prognosis and survival

Forecasts for sarcoma are determined by the stage of the tumor process, its shape and localization, the presence of metastases, etc.

For example, gastric sarcoma in a third of cases is characterized by early metastasis, which negatively affects the prognosis. Retroperitoneal sarcoma is difficult to predict, because it has a lot of options clinical course with different outcomes.

Survival depends on the stage of development of such a tumor, the presence of metastases and the response to therapeutic effects. If the tumor was detected at the final or thermal stage, then the survival rate is low.

More accurate predictions of survival depend on the specific type of sarcoma, each of which requires an individual approach. Of no small importance is the response to treatment, the condition of the patient and other factors.

In other words, myeloid sarcoma ("chloroma", "granulocytoma") is one of the extramedullary (that is, extra-medullary) manifestations of acute myeloid leukemia. That is, it is a collection of leukemic cells, characteristic of acute myeloid leukemia, somewhere outside the bone marrow and blood.

Historical information

The disease, now known as myeloid sarcoma, was first described by the British physician A. Burns in 1811. . However, the term chloroma” in relation to this disease was first used only in 1853. The term is derived from the Greek χλωροΣ (chloros, chloros), meaning "green", "pale green", as these tumors are often green or pale green in color due to the presence of myeloperoxidase in them. The close relationship between "chloroma" and acute myeloid leukemia was first discovered in 1902 by Warthin and Doc. However, since up to 30% of these tumors may be white, grey, pink, or Brown color instead of the "classical" green or greenish, and also in order to more accurately classify these tumors histologically, Rappaport in 1967 suggested calling them not by color, but by cell type - the term "granulocytic sarcoma". Since then, the term has become practically synonymous with the obsolete term "chloroma". But, since the cells that make up this tumor are still not mature granulocytes, but blast cells, and, in addition, may not belong to granulocytic, but, for example, monocytic (in acute monocytic leukemia), erythroid, etc. hematopoietic germ, in accordance with the form of AML according to FAB, then in last years instead of the term "granulocytic sarcoma", the more scientifically correct term "myeloid sarcoma" is used.

Currently, according to the definition of the term, any extramedullary (extramedullary) manifestation of acute myeloid leukemia can be called myeloid sarcoma. However, according to the established historical tradition, some special leukemic lesions are called by their specific names:

Skin leukemids, a term describing the infiltration of the skin by leukemic cells with the formation of specific infiltrate nodules, they are also called "cutaneous myeloid sarcoma" (formerly "cutaneous granulocytic sarcoma").
« meningoleukemia" or " meningeal leukemia”, a term describing the invasion of leukemic cells into the subarachnoid space and involvement in the leukemic process meninges, is usually considered separately from myeloid sarcoma ("chloroma"). However, those very rare cases in which a solid tumor of leukemic cells arises in the CNS may nevertheless, by definition, be called CNS myeloid sarcoma.

Frequency and typical clinical manifestations

For acute leukemia

Myeloid sarcomas are rare disease. The exact frequency of their occurrence is unknown, but they are rarely observed even by hematologists who specialize in the treatment of acute myeloid leukemia.

Myeloid sarcomas may be somewhat more common in patients with the following disease features:

FAB class M2, i.e. Acute myeloblastic leukemia with maturation;
Patients whose leukemic cells have certain specific cytogenetic abnormalities, such as t(8;21) or inv(16);
Patients whose myeloblasts express CD13 or CD14 T-cell surface antigens
Patients with a high number blast cells in the blood or a high level of LDH, that is, with a large total tumor mass.

However, even in patients with the above risk factors or a combination of them, myeloid sarcoma is rare complication AML.

Sometimes myeloid sarcoma can develop as the first (and for the time being the only) manifestation of recurrence after seemingly successful treatment of acute myeloid leukemia. In line with the clinical behavior of myeloid sarcomas, which always are systemic disease from the very beginning (the concept of "metastasis" does not apply to them), all these cases should be considered and treated as early signs of systemic recurrence of AML, and not as a localized process. So, in one review, 24 patients in whom, after seemingly successful treatment AML developed relapses in the form of isolated myeloid sarcomas, it was shown that the average time from the onset of myeloid sarcoma to the establishment of a clear bone marrow recurrence was only 7 months (range - from 1 to 19 months). And this review was published in 1994, long before the invention of modern molecular techniques that can demonstrate the presence of bone marrow "molecular" recurrence much earlier than it becomes obvious histologically.

