Sarcoma: symptoms, treatment — bone, soft tissue
Sarcoma is a group of tumors, the development of which are the different types of connective tissue, and to date, described for about one hundred varieties of them. Sarcoma, though malignant, but cancer to name it is impossible, since the term «cancer» is used for epithelial tumors (carcinoma). If the cancer has certain characteristics and differences that depend on its localization, that is, cancer of the stomach will vary according to the structure, form, growth, disease from, say, lung cancer or uterine sarcoma that has no such reference to a particular authority, and especially it will depend on the type of fabric that gave her a start.
Probably not in the body of a place where you could grow a sarcoma, for those or other variants of connective tissue there is in the internal organs and in the skin and in the brain. For example, vessels that can be a source of these tumors, do not exist except in the cornea, and muscle and fibrous connective tissue form the basis of the walls of hollow organs and skeleton of many parenchymal.
All types of connective tissue in the process of fetal development originate from the middle germ layer – the mesoderm, and the bulk of them are represented by muscles, bones, fat, blood vessels, therefore, not surprising that among the sarcomas predominate tumors of bone, muscle, cardiovascular origin.
According to statistics, the share of sarcomas account for only about 1% of all malignant tumors, but with such rarity they are very dangerous and are in second place on mortality after epithelial tumors (cancers). Feature of connective tissue diseases is their rapid, sometimes explosive character of growth when the tumor rapidly increases in size, starts to metastasize in a short period of time, leaving no chance for a cure.
Among patients with a particular form of sarcoma about a third were young people (under 30 years), children, adolescents. Children in particular can often be detected sarcoma of bone and soft tissue. This fact explain the rapid increase in cell mass in the growing organism, so the risk of mutations or violations of division at some stage of growth of the skeleton, or muscle is higher than in adults, whose body has already been formed. The highest number of cases of tumors of connective tissue origin accounted for the second decade of life, and the advantage of cases are boys tall.
Certain types of sarcomas can be found in people of Mature and elderly more than children. They are sarcoma of the uterus, originating from the myometrium, stromal tumors of the stomach wall or esophagus, Kaposi’s sarcoma and other vascular tumors.
Causes and types of sarcomas
The reasons for the growth of connective tissue tumors varied, but differ little from those causing epithelial neoplasias (cancers). Risk factors and adverse effects that contribute to carcinogenesis, fully apply to sarcomas. So, to the appearance of sarcomas lead:
- Precancerous lesions and the presence of benign tumors that can become malignant
- Genetic predisposition, when certain forms of tumors seen in close relatives, as well as genetic syndromes and genetic abnormalities accompanied by mutations in the chromosomes.
- External carcinogens and harmful habits such as ultraviolet light, radiation, Smoking, industrial emissions, household chemicals, etc.
- Professional harmful factors when working in the chemical industry, at high temperatures, etc.
- Carrier of viral infection (herpes, for example).
- Mechanical injury – a burn, wound, fracture, injury, long-term presence of shrapnel or other foreign bodies in the soft tissues or bones.
- Rapid growth in puberty and fluctuations in hormonal levels, which is especially clearly seen for the osteogenic sarcoma in children and adolescents.
- Reduction of immune forces of an organism, characteristic for the elderly, HIV-infected patients.
Under adverse conditions the connective tissue cells begin to proliferate, and the appearance in them of mutation triggers uncontrolled division and rapid growth of the tumor.
Sarcoma look often resembles fish meat has a soft texture and grows without distinct borders, spreading in the periphery in healthy tissue. Often tumors can be detected necrosis (necrosis), hemorrhage, deposition of lime, mucus, which gives it a rather motley appearance. Unlike cancer, sarcoma prefers hematogenous metastasis, when tumor cells are carried throughout the body by the bloodstream.
