Varieties of the disease. Classification of chondrosarcomas Causes of disease development. Clinical picture of the pathology Degree of malignancy of the pathology. Possible complications of tumor development Diagnosis of pathology in Israel Treatment of pathology in Israel
Malignant neoplasms developing in the human skeleton are no less dangerous types of cancer that have a decisive impact on human life and health, significantly worsening the patient’s quality of life. Chondrosarcoma is considered one of the most common skeletal cancers.
. The pathology is quite common in medical practice, accounting for approximately 10-15% of cases of the total number of cancers of the bones and other segments of the musculoskeletal system.
The most commonly affected areas are the pelvic bones, shoulder girdle, ribs, femurs and humerus. In extremely rare cases, the disease is found in the hands, affecting the fingers and radius bones. Chondrosarcoma
occurs much more often in middle-aged and older patients, however, pathology often develops in patients of other age categories, from early childhood to elderly patients. The disease is most common among the male population, occurring 1.5-2 times more often in men than in the fair sex.
What's happened
This type of cancer is a malignant tumor that comes from cartilage tissue. This may be a tumor degenerated from a benign formation or a tumor that develops on bones that do not undergo changes. It is the second most common disease among skeletal cancers.
The age at which this disease usually appears is forty to sixty years. Moreover, men suffer from this type of cancer more often than women.
One of the signs of this disease is swelling of the skin in the area of the cancer site and increasing pain. There may also be an increase in the venous network over the malignant tumor, a slight increase in temperature, and limited movement in the joint.
Chondrosarcoma most often requires surgical intervention, and the tumor is removed along with adjacent tissues.
Causes of pathology
It is not known for certain what exactly provokes the development of the oncological process, but there are a number of factors that are prerequisites:
- Frequent bone injuries - fractures, joint dislocations lead to the integrity of bone and cartilage tissue being disrupted. This, in turn, affects metabolic processes and also triggers natural regeneration processes, which can stimulate the active growth of cartilage cells.
- Autoimmune processes in the body that disrupt the synthesis of cartilage cells, causing them to divide chaotically.
- Taking certain medications that can affect the metabolic processes of the musculoskeletal system.
- Prolonged exposure to radiation or other types of exposure.
Frequent bone fractures can cause the appearance of chondrosarcoma.
Also, the tumor process can develop against the background of progressive primary diseases, such as:
- enchondroma and ecchondroma, which are benign neoplasms;
- osteochondromatosis;
- Paget's disease;
- chondromyxoid fibroma.
Most often, the disease is of a primary nature, the etiology of which is unknown. Secondary forms of chondrosarcoma are a consequence of progressive diseases of the musculoskeletal system.
Classification
Origin
According to its origin, chondrosarcoma is of two types:
- Primary;
- Secondary.
Primary
This type of chondrosarcoma is detected in 90% of cases and is divided into two more subtypes:
- Periosteal (this subtype of chondrosarcoma grows outside the bone surface);
- Central (this subtype of chondrosarcoma forms inside the bone).
Secondary
It is detected in 10% of cases and is formed from the following types of tumors:
- Chondroblastomas;
- Chondromas;
- Fibromas of chondromyxoid nature;
- Exostoses of an osteochondral nature;
- Tumors in Ollier-Maffucci disease.
Structural features
If you look at the structure of chondrosarcoma, the following subtypes are distinguished:
- Undifferentiated;
- Anaplastic;
- Clear cell;
- Typical.
Malignancy grade
Based on this criterion, the results of histological studies are taken into account, on the basis of which the degree of malignancy of chondrosarcoma is determined:
- I degree of malignancy: absence of mitotic figures, a small number of multinucleated cells, dense small-nucleated chondrocytes are present in the chondroid tissue;
- II degree of malignancy: there are cellular accumulations along the lobar periphery, an increase in nuclei is observed, there are zones of destruction and some mitotic figures, the intercellular substance is thus myxoid in nature;
- III degree of malignancy: also a myxoid intercellular substance with cells arranged in groups and cords, a large number of irregularly shaped cells, there are a large number of multinucleated and enlarged cells, there is mitosis and extensive areas of destruction.
Ewing's sarcoma
The degree of malignancy of this type of cancer directly determines how quickly metastases will form during the course of the disease, and whether relapse is possible if the pathological tumor is removed.
Clinical manifestations
They depend on a number of related factors that need to be recognized as carefully as possible.
- The extent of the spinal lesion.
- The level of destruction to which the spinal cord has been subjected.
- A structural element that has been affected.
- The place of formation is directly in the spinal tissue or paravertebral area.
- Where is the sarcoma located - on the posterior, anterior or lateral surface of the spinal canal.
- How quickly do sarcoma cells develop?
- Is there extra space in the spinal cord canal and what is its size?
- What is the general health and physical condition of the patient.
Symptoms
Symptoms and development of the disease depend on the type of chondrosarcoma.