With myelodysplastic and myeloproliferative syndromes, including chronic leukemias

Myeloid sarcomas may occur in patients diagnosed with myelodysplastic syndrome or myeloproliferative syndrome, such as chronic myeloid leukemia, polycythemia vera, essential thrombocytosis, or myelofibrosis. The discovery of myeloid sarcoma of any localization in a patient with such a diagnosis is considered de facto evidence that these premalignant or low-grade chronic diseases have transformed into acute myeloid leukemia, requiring immediate adequate treatment. For example, the appearance of myeloid sarcoma in a patient with chronic myeloid leukemia is sufficient evidence that this patient's CML has passed into the "blast crisis" phase. At the same time, the presence of other signs, such as bone marrow blastosis or blastosis in the blood, is not necessary to state the fact of a blast crisis.

Primary myeloid sarcoma

In very rare cases Myeloid sarcoma can occur in a patient without simultaneously meeting the criteria for the diagnosis of acute myeloid leukemia (by bone marrow and blood), myelodysplastic or myeloproliferative syndrome (including chronic myeloid leukemia) and without a previous history of suffering from these diseases. This condition is known as "primary myeloid sarcoma". Diagnosis in these cases can be particularly difficult. In almost all cases of primary myeloid sarcoma, classic, systemic ("bone marrow") acute myeloid leukemia soon develops. The median time from diagnosis of "primary myeloid sarcoma" to the development of overt acute myeloid leukemia is 7 months (range, 1 to 25 months). Therefore, the detection of primary myeloid sarcoma should be considered as an early initial manifestation acute myeloid leukemia, and not as a localized process, and, accordingly, serve as the basis for the diagnosis of "acute myeloid leukemia" of the corresponding histological form and prescribing treatment appropriate to the histological form of AML, risk group, cytogenetics and immunophenotype of the tumor. In particular, if a myeloid sarcoma consisting of promyelocytes is detected (AML type M3 according to FAB, acute promyelocytic leukemia), then treatment should correspond to AML M3 and include not only and not so much chemotherapy, but, above all, the use of all-trans retinoic acid ( ATRA) and arsenic trioxide.

Location and symptoms

Myeloid sarcoma can occur in almost any organ or tissue. However, the most common localizations of the process are the skin (a condition known as "skin leukemids", English leukemia cutis) and gums. Skin involvement in a leukemic process usually appears as pale, sometimes purple or greenish, painless, raised plaques or nodules that are infiltrated by leukemic cells (myeloblasts) on biopsy. Cutaneous leukemids must be distinguished from the so-called "Sweet's syndrome", in which the skin is infiltrated by healthy (non-cancerous) mature neutrophils, which is a reactive paraneoplastic process. Involvement in the leukemic process of the gums leads to characteristic manifestation Pale, swollen, hyperplastic, sometimes painful gums that bleed easily when brushing or other minor trauma.

Other organs and tissues that may be involved in the leukemic process include, in particular, the lymph nodes, stomach, small and large intestines, abdominal cavity and mediastinum, lungs, epidural spaces, testicles, uterus and ovaries, orbit of the eye. Symptoms of myeloid sarcoma in this case depend on its anatomical localization. Myeloid sarcomas can also be asymptomatic and discovered incidentally during the examination of a patient, especially a patient with acute myeloid leukemia.

However, with the advent of modern diagnostic techniques, such as immunophenotyping and immunohistochemistry, the diagnosis of myeloid sarcoma today can be made much more reliably than before, with less delay in establishing correct diagnosis and with fewer initial diagnostic errors (misdiagnosis). Thus, Travek et al described the successful use of a commercially available panel of anti-myeloperoxidase monoclonal antibodies, CD68, CD43, and CD20 surface antigens for tissue immunohistochemical staining in order to accurately and correctly diagnose myeloid sarcomas and distinguish them from lymphomas. Today, in order to diagnose and differentiate myeloid sarcomas and lymphomas, immunohistochemical staining with monoclonal antibodies to CD33 and CD117 antigens is mainly used. Increasing availability and ever more accurate and correct use flow cytometry also contributed to the improvement of early and correct diagnosis these tumors.

predictive value

Experts disagree on the prognostic value of the presence of myeloid sarcomas in patients with acute myeloid leukemia. In general, it is generally accepted that the presence of myeloid sarcomas means a worse prognosis, with poorer response to therapy, less chance of remission, and worse overall and disease-free survival. However, other experts believe that the presence of myeloid sarcomas in itself is associated with other unfavorable biological markers of the tumor, such as the expression of adhesion molecules, T-cell antigens, unfavorable cytogenetic abnormalities, a large tumor mass ( high level LDH in the blood or high blast leukocytosis), and therefore the presence of myeloid sarcomas in itself does not carry any additional prognostic information and is not an independent prognostic factor.