The different characteristics of sarcoma cells, indicating its malignant potential, are reflected in the determination of the degree of tumor differentiation:
Poorly differentiated sarcomas behave the most aggressive, grow rapidly, metastasize early and actively, and tend greatly to injure the surrounding healthy tissue. The cells are so far «left» from their «progenitor», often to determine the source of neoplasia even with a microscope it is simply impossible. In such cases, resort to the determination of specific proteins characteristic of a particular type of connective tissue. If this does not help, the tumor is considered to be undifferentiated, that is, the source to define it and failed. This is probably one of the worst options for the patient, because these sarcomas are extremely ill for any possible treatment. For these tumors, surgical removal is the main, although sometimes used radiation or chemotherapy, which nizkoeffektivnyj most sarcomas are not sensitive.
Moderately and vysokomehanizirovannoe connective tissue tumor composed of cells resembling more healthy, which gave them beginning. They respond better to therapy and are more sensitive to chemotherapy, the use of which has allowed in recent decades to improve the prognosis and survival of patients. Vysokomehanizirovannoe sarcomas are more likely to give local recurrences after treatment, and metastasize less frequently, while the poorly differentiated tumors early enough may manifest itself in distant metastases.
In addition to the degree of differentiation, it is important to determine the source of the tumor. Depending on origin, there are:
- Osteosarcoma – tumors of the bone tissue;
- Rhabdomyosarcoma and leiomyosarcoma originating from striated and smooth muscles;
- Angiosarcoma – tumors of vascular origin;
- Malignant fibrous histiocytoma growing from fibrous connective tissue;
- Synovial sarcoma;
- Liposarcoma, the source of which is adipose tissue;
- Neurogenic sarcoma found in the nervous tissue;
- Gastrointestinal stromal tumors.
In this list only the most common forms of neoplasms of connective tissue, but there are undifferentiated options, neoplasia of a mixed structure (carcinosarcoma), which simultaneously detected structures of sarcomas and epithelial tumors (cancer), and mesenchyma growing from embryonic tissue and characterized by extreme malignancy.
Stages of sarcoma
Staging of sarcomas is a difficult task, especially as the approaches may vary depending on the lesion. American oncologists have been proposed staging system for sarcomas, based on their size and degree of differentiation of tumor cells:
So, stage I, correspond to vysokomehanizirovannoe neoplasia II – moderately differentiated III – poorly differentiated sarcoma. In the presence of metastases tumor automatically refers to the fourth stage of the disease.
Addition to the first three stages of the index A or b indicates the size of the tumor up to 5 cm And more than 5 cm in diameter – Century For the fourth stage, the index And mean local metastasis, In the presence of distant metastases.
Sarcomas of various localization have certain particularities, not only the causes, but in the course and prognosis of the disease, so the approach in each case is individual, and the treatment extends not only to oncologists but also physicians of other specialties (neurosurgeons, gynecologists).
Soft tissue sarcoma: rhabdomyosarcoma, liposarcoma, leiomyosarcoma
The largest group of connective-tissue malignant tumor is soft tissue sarcoma. This notion of a collective, including tumors of muscle, fat, fibrous connective tissue, ligaments and tendons, etc. To the soft tissues are not considered neuroglia and supporting framework of internal organs.
Soft tissue sarcoma, are diagnosed more often in patients who fiftieth anniversary, and the preferential localization – the lower extremities that affects almost half of the cases. The tumor may be located on the thigh and lower leg in the region of large joints. Should not remain without attention of specialists swelling in the leg, especially if that was preceded by serious trauma hard surgery with blood circulation and lymphatic drainage that took place even many years ago.
Generally, sarcomas arise without clear apparent reason, although the role of carcinogens, immunosuppressive therapy, irradiation and receiving cytotoxic drugs, HIV infection, congenital immune system disorders not denied. The tumor is able to achieve quite large sizes in General satisfactory condition of the patient, and the absence of pain does not exclude a malignant nature of the tumor. In addition, the sarcoma may be located not only on the lower extremities, but also in the retroperitoneal space, the abdominal or chest cavity, when to notice it in the form of puffiness and did not.
