Chondrosarcoma

21.03.2024

CHONDROSARCOMA DIAGNOSIS AND TREATMENT

Chondrosarcoma is a rare type of malignant tumor arising from bone and cartilage tissues. Chondrosarcomas usually occur in bones or cartilage and can occur in different parts of the body. Significant characteristics of such tumors may include a tendency to grow slowly and symptoms such as pain and swelling. Chondrosarcomas have an important place in diagnosis and treatment because the degree of malignancy of these tumors may vary. While low-grade chondrosarcomas generally have a better prognosis, high-grade chondrosarcomas can be more aggressive and make treatment more difficult. Therefore, pathological examination is very important to correctly classify the tumor and determine the treatment plan. Although chondrosarcomas are a rare type of tumor, successful results can be achieved with early diagnosis and appropriate treatment. Treatment options may include surgery, radiotherapy and chemotherapy. An important aspect of chondrosarcomas is that early diagnosis and correct treatment can significantly affect the patient's quality of life and survival. Therefore, when symptoms of such tumors appear, it is important to consult a physician quickly.

WHAT ARE THE RISK FACTORS?

Although the cause of chondrosarcomas is not fully understood, some risk factors may increase the risk of developing these tumors.

Age: Chondrosarcomas usually occur in young or middle-aged adults, but they can occur at any age throughout life.

Radiation Exposure: The risk of chondrosarcoma may increase, especially in people who have been exposed to the area treated with radiotherapy for a long time. Therefore, people who have received radiotherapy in the past may be at risk.

Genetic Factors: Although familial cases of chondrosarcoma appear to be rare, family history or genetic factors may increase the risk in some cases.

Paget's Disease: Paget's disease is a disease that affects bone tissue, and people with this disease may have an increased risk of chondrosarcoma.

Li-Fraumeni Syndrome: Rare genetic syndromes, such as Li-Fraumeni syndrome, may increase the risk of developing several types of cancer and may also increase the risk of chondrosarcoma.

Chondrosarcoma History: People who have previously been diagnosed with chondrosarcoma may have an increased risk of developing new chondrosarcomas in the same or different sites.

Exposure to Radiation: Exposure to high doses of radiation, especially during childhood or teenage years, may increase the risk of chondrosarcoma.

Remember that these risk factors are just one factor, and there may be more than one factor that affects a person's likelihood of developing chondrosarcoma. If in any doubt or if you have risk factors, it is important to consult a physician and have regular health checks. Early diagnosis may increase the chances of treating chondrosarcoma and other tumors.

Image 1: Chondrosarcoma is a rare tumor that develops from bone and cartilage.

HOW IT OCCURS?

Although the mechanisms of development of chondrosarcomas are still not fully understood, several factors are thought to contribute to the formation of this rare type of cancer. Genetic mutations, radiation exposure, and some genetic syndromes may play a role in the formation of chondrosarcomas. Mutations and chromosomal abnormalities, especially in the p53 gene, may be effective in the development of these tumors. Additionally, the risk of chondrosarcoma may be increased in people receiving radiotherapy. However, more research is needed to understand exactly how chondrosarcomas develop, and the mechanisms of development of this disease are still being studied in depth.

WHAT ARE THE SYMPTOMS?

Chondrosarcoma findings can vary depending on the size of the tumor, where it develops, and how aggressive it is. Additionally, such tumors sometimes have no obvious symptoms and can be detected incidentally during random X-rays or imaging tests.

The following symptoms and signs may usually be observed:

Pain: Chondrosarcomas often cause pain. This pain may be felt constantly or intermittently in the affected area and may increase over time.

Swelling: Chondrosarcomas may form a swelling or mass in the affected area. This swelling may be firm or painful to the touch.

Fractures and Weakness: Because chondrosarcomas can develop in bones, affected bones may experience problems such as weakness, easy fractures, or involuntary bone breaking.

Movement Restriction: When chondrosarcomas develop in or near joints, there may be limited movement or limited movement in the affected joint.