Treatment

As described above, myeloid sarcomas should always be considered as another manifestation of a systemic disease - acute myeloid leukemia, and not as an isolated local phenomenon, and therefore should be treated systemically according to the protocols intended for the treatment of acute myeloid leukemia. Accordingly, in a patient with both primary myeloid sarcoma and newly diagnosed acute myeloid leukemia, systemic chemotherapy according to protocols designed for the treatment of acute myeloid leukemia (such as 7+3, ADE, FLAG, etc.) should be used as first line therapy. Given the less favorable, on average, prognosis in patients with myeloid sarcoma compared with patients with AML without extramedullary manifestations, it may make sense to use more aggressive induction and consolidation chemotherapy regimens (for example, ADE or HDAC instead of "7 + 3") and early - in the first remission - high-dose chemotherapy and allogeneic transplantation of hematopoietic stem cells. Topical treatment is generally not indicated and is not necessary, as myeloid sarcomas tend to be quite sensitive to standard systemic antileukemic chemotherapy. Besides, local treatment(surgery or radiation therapy) is associated with the risk of complications (for example, in the case of surgery - infections and bleeding) and postponing the start of chemotherapy, which is dangerous in AML with its rapid progression. An exception is cases when the anatomical localization of myeloid sarcoma threatens the functioning of one or another vital important body(for example, causes compression of the spinal cord with dysfunction pelvic organs or threatened rupture of the spleen, or intestinal obstruction). In this case it can be shown emergency operation or radiation therapy to the affected area in parallel with possibly more early start intensive anti-leukemic chemotherapy. Also, local radiation therapy or surgery can be a palliative measure for those who cannot receive chemotherapy of any kind (which is rare - there are alternative regimens for the elderly, debilitated) or who refuse it.

If myeloid sarcoma persists (remains in place) after completion of induction chemotherapy, the management should be the same as for resistant (refractory) acute myeloid leukemia - that is, try second- and third-line chemotherapy that does not cross-resistance with the first regimen, high-dose chemotherapy and allogeneic hematopoietic stem cell transplantation. In addition to or as a palliative measure (for those who cannot continue chemotherapy) - but only in addition, not instead of systemic chemotherapy II or III line, allotransplantation - surgical removal of myelosarcoma or local radiation therapy may be considered. However, none of local methods does not improve patient survival.

Patients with isolated primary myeloid sarcoma should also receive systemic antileukemic therapy rather than topical treatment, as the development of the typical "bone marrow" acute myeloid leukemia soon (measured in weeks or months) after the diagnosis of primary myeloid sarcoma is almost inevitable, and treatment for both conditions equally. In fact, such patients in most cases are diagnosed with acute myeloid leukemia, initial extramedullary manifestations of the corresponding histological form, and not with the diagnosis of primary myeloid sarcoma.

Patients treated for acute myeloid leukemia who relapse after treatment with isolated myeloid sarcoma should be treated in the same way as patients with systemic relapse (i.e., line II and III chemotherapy, allogeneic bone marrow transplantation). However, as with any relapse of acute myeloid leukemia, the prognosis is usually poor, especially if it is not the first recurrence (than more quantity already experienced relapses, the more difficult it is to achieve remission with chemotherapy, the shorter the remission, the more aggressive the behavior of the tumor and the higher its resistance to chemotherapy).

Patients with "pre-leukemic" conditions, such as myelodysplastic syndrome, chronic myeloid leukemia, polycythemia vera, and other myeloproliferative diseases, should be treated as if their disease had progressed to acute myeloid leukemia (or, in case of CML, suffered a "blast crisis"). That is, again, they should receive systemic antileukemic chemotherapy. Taking into account the fact that in patients with a history of myelodysplastic or myeloproliferative syndrome (in particular, with CML blast transformation), the prognosis is always worse than in patients with de novo AML, more aggressive induction and consolidation chemotherapy and early allogeneic chemotherapy in the first remission make sense for them. transplantation of hematopoietic stem cells.