The most frequent varieties-tissue sarcomas are liposarcoma (adipose tissue of the extremities, retroperitoneal space, in the back, neck), leiomyosarcoma and rhabdomyosarcoma originating from smooth or striated muscle, synovial sarcoma of the membranes of the joints, vascular tumors.
Liposarcoma is a malignant tumor of adipose tissue, favorite places growth is considered to be the thigh, inguinal region, retroperitoneum, buttocks. Many have heard about lipoma – a benign «fatty» tumors, which are very common in various parts of the body, but do not be afraid, if the lipoma is or was once deleted, because it is one of the most benign human tumor and its transformation to a liposarcoma is considered impossible. The disease with liposarcoma depends on the degree of differentiation that determine the rate of growth and metastasis of neoplasia.
Sarcoma of the uterus, leiomyosarcoma, rhabdomyosarcoma
Leiomyosarcoma (smooth muscle tumor) can be detected in the myometrium, the wall of a hollow organ (stomach, for example), and rhabdomyosarcoma are more common in the thigh, where usually well developed striated muscle tissue. Tumors of muscular origin is quite malignant, prone to the rapid growth and spread throughout the body, so early diagnosis is very important for them.
Sarcoma of the uterus occurs in women of childbearing age and Mature, and it predispose to fluctuations in hormonal levels, inflammation, intrauterine intervention.
It is believed that benign fibroids so frequently diagnosed in women, not prone to malignant even with multiple character growth, but fast-growing and large components with a diameter of 7 cm or more should always be a reason to exclude sarcoma.
The presence in the tumor areas of necrosis (necrosis), formation of slime or lime deposits does not necessarily indicate the malignancy of education, and especially often these changes can be detected when a large myoma nodes is quite benign structure, which is disturbed blood supply because of the large size. On the other hand, the mere size and, especially, the rapid increase of the tumor in a short period of time needs to push the idea about sarcoma. No specific clinical signs or symptoms to differentiate leiomyosarcoma from benign fibroids, so only removal of the tumor and subsequent thorough histological examination of different sections of the tumors should resolve the problem.
Rhabdomyosarcoma is quite aggressive, prone to rapid growth, capable of reaching considerable size in a few months or even weeks, accompanied by massive damage to surrounding tissue and early distant metastasis to lungs and liver. Certain types of tumors «choose» is not only predominant localization in the body, but also the age of the patient. So, children often reveals a neoplasm of embryonic type in the head and neck in adolescence – alveolar rhabdomyosarcoma, which is extremely aggressive, and in adults is polymorphic tumor, the survival of which does not exceed 25%.
Still not clearly articulated the causes of this tumor, but seen its relationship with some hereditary syndromes. The influence of external harmful factors in the case of rhabdomyosarcoma are virtually eliminated, so even the x-ray irradiation of the pregnant woman or injury cannot be attributed to the cause of rhabdomyosarcoma.
Rhabdomyosarcoma, which is limited to one area of the body, is small and has not metastasized can be treated and cured, and tumors of this species growing in the head or neck have a better prognosis as compared to those in the extremities, back, abdomen or retroperitoneal space.
Synovial sarcoma of soft tissue
Synovial sarcoma develops from the synovial membrane of the joints in children and adults. The tumor is malignant, it is sufficient, therefore, to survive, according to statistics, not more than half of the patients. Is synovial sarcoma most often in the joints of the hands and feet and is a solid education in places of fastening of the tendons. In some cases such a tumor may be accompanied by pain syndrome, and the deposition of lime in it causes a characteristic radiological picture.
Malignant fibrous histiocytoma develops from fibrous connective tissue and affects mainly adults above 40. There is evidence that the incidence of this tumor has increased significantly in recent time, reaching, according to some researchers, the first place among all soft-tissue tumors. In General, the share of fibrous histiocytoma accounts for about 40% of soft tissue sarcomas. To detect this tumor at the tibia, hip, and slightly less often on the trunk or in the retroperitoneal space. Located in the deep muscle layers of the limb or trunk, a long time neoplasia is growing slowly, but at some point manifests itself spasmodic rapid growth and can be detected.