Other Symptoms: Chondrosarcomas can develop in different parts of the body, so symptoms may vary depending on the location of the tumor. For example, if chondrosarcomas develop in the spine area, symptoms such as back pain and spinal curvature may occur.

HOW IS IT DIAGNOSED?

Chondrosarcoma is usually diagnosed using a number of clinical and imaging methods. The first step is a careful evaluation of the patient's medical history and symptoms. Then, the size and location of the tumor is determined by physical examination. Chondrosarcomas can be confused with benign cartilage tumors. The most important step in diagnosis is imaging studies. Detailed images are obtained to determine the location and characteristics of the tumor using methods such as x-ray, magnetic resonance imaging (MRI), computed tomography (CT) and positron emission tomography (PET). A biopsy is performed to confirm the diagnosis, during which tissue samples are taken from the tumor and pathological examination confirms that the tumor is malignant and chondrosarcoma. Pathological examination also determines how aggressive the tumor is, which can affect treatment options. Diagnosis and classification of chondrosarcoma usually requires a multidisciplinary approach and is evaluated together by an oncologist, orthopedist, radiologist and pathologist.

WHAT ARE THE PATHOLOGICAL TYPES?

Chondrosarcoma is a type of tumor that can be divided into different pathological types according to its histopathological features. Different pathological types of chondrosarcoma can be:

Conventional Chondrosarcoma: This is the most common type of chondrosarcoma and involves atypical (deviating from normal) growth of cartilage cells.

Dedifferentiated Chondrosarcoma: This type has more complex histological features than conventional chondrosarcoma and may be more malignant.

Cartilage Cell Chondrosarcoma: This type of chondrosarcoma has more prominent cartilage cells and may generally have a better prognosis.

Clear Cell Chondrosarcoma: Tumor cells of this type are characterized by a clear cytoplasm and are a rarer subtype.

These pathological types are defined based on the morphological and histological features of the tumor and may affect treatment options and prognosis. Treatment and prognosis may vary depending on the histological type of the tumor, its size, location and the general health of the patient. Therefore, when chondrosarcoma is diagnosed, it is important for pathologists to determine the pathological type of the tumor and convey this information to the treatment team. Evaluation of receptors is of great importance in the treatment of chondrosarcoma in order to understand the biological behavior of the tumor and optimize treatment options. Some critical receptors and biomarkers in this context are: Ki-67 is a marker that indicates the rate at which tumor cells divide, and its high levels may indicate a tendency for the tumor to grow rapidly. PD-L1 (Programmed Death-Ligand 1) is a critical marker in assessing response to immunotherapy; high positivity may indicate that the tumor may be amenable to immunotherapy. These receptors and markers are evaluated by pathological examination and molecular analysis to determine the biological characteristics of the tumor and potential treatment options. Treatment planning is customized based on these biomarkers and receptors and is of paramount importance to maximize the patient's response to treatment.

Image 2: Risk factors such as genetic mutations, radiation exposure, and Paget disease can lead to the development of chondrosarcoma.

HOW IS TUMOR STAGING DONE?

Chondrosarcoma tumor staging is a procedure used to determine the size, spread, and areas of the body of the tumor. Staging of chondrosarcoma is done according to cancer staging systems developed by the American Joint Cartilage and Cartilage Tissue Consortium or Cancer Staging Committee (AJCC). Basically chondrosarcoma staging is based on the following factors:

Tumor Size (T): Tumor size is an important factor in staging chondrosarcoma. T stage refers to a category that indicates the size of the tumor and how far it has spread.

Lymph Node Involvement (N): Because chondrosarcoma is unlikely to spread to the lymph nodes, stage N is usually classified as "N0" (no lymph node involvement).

Distant Metastases (M): Chondrosarcoma is also unlikely to spread to other organs, so stage M is usually classified as "M0" (no distant metastases).