Links

James, William D.; Berger, Timothy G.; et al. Andrews" Diseases of the Skin: clinical Dermatology. - Saunders Elsevier, 2006. - ISBN 0-7216-2921-0.

Epithelioid sarcoma

Epithelioid sarcoma is a tumor up to five centimeters in size, which looks like a dense knot. When cutting her body, it is determined light color structure with rare patches of brown or red. At histological examination, epithelioid sarcoma includes eosinophilic epithelioid and spindle cells.

In the photo: Histology of epitheloid sarcoma

Diagnosis is based on characteristic clinical picture, physical examination, instrumental and laboratory data. Differential diagnosis epithelioid sarcoma is exposed in order to distinguish it from tumors such as histiocytoma, fibromatosis, rhabdomyosarcoma. Epithelioid sarcoma must be susceptible complex therapy, only in this case a favorable outcome of this pathology is possible.

Gliosarcoma

Gliosarcoma is a malignant neoplasm that develops in the structures of the central nervous system from glia. It contains neuroglial cells, as well as components of sarcomatous origin. Gliosarcoma originates as a result of mesodermal and ectodermal cellular degeneration.

Diagnosis is based on the application of such measures as:

Echoencephaloscopy.
Magnetic resonance imaging.
The use of computed tomography.
Collection of material by biopsy for histological examination.

Medical tactics in case of detection of gliosarcoma, it comes down to the intervention of neurosurgeons, the use of cytostatics and other components of chemotherapy, as well as radiation exposure to the tumor.

Neurosarcoma

Neurosarcoma is another type of tumor that develops in the central nervous system from neuroblasts and ganglionocytes. There are such types of it:

Ganglioneuroblastoma.
Astroblastoma.
Neuroblastoma.
Glioblastoma.
Schwann tumor

Among the reasons for its formation, experts often mention gender, a certain age, genetic predisposition, exposure to radiation and other carcinogens, as well as occupational hazards. characteristic symptoms it is customary to consider frequent epileptic seizures, up to epistatus, the presence of focal cerebral symptoms, as well as hemorrhagic strokes.

As diagnostic methods, tomography, magnetic resonance imaging, radioisotope analysis, angiography, and cerebrospinal fluid examination are used. Treatment tactics include radiosurgery and chemotherapy.

Neurofibrosarcoma

Neurofibrosarcoma is a tumor that includes in its structure the occurrence nerve cells. Tumors in this class include the following:

Neurogenic sarcoma.
Neurofibrosarcoma.
Neurinoma.

Neurogenic sarcoma occurs in five percent of cases among all sarcomas. The development of this pathology is most often observed with malignant transformation of neurofibromatosis. The substrate is the shell nerve fibers. The risk group includes young people under thirty-five years of age. Macroscopically, the thickening of the nerve trunk is determined. Sometimes germination develops in blood vessels. Infrequent metastasis is a good predictor of survival, which is ninety percent.

Spindle cell sarcoma

Spindle cell sarcoma is one of the subspecies, which is characterized by a feature in histological structure. On a section of a micropreparation, it consists of cells that look like a spindle, namely, they have an elongated shape and a hyperchromic nucleus.

Polymorphic cell sarcoma

Polymorphic cell sarcoma is a primary developing tumor, which is a dense dermal nodule, which is characterized by more peripheral growth and the presence of a border of reddened skin. The feature lies in total absence symptoms with the presence of cachexia and syndrome complete exhaustion. The treatment used today is only surgical.

Histiocytic sarcoma

Histiocytic sarcoma is a rare, aggressive tumor that most often involves the digestive tract, skin, and soft tissues. Metastatic lesions of the spleen, liver, bone marrow and structures of the central nervous system are the most frequent.

Clear cell sarcoma

Clear cell sarcoma - slow developing species tumor lesion soft tissues, which is most often observed in the area distal departments upper limbs.