Kaposi’s sarcoma is a tumor of vascular origin, previously rarely encountered primarily in older men on the lower extremities in the form of single nodes. This sarcoma has a favourable prognosis and even tend to regress. The spread of HIV across the globe were forced to take another look at Kaposi’s sarcoma, which is currently considered one of the main indicators formed of immunodeficiency. In this case, the swelling becomes generalized, affecting the skin, soft tissue and internal organs, and may become the cause of death of an AIDS patient.
Video: soft tissue sarcoma — lecture on the morphology and diagnosis
Sarcoma of bone, osteosarcoma, Ewing’s sarcoma
Malignant tumors of bone often found in children. I think the most common osteogenic sarcoma and Ewing’s sarcoma, ranked second.
Ewing’s sarcoma was first described early last century as a bone tumor, but there are also nekotrye types. Ill mostly children, the peak incidence occurs in 10 to 15 years. The tumor occurs in long tubular bones legs, arms (humerus, tibia, femur), much less frequently affected bones are the pelvis and thorax.
Ewing’s sarcoma manifests itself by swelling and pain due to damage of the periosteum or of pathological fracture in the area of growth neoplasia. Similar symptoms can be and when injury or inflammation, but treatment with antibiotics and anti-inflammatory drugs will lead to improvement, whereas in tumors these measures will have a positive effect.
At the time of diagnosis of Ewing’s sarcoma about one third of patients already have metastases in the lungs or other bones, and the tumor has grown deep into the bone and reached the marrow. The prognosis in such cases is very serious, and treatment may be associated with a large number of complications.
Video: Ewing’s sarcoma — the program «Live healthy!»
Osteogenic sarcoma (osteosarcoma)
Osteogenic sarcoma is one of the most malignant human tumors, and source development becomes the bone, which can produce themselves malignant cells. The disease affects children and young people up to 30 years and among patients more than men. Favorite localization of osteogenic sarcoma is the femur, which is affected in half of all cases. Often the tumor is located in the knee joint, but the patella is affected only rarely.
In addition to the bones of the lower limbs, osteogenic sarcoma can be diagnosed in the lower jaw, where there is a fairly clear clinical picture of very rapid growth, destruction of bone tissue and the introduction of the tumour into surrounding structures of the face lead to a noticeable cosmetic defect, the violation of the processes of mastication, the ability to speak clearly. Lung metastases can be detected already in the initial stages of the disease, and progressive cachexia (wasting syndrome) will inevitably lead to the death of patients in a short time.
The only external factor that may lead to osteogenic sarcoma, consider radiation, and from the time of exposure prior to the growth of the tumor may take up to several decades. Trauma, which also bind the tumor soon becomes the reason for going to the doctor and x-rays than the root cause of the disease.
Rapid growth, early and rapid metastasis, make a tumor is extremely dangerous, and more recently, the survival rate was low. Modern methods of chemotherapy, radiation, and rational, to the extent possible, sparing surgery can achieve good results more than half of the patients, with 5-year survival rate has reached 70-90%.
The most difficult to diagnose and very rare is sarcoma of the spine, which can have the structure of osteosarcoma, chondrosarcoma, Ewing’s sarcoma, etc., we Should distinguish between primary sarcomas in the structures of the spine and metastases, which may be accompanied by other tumors, including epithelial origin (cancer of the prostate or the thyroid, for example).
Video: osteogenic sarcoma of the program «Live healthy!»
Sarcoma of the brain
By itself, the neural tissue can be a source of connective-tissue tumors, but the meninges and blood vessels are quite capable to give rise to sarcoma. Such a defeat occurs no more than 2% of cases of malignant brain tumors and tumor-affected people of any age.