Over the course of many years, the disease develops with highly differentiated chondrosarcoma. At first, there is discomfort in the affected area, then a pain syndrome gradually appears, which begins to progress, intensifies at night and does not disappear after moments of rest. The tumor in this type of cancer can be quite large and can be clearly identified by palpating the sore spot. The veins above the tumor may be enlarged, the local temperature may rise, and movement in the adjacent joint may be limited.
A more rapid course of the disease is observed with poorly differentiated chondrosarcoma. Symptoms usually appear in the first months of the disease. Pain syndrome appears, intensifies at night and does not go away after moments of rest. It grows quickly, and the tumor grows rapidly. There is a local increase in temperature, dilation of the veins above the tumor, and movements in the adjacent joint become limited. This form of chondrosarcoma can often recur some time after surgery.
If chondrosarcoma is located in the pelvic bones, then the tumor produces pressure on nearby tissues and organs. Because of this, pain occurs along the posterior femoral surface and in the buttock, urination may be difficult, and the lower limb may swell.
The occurrence of distant metastases is possible in poorly differentiated chondrosarcoma. As a rule, the affected areas are the lymph nodes, lungs, brain and liver.
A special type of satellite metastases appears in periosteal chondrosarcoma. These metastases have no connection with the main tumor and are located in healthy tissues or in the reactive zone.
Complications
If the cancer process progresses, complications such as:
- The appearance of metastases and their transportation by the lymphatic system to distant tissues and organs, which reduces life expectancy tens of times.
- A high rate of relapse if treatment did not produce the desired results and the oncological process could not be stopped.
- The need for constant diagnosis and preventive treatment - chondrosarcoma can return at any moment of a person’s life, therefore, after successful treatment, it will be necessary to constantly monitor the health status through radiography and preventive treatment.
When limbs are amputated, disability develops. A person becomes incapacitated, but continues to live and adapt to new conditions. Death is the most dangerous complication of chondrosarcoma, but with early diagnosis there is every chance of a favorable outcome.
Diagnosis
First, the patient is sent to an oncologist, who examines the affected area and prescribes various studies: radiography, trepanobiopsy, and, as additional studies, MRI, CT and osteoscintigraphy.
Radiography
If the patient has central chondrosarcoma, then the image will show an irregularly shaped lesion, its edges will be unclear, and due to small lesions the tumor may take on a speckled appearance.
If the patient has peripheral chondrosarcoma, then on the x-ray a lumpy tumor will be visible, its edges will be unclear, and it will be located on the outer bone surface. There will be uneven compaction between the tumor and normal bone. The tumor may have a mottled appearance, and the cortical bone layer will be uneven.
Trephine biopsy of bone
With this research method, biomaterial is taken from different areas of the formation. A histological examination is performed, as a result of which the degree of malignancy of the cancer will be determined.
Chondrosarcoma
Various tests and investigations may be needed to diagnose chondrosarcoma. Usually, an x-ray of the painful area of the bone is enough to identify this growth. Other diagnostic cancer screening procedures are often performed to check the extent to which cancer has spread to other places in the body. X-ray signs of malignancy of a tumor of cartilage tissue are a rapid increase in destruction, destruction of the cortical layer, which can be observed in a small area, and tumor exit into the soft tissue adjacent to the bone. Periosteal layers in the form of a visor or spicules are not clearly expressed.
With osteochondral exostoses, the first signs of malignancy are pain and rapid growth of exostosis, discrepancy between the clinically determined size of the tumor and the radiologically detected one. Malignization occurs due to the cartilaginous part, so the palpable tumor can be larger than that visible on the x-ray. The X-ray picture is characterized by unclear boundaries of osteochondral exostosis, blurred contours, and foci of destruction in the bone base of the exostosis.
Based on location, chondrosarcomas are divided into central and peripheral. Central ones arise inside the bone, peripheral ones - on the surface of the bone.
In case of central chondrosarcomas, intraosseous areas of destruction are identified radiographically, usually having an irregular round shape and blurred outlines. In these cases, also against the background of the tumor, spotty inclusions of calcifications are usually detected. When the tumor is localized in long tubular bones, the affected area may appear fusiformly thickened.
Peripheral chondrosarcoma appears as a tuberous formation, contrastingly revealed against the background of soft tissue adjacent to the bone. In the place where the tumor is directly adjacent to the bone, erosion of the surface of the cortical layer or uneven sclerotic compaction is noted. In the thickness of the tumor formation, foci of calcification are identified, creating a mottling picture. Heavy calcifications are often detected along the edges of the tumor. The outline of the tumor is usually unclear and uneven.
MRI (magnetic resonance imaging). This diagnostic technique is similar to a CT scan, but instead of X-rays, it uses magnetism to create cross-sectional images of the patient's body.
Penetrating needle biopsy . For this study, a small tumor sample is taken from the affected bone. A medical professional will then examine the sample under a microscope to determine the exact type of tumor.