Pleomorphic sarcoma

Pleomorphic sarcoma is a diagnosis that is established on the basis of an immunohistochemical study in the presence of an undifferentiated tumor.

myeloid sarcoma

Myeloid sarcoma, or as it is also called, granulocytic sarcoma, is a malignant neoplasm that develops in the hematopoietic system. It is classified as a myeloid leukemia. The exact causes of development are still not known to modern medicine, but a genetic predisposition has been proven. Myeloid sarcoma manifests itself at the initial stages of its development as a seal skin, which is slightly elevated above the level of other tissues.

It then develops into hyperplasia. purple, to which is attached pain syndrome. Myelosarcoma is enough dangerous disease, because long time does not show itself. Possible symptoms iron deficiency anemia, which later becomes aplastic. With a long course, myeloid sarcoma can be characterized by hematogenous metastasis to distant tissues with their secondary lesion. Myeloid sarcoma is diagnosed based on the following methods:

Histological examination of the material taken during trepanobiopsy.
Study of the cerebrospinal fluid.
Clinical blood test.
Ultrasound examination of the spleen and liver.
Computed, magnetic resonance imaging.

Myeloid sarcoma must necessarily be subject to complex treatment, which includes chemotherapy and exposure therapeutic doses radiation.

A malignant tumor, a tumor that is characterized by invasiveness (the ability to grow into surrounding tissues and destroy them) and metastasis. The two main types of cancer are cancer and sarcoma. Leukemias are also classified as malignant tumors.

Sarcoma

Sarcoma (from Greek sbrx, Genitive sarkus - meat and - oma - ending in the names of tumors; the name is due to the fact that S. on the cut resembles raw fish meat), malignant tumor from connective tissue. There are mesenchymoma - sarcoma from embryonic connective tissue and sarcoma from mature tissues of mesenchymal origin - bone (osteosarcoma) and cartilage (chondrosarcoma), vascular (angiosarcoma) and hematopoietic (reticulosarcoma), muscle (leiomyosarcoma, rhabdomyosarcoma) and supporting elements of the nervous tissue (gliosarcoma) . Sarcomas account for up to 10% of all malignant tumors, in some countries of Africa and Asia they are relatively more common. Among sarcomas, bone tumors are the most common, followed by soft tissue tumors - muscular, vascular, nervous; sarcoma of hematopoietic organs are less commonly observed. According to the histomorphological picture, round-celled, polymorphic-celled (sometimes giant-celled), spindle cell - full-time sarcomas (they all differ in the shape and size of cells) and fibrosarcomas (they differ in the predominance of fibrous elements over cellular ones) are distinguished. The property of all malignant tumors - to grow into the surrounding tissues and destroy them - is especially pronounced in sarcoma. Unlike cancer that metastasizes on a relatively early stages in the next The lymph nodes, sarcomas usually spread along blood tract and often give early metastases to distant organs. The principles and methods of diagnosis, prevention and treatment of sarcoma are the same as for other malignant tumors.

Leukemia

Leukemia (from the Greek leukus - white), leukemia, leukemia, a tumor systemic disease of the hematopoietic tissue. With L., a violation of hematopoiesis occurs, expressed in the growth of immature pathological cellular elements both in the hematopoietic organs themselves and in other organs (kidneys, vessel walls, along the nerves, in the skin, etc.). - a rare disease (1 per 50 thousand people). There are spontaneous leukemias, the cause of which is not established, radiation (radiation) leukemias that occur under the influence of ionizing radiation and leukemias that occur under the influence of certain chemical, so-called leukemic (blastomogenic) substances. In a number of animals (chickens, mice, rats and dogs, cats and large cattle), suffering from leukemia, it was possible to isolate leukemia viruses. Viral etiology human leukemia has not been proven. Depending on the cellular morphology, leukemia is divided into reticulosis and hemocytoblastosis, myeloid leukemia and erythromyelosis, megakaryocytic leukemia, etc. The following forms of leukemia are distinguished from the degree of increase in the total number of leukocytes and the "flood" of blood with young, pathological cells: leukemic, subleukemic, leukopenic and a leukemic (the number of leukocytes in the blood is not increased, and at the same time, young, pathological forms). Aleukemic L., occurring with pronounced tumor growth, is usually referred to as reticulosis.

