What is chondrosarcoma and why is it dangerous?

Chondrosarcoma is a malignant tumor that develops from cartilage tissue. It accounts for more than 20% of all primary bone tumors. Typically, chondrosarcomas are localized in the pelvis or affect long tubular bones (femur, humerus, ribs).

The danger of chondrosarcoma lies in its high aggressiveness. These tumors tend to grow rapidly and invade surrounding tissues.

They can affect joints and periarticular tissues, leading to destruction of structures and limited functionality of the affected area. Chondrosarcomas metastasize quickly. Cancer cells spread from the original pathological focus to distant organs and tissues. This complicates treatment and worsens the prognosis.

Another feature of chondrosarcoma is that this tumor is quite rare. Therefore, its diagnosis and treatment should be carried out by a specialist who has previously encountered a similar disease. MedTour company cooperates with the best oncology clinics and doctors specializing in the treatment of sarcomas. Leave a request on our website to contact the coordinating doctor. The medical coordinator will help you select a medical center and an experienced doctor, as well as resolve all organizational issues.

Causes of chondrosarcoma

Most chondrosarcomas develop sporadically, that is, accidentally. However, some tumors arise as a result of malignant transformation of osteochondromas and endochordomas. About 5% of osteochondromas, both multiple and single forms, turn into chondrosarcoma.

The reasons for the development of the disease are not completely clear, but scientists have identified factors that increase the risk of its occurrence. These include:

• Gender. In men, chondrosarcoma is diagnosed somewhat more often than in women.
• Age. In most cases, malignant tumors of cartilage tissue develop in people over 40 years of age.
• Cancer treatment. The risk of developing chondrosarcoma increases if patients have previously undergone chemotherapy or radiation therapy.

In addition, risk factors include rare bone diseases:

• Paget’s disease. A chronic skeletal disease characterized by abnormally rapid division of bone cells.
• Ollier disease. It is accompanied by the growth of multiple cartilaginous tumors. The disease usually appears before the age of 10 years. The risk of developing chondrosarcoma in patients with Ollier disease is 20-25%.
• Maffucci syndrome. It is characterized by the appearance of multiple cartilaginous tumors, hemangiomas and disruption of bone structure. The probability of developing chondrosarcoma in Maffucci syndrome is 25-30%.

Many patients with Ollier disease and Maffucci syndrome have mutations in the IDH1 or IDH2 genes. Recently, scientists are considering these mutations as targets for targeted therapy for chondrosarcoma.

Symptoms and signs of chondrosarcoma

Deep, dull, aching pain is a common symptom of chondrosarcoma. It usually gets worse at night. According to the Rizzoli Institute, the average time interval from the onset of pain to diagnosis is 19.4 months for grade I and II chondrosarcomas and 15.5 months for grade III chondrosarcomas.

If the tumor is located near a neurovascular bundle, such as in the pelvis, the patient may experience dysfunction of the lumbosacral plexus nerves, as well as the sciatic or femoral nerves.

When chondrosarcoma is located close to a joint, it can limit range of motion and impair joint function. Many patients are also prone to pathological fractures. Clear cell and mesenchymal chondrosarcomas may not manifest themselves for a year or more due to the slow development of the tumor.

Types of chondrosarcoma

Central chondrosarcoma

They account for almost 80-90% of all chondrosarcomas and 20-27% of all primary bone sarcomas. The affected areas may be:

• pelvis and ribs – 45%;
• ilium – 20%;
• femur – 15%;
• humerus – 10%;
• other bones – 10%.

The spine and craniofacial bones are rarely affected.

Dedifferentiated chondrosarcoma

It occurs in 10% of cases. Most often it affects the femur (about a third of all diagnosed tumors). Other sites of localization include the pelvis (20%), humerus (16%), ribs (7%) and scapula (7%).

Clear cell chondrosarcoma

It accounts for less than 5% of all chondrosarcomas. It is localized mainly at the ends of long bones near the epiphyseal growth plate. Like chondroblastomas, these tumors spread to the articular cartilage. The proximal femur is most commonly affected (45%), followed by the proximal humerus.

Mesenchymal chondrosarcoma

It is diagnosed in only 2% of cases. It is localized mainly in the upper and lower jaw, vertebrae, ribs, and pelvic area. The upper and lower extremities are rarely affected.

Chondrosarcoma – metastases

Chondrosarcomas are aggressive tumors prone to local invasion of surrounding tissues and distant metastasis. Neoplasms of this type spread mainly to the lungs, less often to bones located far from the primary focus.

The presence of metastases significantly worsens the prognosis of the disease. Thus, the 5-year survival rate for localized (not spread) pelvic chondrosarcoma is 67.4%; for a metastatic tumor this figure drops to 14.7%.

Timely consultation with a doctor makes it possible to diagnose chondrosarcoma at an early stage, before it has time to spread to other organs and tissues. Leave a request on our website, and the MedTour coordinating doctor will select for you the best clinic and experienced doctor for early diagnosis and treatment of chondrosarcoma.

Stages of chondrosarcoma

Staging of a tumor depends on its location. Chondrosarcomas can be:

• intracompartmental (limited in a confined space), for example, in the bone, joint cavity, muscle group within the fascial sheath;
• extracompartmental, that is, located in interfascial or extrafascial spaces without natural barriers to spread, for example, in the pelvis or axillary region.