Open biopsy . This type of biopsy is rarely used because penetrating needle biopsy is much faster and easier. In an open biopsy, a piece of bone is removed during a minor operation while the patient is under general anesthesia. This oncological examination will be needed if a penetrating biopsy cannot be done for some reason or does not give a clear result.
Osteoscintigraphy . This is a more sensitive test than plain x-rays and shows any abnormal bone tissue more clearly. For this test, a small amount of a "soft" radioactive substance is injected into a vein. The bone abnormality absorbs more radiation than healthy bone, and these areas are highlighted and indicated by the scanner as “hot spots.”
CT (computed tomography). A CT scan captures a series of X-rays to create a three-dimensional image of the inside of the body. The scan is painless and takes 10 to 30 minutes. A CT scan uses little radiation and is unlikely to cause harm to the body.
Chest X-ray . In some people with chondrosarcomas, the cancer may spread to the lungs. A chest x-ray is usually done to check whether this is true or not.
Dedifferentiated chondrosarcoma (syn. chondrosarcoma with an additional mesenchymal component) is a highly malignant tumor arising from a less malignant cartilaginous neoplasm. The adjective “dedifferentiated” is poorly chosen. This is not about “dedifferentiation” of mature cartilage cells, but about disruption of the maturation of mitotically active stem cells. The frequency of findings of “dedifferentiated” chondrosarcoma is 1% of the total number of chondrosarcomas. The gender and age of the patients do not matter. On average, for 6-10 months, patients complain of pain, swelling and paresthesia at the site of the tumor. Localization: “dedifferentiated” chondrosarcoma most often affects the pelvic bones, the proximal end of the femur and humerus, the leafy ends of the femur and ribs. An X-ray examination reveals either an intraosseous lesion of a lytic type with unclear contours, or a non-calcified neoplasm located in the soft tissues, but associated with a cartilaginous tumor. Very often there is destruction of the cortical layer. Noteworthy is the sharp contrast between the chondroil and “dedifferentiated” parts of the tumor. “Dedifferentiated” chondrosarcoma almost always arises from central chondrosarcoma, which, in turn, from enchondrosarcoma. A slightly different histogenetic sequence occurs if “dedifferentiated” chondrosarcoma develops from peripheral chonrosarcoma: the predecessor of the latter is either a solitary osteochondroma or one of the tumors in hereditary osteochondromatosis.
Externally, “dedifferentiated” chondrosarcoma looks like a tumor of mature hyaline cartilage, but on the cut surface there are reddish or brown areas that have a softer consistency than cartilage. It is these areas that correspond to the “dedifferentiated” component of the tumor, and they can have different volumes. Regardless of the type of surgical intervention, the prognosis for “dedifferentiated” chondrosarcoma is extremely unfavorable. Up to 90% of patients die within 2 years, and most of them die within 1 year after diagnosis. In this case, extensive metastasis of the tumor occurs (mainly to the lungs), and the microscopic structure of the metastases repeats the non-cartilaginous component of the tumor. There are also metastases of undifferentiated chondrosarcoma to other bones and internal organs.
Under the microscope, an “undifferentiated” chondrosarcoma consists of a chondroid, the same as in a well-differentiated chondrosarcoma, and a noncartilaginous component, clearly demarcated from each other. The noncartilaginous component can be represented by tissue from fibrosarcoma, malignant fibrous histiocytoma, osteo- or rhabdomyosarcoma, and occasionally angiosarcoma. The most common picture is of malignant fibrous histiocytoma, in which intricately intertwined bundles of atypical fibroblasts alternate with giant multinucleated cells. The fibrous-histiocytic nature of the cells is confirmed by immunohistochemical study. Some tumors show bundles of striated muscle fibers and cytokeratin deposits.
When differentially diagnosing “dedifferentiated” chondrosarcoma, tumors such as highly malignant chondrosarcoma, which contains many spindle cells, mesenchymal chondrosarcoma, chondroblastic osteosarcoma, malignant fibrous histioiitoma and fibrosarcoma should be kept in mind.
Treatment
The main treatment for chondrosarcoma is surgery, since this type of cancer is generally resistant to radiation therapy and chemotherapy.
If the patient has malignant chondrosarcoma of grade I or II, the tumor is surgically removed. First, it is excised with tissue from the reactive zone and a supply of nearby healthy tissue. Next, a resection of part of the bone or its complete removal can be performed (this depends on the location and size of the tumor). Further endoprosthesis replacement is possible.
With grade III malignant sarcoma, disarticulation or amputation of the limb where the chondrosarcoma is located is possible.
If tumor removal is not possible, chemotherapy or radiation therapy is done. These methods may slow the growth of chondrosarcoma.
Chodrosarcoma of the femur
In many cases, the localization of a cancerous tumor is diagnosed in the femoral part of the body, as mentioned above.
In addition to all the symptoms by which this disease can be recognized, with hodrosarcoma of the femur, severe pain in the hip area is also added.
They arise due to compression of the sciatic nerve by the tumor.
Diagnosis and treatment of chondrosarcoma of the femur are based on general methods used for other cancer sites.