Sarcoma of the brain may be located within the body and outside it. The first endowed with all the characteristics of a malignant tumor – blurring boundaries, rapid growth spread to the surrounding tissue, metastasis. Extracerebral sarcoma originating from the meninges, can have a detail view with more or less clear boundaries, but still invasive growth in ingrowth and damage to adjacent tissues is characteristic for them.
Signs of sarcoma of the brain are reduced to focal neurological symptoms (paralysis, impaired vision, speech etc.) and cerebral symptoms due to increased intracranial pressure. Specific manifestations of nervous tissue sarcoma no. Tumors of this type are very malignant in their course, are apt to recur after removal and to metastasize. Over the next two years after treatment survive only 30-40% of patients.
Sarcoma of the internal organs, cm, angiosarcoma
Among parenchymal organs, most common sarcoma of the lung. This vital organ not only takes the «blow» of the metastatic process in other malignant tumors (like cancer), but also can become the center of many other diseases. Since light is widely represented by different types of connective tissue, and Kaposi here – the notion of a collective, including angiosarcoma, cm, tumors of the cartilage of the bronchial tree, smooth and striated musculature, and even lymph nodes.
Sarcoma of the liver or kidneys are diagnosed only rarely, but they can occur from a vascular tract or interlayer connective tissue inside of themselves. As connective tissue tumors at other sites, these tumors tend to grow rapidly in size, growing in the surrounding tissues and structures, and also spread through the blood or lymph.
Symptoms of sarcoma and diagnostic methods
Symptoms of sarcomas are entirely determined by their localization and the degree of malignancy, but there are common signs found in most cases of these tumors (pain, for example).
The symptoms of sarcoma can be grouped into several groups:
To make a diagnosis of sarcoma, in addition to the characteristic clinical signs of, data examination and clinical tests are carried out:
- Radiography in primary tumors of the bones and the exclusion of metastatic lung lesions.
- Ultrasound to detect tumors in the soft tissues and internal organs.
- CT scan and MRI.
- Radioisotope scanning.
- Morphological study of tumor tissue – cytological, histological, immunohistochemical.
Treatment of sarcomas
Treatment of malignant connective tissue tumors is subject to the basic principles of cancer care and consists in the use of radiotherapy and radical removal of the tumor.
Surgical treatment of sarcoma is considered to be the primary and most effective way to get rid of neoplasia. Operation is quite traumatic and often require amputation of the limb, so plastic and prosthetics play an important role for the subsequent rehabilitation of the patient.
In recent years, increasingly began to apply gentle methods of tumor removal with preservation of functional activity of the limb. As practice shows, in the case of the possibility of such operations (early stage of sarcoma), the prognosis is even better than total deletion of the hands or feet.
In addition to surgery, sarcoma treated with chemotherapy and radiation, which can significantly improve the efficiency of surgical treatment of sarcoma. These methods can be applied both before surgery and after it.
Children have a particularly high likelihood of complications in the form of other tumors after administration of chemotherapy or radiotherapy, there are leukemias due to the negative impact on the bone marrow, so in some cases there is a need for the transplant.
In the case of sarcoma, and the patient and his relatives should be prepared for the fact that the treatment will be long and difficult, so these patients are very important the help and support of loved ones.
The prognosis of sarcoma is always serious and depends on the specific type of tumor, stage, location, differentiation grade of tumor cells, their sensitivity to chemotherapy. With early detection of the disease in some cases it is possible to achieve a fairly high survival rates (70-80%), and some types of sarcoma do not leave a chance to the patient even under intensive treatment.
The tumor pathology does not spare not only people but also animals, and to lovers of Pets advised of the possibility of growth of tumors in the wards, particularly dogs and cats. Veterinarians are often faced with cancer in animals, which among connective tissue tumors often have to diagnose sarcoma of the breast, lymphosarcoma, osteosarcoma (especially after injuries of the limbs) and the so-called venereal sarcoma, which is found exclusively in dogs and has interesting features described in the veterinary literature. Our younger brothers, as man, is better as fast as possible to get on reception to the expert, otherwise the tumor can in a short time deprive them of life.