A distinction is made between acute and chronic leukemias. Acute are characterized by a rapid course and characteristic picture blood, which is caused by a break in hematopoiesis at a certain stage, as a result of which the most immature forms- blasts into mature blood cells, the hemogram is characterized by varying degrees of "blastemia" with a small number of mature leukocytes and the absence of transitional forms. As a rule, acute leukemia occurs with fever, severe anemia, bleeding, ulceration and necrosis in various organs. Chronic leukemias are distinguished depending on the lesion of one or another branch of hematopoiesis: chronic myelosis (myeloid leukemia), lymphadenosis (lymphatic leukemia), histio-monocytic L., erythromyelosis, megakaryocytic L. The most common form is chronic myelosis, characterized by hyperplasia (growth) of elements of the bone marrow ( myeloid) hematopoiesis both in the bone marrow itself (fatty bone marrow of long tubular bones is replaced by red, hematopoietic bone marrow), and in the spleen, which reaches a significant size, in the liver, in the lymph nodes, where normal lymphoid tissue is replaced by pathological myeloid elements. The blood is flooded with granular leukocytes (young, mature and transitional forms). Chronic lymphadenosis, as a rule, proceeds for a long time, relatively benignly. The disease develops gradually, characterized by an increase in predominantly lymph nodes, although sometimes an increase in the spleen and liver predominates. In the bone marrow, there is a replacement of normal, myeloid bone marrow by lymphoid. The blood is flooded with lymphocytes with a predominance of mature forms. During exacerbations, blasts appear. Over time, anemia develops due to suppression of the normal hematopoietic function of the bone marrow by lymphoid infiltrates, as well as as a result of the loss of immune competence by pathological lymphocytes, the production of autoaggressive antibodies, in particular anti-erythrocyte antibodies, causing hemolysis; in some cases, abnormal lymphocytes produce antiplatelet antibodies, leading to thrombocytopenia and bleeding. With an exacerbation of chronic L., fever, sweating, exhaustion, bone pain and an increase in general weakness, anemia, bleeding, etc.

Treatment of acute leukemia, as well as exacerbations of chronic leukemia, is carried out in hospitals (preferably specialized hematological) under the control of blood and bone marrow tests. Combinations of cytostatic agents are used with steroid hormones. In some cases, x-ray therapy, blood transfusions, restorative, antianemic agents, multivitamins are prescribed; to prevent and combat infectious complications- antibiotics. During the period of remission, patients with acute and chronic L. are given maintenance treatment under dispensary observation in specialized hematological departments of the polyclinic. According to the existing situation in the USSR, all patients with L. receive free of charge all the medicines shown to them.

myeloid sarcoma

myeloid sarcoma(obsolete titles " chloroma», « chloroleukemia», « granulocytic sarcoma», « granulosarcoma", :744 " extramedullary myeloid tissue/tumor”), is a solid malignant tumor, consisting of immature white blood cell cells of the bone marrow, the so-called myeloblasts, similar to those that cause acute myeloid leukemia. . In other words, myeloid sarcoma ("chloroma", "granulocytoma") is one of the extramedullary (that is, extra-medullary) manifestations of acute myeloid leukemia. That is, it is a collection of leukemic cells, characteristic of acute myeloid leukemia, somewhere outside the bone marrow and blood.

Historical information

The disease now known as myeloid sarcoma was first described by the British physician A. Burns in 1811. . However, the term chloroma” in relation to this disease was first used only in 1853. The term is derived from the Greek χλωροΣ (chloros, chloros), meaning "green", "pale green", as these tumors are often green or pale green in color due to the presence of myeloperoxidase in them. The close relationship between "chloroma" and acute myeloid leukemia was first discovered in 1902 by Warthin and Doc. However, since up to 30% of these tumors may be white, grey, pink or brown instead of the "classic" green or greenish, and in order to more accurately classify these tumors histologically, Rappaport in 1967 suggested that they be called not by color, but by cell type - the term "granulocytic sarcoma". Since then, the term has become practically synonymous with the obsolete term "chloroma". But, since the cells that make up this tumor are still not mature granulocytes, but blast cells, and, in addition, may not belong to granulocytic, but, for example, monocytic (in acute monocytic leukemia), erythroid, etc. germ hematopoiesis, in accordance with the form of AML according to FAB, in recent years, instead of the term "granulocytic sarcoma", the more scientifically correct term "myeloid sarcoma" has been used.