Stages of chondrosarcoma according to Enneking system

Diagnosis of chondrosarcoma

Often the tumor is detected on x-ray after a physical examination. It is impossible to distinguish a benign tumor from a malignant one using X-rays. For this purpose, additional tests are prescribed, such as:

• Magnetic resonance imaging;
• CT scan;
• positron emission tomography;
• bone scintigraphy.

To make a final diagnosis, a biopsy is performed – taking a small sample of the tumor for pathohistological examination.

Innovative methods for diagnosing chondrosarcoma

Abnormal tissue samples obtained by biopsy provide the doctor with valuable information about the sarcoma and possible treatments. There are no two tumors that are identical to each other, even if they have the same location. Each of them has its own unique feature. Scientists use these features as targets when creating targeted drugs that are used as part of personalized therapy.

Currently, doctors use the following to treat chondrosarcomas:

• cyclin-dependent kinase inhibitors;
• inhibitors of mTOR (a protein that helps the survival of pathological cells);
• IDH inhibitors (mutations in the IDH1 and IDH2 genes).

However, for therapy to be effective, it is necessary to know exactly whether the selected “target” is present in a particular tumor. For example, mutations in the IDH1 and IDH2 genes are present in approximately 50% of patients with chondrosarcoma. For them, treatment with IDH inhibitors will be successful. For people who do not have such mutations, it will not be effective. Knowing the exact characteristics of the tumor allows the doctor to choose the right therapy.

It is possible to create a molecular profile of the tumor and identify its weak points in special laboratories. Leave a request on the website, and the MedTour coordinating doctor will select for you an affordable medical center for diagnosing chondrosarcoma.

Chondrosarcoma – prognosis, survival rate

The prognosis depends on the stage and degree of the lesion at the time of diagnosis. Tumor location also plays an important role, as tumors located in areas where complete wide resection is possible are associated with a better prognosis. In general, chondrosarcomas of the head and neck have a higher survival rate than tumors located elsewhere. In children and adolescents, chondrosarcoma of the bone has a very good prognosis and is less aggressive than in older patients.

Survival rates for different types of chondrosarcomas

• Regular. The prognosis depends on the histological grade of the tumor. With grade I, the 5-year survival rate of patients is 90%, with grade II – 81%, and with grade III – 29%. The overall 5-year survival rate for conventional chondrosarcomas is 48-60%. Intralesional surgery is not recommended even for stage I lesions, especially in the pelvis, since the local recurrence rate in such cases is 100%.
• Dedifferentiated. It has a high degree of lethality. The one-year survival rate for patients is only 10%. Even with early surgical treatment, most patients develop metastases.
• Clear cell. Although chondrosarcomas of this type are low-grade tumors, they can lead to distant metastases. The relapse rate of the disease is 16%.
• Mesenchymal. The five-year survival rate for mesenchymal chondrosarcoma is less than 50%, and the overall 10-year survival rate is 28%.

Treatment of chondrosarcoma

Radiation therapy and chemotherapy

These methods have a limited role in primary treatment. Doctors mainly use them as adjuvant (postoperative) therapy or as palliative treatment for inoperable tumors. The main indication for the use of radiation and chemotherapy is diffuse metastases.

CyberKnife

CyberKnife or stereotactic radiosurgery is a method of targeted radiation therapy for tumors. The procedure involves creating a three-dimensional model of the tumor and subsequent targeted irradiation. The main advantage of this technique is the precise impact of radiation rays on the pathological focus, in which they do not damage the surrounding healthy tissue.

CyberKnife for chondrosarcomas is used if the tumor is located in an area inaccessible to surgical resection.

Most often these are lesions of the base of the skull. They are quite rare, in only 2% of cases. Typically, chondrosarcomas of the skull base are of low grade and complete resection can be curative. However, they are often considered inoperable due to the complexity of the approach. In this case, the use of CyberKnife may be the optimal solution.

MedTour company cooperates with the best oncology centers that offer treatment using stereotactic surgery. Leave a request on the website, and our coordinating doctor will select a radiation oncologist for you who will help determine whether it is possible to use CyberKnife technology in your case.

Surgery

Surgery is the main treatment for any chondrosarcoma. Complete, wide surgical excision of the tumor is the preferred approach when possible. The success of surgery depends on the stage of the disease: low-grade intracompartmental lesions have a better prognosis after surgical resection with clear margins.

The best clinics for the treatment of chondrosarcoma

MedTour company cooperates with the best medical centers around the world. Currently, the field of oncology is developing rapidly in Turkey. Chondrosarcomas are successfully treated in large multidisciplinary centers, such as University hospital LIV Istinye, Medipol University Hospital, and the Medical Park Hospitals Network. Many patients prefer Korean medicine. One of the leaders in cancer treatment in South Korea is Samsung Medical Center. Spanish doctors at the Teknon Medical Center have achieved impressive success in the fight against malignant tumors. And this is not a complete list of oncology clinics that provide advanced medical care to patients with chondrosarcoma.

Leave a request on the website, and the MedTour coordinator doctor will contact you as soon as possible. The medical coordinator will help you choose the best cancer center in accordance with your wishes and financial capabilities.

The best doctors for the treatment of chondrosarcoma

The MedTour platform contains information about leading specialists in the field of treatment of malignant tumors from around the world. Chondrosarcoma is a rare tumor, so it is important to have an experienced oncologist diagnose and treat it. Leave a request on the website and we will select a highly qualified specialist for you.

Published: 23.11.2023

Updated: 26.11.2023

Medical author, Medical editor:

Olena Kursabaieva

Medical author, Medical editor:

Natalia Segen