By combining this information, the tumor stage is determined. For example, a staging of T2N0M0 indicates that the tumor is large but has not spread to lymph nodes or distant organs. Tumor staging is important to determine the patient's treatment plan and make prognosis predictions. Higher stage tumors may often require more aggressive treatment. Chondrosarcoma tumor staging is important to improve patient quality of life and survival and should be performed carefully by a multidisciplinary healthcare team.

HOW IS TREATMENT DONE ACCORDING TO STAGES?

Chondrosarcoma treatment is customized depending on the stage and type of tumor, the patient's age and general health.

Stage I and Stage II Chondrosarcoma: In these early stages, treatment usually begins with surgical intervention. The aim is to completely remove the tumor. If it is possible to remove the tumor and maintain healthy function after this procedure, surgical treatment alone may be sufficient. However, in cases where the tumor is large or complex, radiotherapy or chemotherapy may be used as additional treatment.

Stage III Chondrosarcoma: In advanced stages, the spread of the tumor may be wider and it may be difficult to completely remove it with surgery. In these cases, radiotherapy may be used to shrink or control the tumor. Chemotherapy and smart drugs can also be used to slow the growth of the tumor or stop its spread. In advanced chondrosarcomas, surgery is often used to relieve symptoms or control the tumor.

Metastatic Chondrosarcoma: In cases where chondrosarcoma has metastasized to distant organs, treatment becomes more complex. Systemic treatments such as chemotherapy, targeted drugs or immunotherapy may be used. In this case, the aim is to control the growth of the tumor and relieve symptoms in cases where surgery is not suitable to remove the entire tumor or there are many metastases.

A multidisciplinary healthcare team is necessary to determine and customize chondrosarcoma treatment. This team may include oncologists, orthopedists, radiation oncologists, pathologists, and other specialists. The treatment plan is determined according to the patient's medical condition and the characteristics of the tumor. The goal of treatment is to control the tumor, relieve symptoms, prevent tumor recurrence, and improve the patient's quality of life.

Image 3: During the diagnosis process of chondrosarcoma, plain radiography, MRI and biopsy are performed.

WHAT ARE THE SYSTEMIC DRUGS USED IN TREATMENT?

Treatment options such as chemotherapies, hormonal drugs, smart drug treatments and immunotherapies used in the treatment of chondrosarcoma may vary depending on the type of tumor, its stage and the specific condition of the patient.

Chemotherapy drugs: Doxorubicin, İfosfamide, Etoposid, Cisplatin

Smart Drug Therapies: “Smart drugs” used to treat chondrosarcoma are often designed to target specific biological properties or molecular targets of the tumor. These drugs target tumor cells through specific molecular mechanisms and cause less damage than healthy cells. Some smart drug treatments used for chondrosarcoma may include:

Tyrosine Kinase Inhibitors: These drugs inhibit tyrosine kinase enzymes that control the growth and spread of tumor cells. Example: imatinib, regorafenib.

Angiogenesis Inhibitors: They prevent the tumor from forming blood vessels by targeting factors such as VEGF (Vascular Endothelial Growth Factor). Such drugs can restrict the nutrition and growth of the tumor. Example: pazopanib.

Immunotherapies: Immunotherapy is not yet a standard option in the treatment of chondrosarcoma, but is being investigated in clinical trials.

Chondrosarcoma treatment is customized based on the characteristics of the tumor and the medical condition of the patient. Therefore, which treatment option to use is information that a healthcare professional must evaluate and share with the patient. Factors such as the histological type, stage and genetic characteristics of the tumor should be taken into account before any treatment plan is created.

HOW SHOULD FOLLOW-UP BE CARRIED OUT AFTER RECOVERY?

Follow-up after chondrosarcoma treatment is vital to monitor the patient's health and assess the risk of tumor recurrence. This follow-up plan is personalized depending on the patient's tumor stage, response to treatment, and general health status. Usually, the follow-up program includes regular physical exams, imaging tests, blood tests, and biopsies when necessary. This approach allows for early diagnosis of tumor recurrence or another health problem. Follow-up is important to improve the patient's quality of life and optimize post-treatment outcomes, and it is important that patients comply with the follow-up plans determined by the physician.