Skin leukemids, a term describing the infiltration of the skin by leukemic cells with the formation of specific infiltrate nodules, they are also called "cutaneous myeloid sarcoma" (formerly "cutaneous granulocytic sarcoma").
« meningoleukemia" or " meningeal leukemia”, a term describing the invasion of leukemic cells into the subarachnoid space and the involvement of the meninges in the leukemic process, is usually considered separately from myeloid sarcoma (“chloroma”). However, those very rare cases in which a solid tumor of leukemic cells arises in the CNS may nevertheless, by definition, be called CNS myeloid sarcoma.

Frequency and typical clinical manifestations

For acute leukemia

Myeloid sarcomas are a rare disease. The exact frequency of their occurrence is unknown, but they are rarely observed even by hematologists who specialize in the treatment of acute myeloid leukemia.

Myeloid sarcomas may be somewhat more common in patients with the following disease features:

Class M2 according to FAB, that is, Acute myeloid leukemia with maturation;
Patients whose leukemic cells have certain specific cytogenetic abnormalities, such as t(8;21) or inv(16);
Patients whose myeloblasts express CD13 or CD14 T-cell surface antigens
Patients with a high number of blast cells in the blood or a high level of LDH, that is, with a large total tumor mass.

However, even in patients with the above risk factors or a combination of them, myeloid sarcoma is a rare complication of AML.

Sometimes myeloid sarcoma can develop as the first (and for the time being the only) manifestation of recurrence after seemingly successful treatment of acute myeloid leukemia. In line with the clinical behavior of myeloid sarcomas, which always are systemic disease from the very beginning (the concept of "metastasis" does not apply to them), all these cases should be considered and treated as early signs of systemic recurrence of AML, and not as a localized process. Thus, in one review of 24 patients who, after seemingly successful treatment of AML, developed relapses in the form of isolated myeloid sarcomas, it was shown that the average time from the onset of myeloid sarcoma to ascertaining a clear bone marrow recurrence was only 7 months (range - from 1 up to 19 months). And this review was published in 1994, long before the invention of modern molecular techniques that can demonstrate the presence of a bone marrow "molecular" recurrence much earlier than it becomes obvious histologically.

With myelodysplastic and myeloproliferative syndromes, including chronic leukemias

Myeloid sarcomas may occur in patients diagnosed with myelodysplastic syndrome or myeloproliferative syndrome, such as chronic myeloid leukemia, polycythemia vera, essential thrombocytosis, or myelofibrosis. The detection of myeloid sarcoma of any localization in a patient with such a diagnosis is considered de facto evidence that these precancerous or low-grade chronic diseases have transformed into acute myeloid leukemia, requiring immediate adequate treatment. For example, the appearance of myeloid sarcoma in a patient with chronic myeloid leukemia is sufficient evidence that this patient's CML has passed into the "blast crisis" phase. At the same time, the presence of other signs, such as bone marrow blastosis or blastosis in the blood, is not necessary to state the fact of a blast crisis.

Primary myeloid sarcoma

In very rare cases, myeloid sarcoma can occur in a patient without simultaneously meeting the criteria for a diagnosis of acute myeloid leukemia (by bone marrow and blood), myelodysplastic or myeloproliferative syndrome (including chronic myeloid leukemia) and without a previous history of suffering from these diseases. This condition is known as "primary myeloid sarcoma". Diagnosis in these cases can be particularly difficult. In almost all cases of primary myeloid sarcoma, classic, systemic ("bone marrow") acute myeloid leukemia soon develops. The median time from diagnosis of "primary myeloid sarcoma" to the development of overt acute myeloid leukemia is 7 months (range, 1 to 25 months). Therefore, the detection of primary myeloid sarcoma should be considered as an early initial manifestation of acute myeloid leukemia, and not as a localized process, and, accordingly, serve as the basis for the diagnosis of "acute myeloid leukemia" of the corresponding histological form and the appointment of treatment corresponding to the histological form of AML, risk group, cytogenetics and tumor immunophenotype. In particular, if a myeloid sarcoma consisting of promyelocytes is detected (AML type M3 according to FAB, acute promyelocytic leukemia), then treatment should correspond to AML M3 and include not only and not so much chemotherapy, but, above all, the use of all-trans retinoic acid ( ATRA) and arsenic trioxide.

Location and symptoms

Myeloid sarcoma can occur in almost any organ or tissue. However, the most common localizations of the process are the skin (a condition known as "skin leukemids", English leukemia cutis) and gums. Skin involvement in a leukemic process usually appears as pale, sometimes purple or greenish, painless, raised plaques or nodules that are infiltrated by leukemic cells (myeloblasts) on biopsy. Cutaneous leukemids must be distinguished from the so-called "Sweet's syndrome", in which the skin is infiltrated by healthy (non-cancerous) mature neutrophils, which is a reactive paraneoplastic process. Involvement of the gums in the leukemic process results in the characteristic appearance of pale, swollen, hyperplastic, sometimes painful gums that bleed easily when brushing teeth or other minor trauma.

However, with the advent of modern diagnostic techniques such as immunophenotyping and immunohistochemistry, the diagnosis of myeloid sarcoma today can be made much more reliably than before, with less delay in establishing the correct diagnosis and with fewer initial diagnostic errors (misdiagnosis). Thus, Travek et al described the successful use of a commercially available panel of anti-myeloperoxidase monoclonal antibodies, CD68, CD43, and CD20 surface antigens for tissue immunohistochemical staining in order to accurately and correctly diagnose myeloid sarcomas and distinguish them from lymphomas. Today, in order to diagnose and differentiate myeloid sarcomas and lymphomas, immunohistochemical staining with monoclonal antibodies to CD33 and CD117 antigens is mainly used. The increasing availability and increasingly accurate and correct use of flow cytometry has also contributed to improving the early and correct diagnosis of these tumors.

predictive value

Experts disagree on the prognostic value of the presence of myeloid sarcomas in patients with acute myeloid leukemia. In general, it is generally accepted that the presence of myeloid sarcomas means a worse prognosis, with poorer response to therapy, less chance of remission, and worse overall and disease-free survival. However, other experts believe that the mere presence of myeloid sarcomas is associated with other unfavorable biological markers of the tumor, such as the expression of adhesion molecules, T-cell antigens, unfavorable cytogenetic abnormalities, a large tumor mass (high LDH level in the blood or high blast leukocytosis) and therefore the presence of myeloid sarcomas does not carry any additional prognostic information and is not an independent prognostic factor.

Treatment

As described above, myeloid sarcomas should always be considered as another manifestation of a systemic disease - acute myeloid leukemia, and not as an isolated local phenomenon, and therefore should be treated systemically according to the protocols intended for the treatment of acute myeloid leukemia. Accordingly, in a patient with both primary myeloid sarcoma and newly diagnosed acute myeloid leukemia, systemic chemotherapy according to protocols designed for the treatment of acute myeloid leukemia (such as 7+3, ADE, FLAG, etc.) should be used as first line therapy. Given the less favorable, on average, prognosis in patients with myeloid sarcoma compared with patients with AML without extramedullary manifestations, it may make sense to use more aggressive induction and consolidation chemotherapy regimens (for example, ADE or HDAC instead of "7 + 3") and early - in the first remission - high-dose chemotherapy and allogeneic transplantation of hematopoietic stem cells. Topical treatment is generally not indicated and is not necessary, as myeloid sarcomas tend to be quite sensitive to standard systemic antileukemic chemotherapy. In addition, local treatment (surgery or radiation therapy) is associated with the risk of complications (for example, in the case of surgery - infections and bleeding) and postponing the start of chemotherapy, which is dangerous in AML with its rapid progression. An exception is cases when the anatomical localization of myeloid sarcoma threatens the functioning of one or another vital organ (for example, causes compression of the spinal cord with dysfunction of the pelvic organs or the threat of rupture of the spleen, or intestinal obstruction). In this case, emergency surgery or radiation therapy to the affected area may be indicated in parallel with the earliest possible start of intensive antileukemic chemotherapy. Also, local radiation therapy or surgery can be a palliative measure for those who cannot receive chemotherapy of any kind (which is rare - there are alternative regimens for the elderly, debilitated) or who refuse it.

Patients treated for acute myeloid leukemia who relapse after treatment with isolated myeloid sarcoma should be treated in the same way as patients with systemic relapse (i.e., line II and III chemotherapy, allogeneic bone marrow transplantation). However, as with any relapse of acute myeloid leukemia, the prognosis is usually poor, especially if this is not the first relapse (the greater the number of relapses already experienced, the more difficult it is to achieve remission with chemotherapy, the shorter the remission, the more aggressive the tumor and the higher its resistance to chemotherapy).