Chordoma is a rare type of cancer that occurs in the bones of the skull base, spine, and sacrum. It is a slow-growing tumor that can invade nearby tissues and cause compression of the spinal cord or nerve roots. Chordoma is classified into several types based on the location and characteristics of the tumor. In this article, we will discuss the different types of chordoma and their features in detail.
- Conventional chordoma:
Conventional chordoma is the most common type of chordoma, accounting for approximately 95% of all cases. It usually occurs in the sacrum or the base of the skull. Conventional chordomas are slow-growing tumors that arise from remnants of the notochord, a structure that develops in the early embryo and disappears as the spine forms. They typically appear as lobulated or irregular masses on imaging studies, and can cause pain, weakness, or numbness in the affected area.
- Chondroid chordoma:
Chondroid chordoma is a variant of conventional chordoma that has a more cartilaginous appearance. It contains more matrix than other types of chordoma and can resemble a benign cartilaginous tumor. Chondroid chordoma usually occurs in the sacrum or the base of the skull, and it tends to have a more favorable prognosis than conventional chordoma.
- Dedifferentiated chordoma:
Dedifferentiated chordoma is a rare type of chordoma that arises from a conventional chordoma and has features of a higher-grade tumor. It tends to be more aggressive than conventional chordoma and can metastasize to other parts of the body. Dedifferentiated chordoma usually appears as a large, irregular mass on imaging studies and may be difficult to distinguish from other types of cancer.
- Chordoma with chondrosarcomatous differentiation:
Chordoma with chondrosarcomatous differentiation is a type of chordoma that has features of both chordoma and chondrosarcoma, a type of bone cancer. It usually occurs in the spine and can cause compression of the spinal cord or nerve roots. Chordoma with chondrosarcomatous differentiation can be difficult to diagnose, as it may resemble other types of bone tumors.
- Poorly differentiated chordoma:
Poorly differentiated chordoma is a rare and aggressive type of chordoma that has features of a high-grade tumor. It tends to grow quickly and can metastasize to other parts of the body. Poorly differentiated chordoma usually appears as a large, irregular mass on imaging studies and can be difficult to distinguish from other types of cancer.
- Extraskeletal chordoma:
Extraskeletal chordoma is a rare type of chordoma that arises from soft tissue rather than bone. It can occur in various parts of the body, including the head and neck, trunk, and extremities. Extraskeletal chordoma tends to be less aggressive than other types of chordoma, but it can still cause local invasion and recurrence.
Causes
Causes of chordoma that you should know about:
- Genetic mutations: Some people may be born with a genetic mutation that increases their risk of developing chordoma.
- Exposure to radiation: Prolonged exposure to ionizing radiation can increase the risk of developing chordoma.
- Family history: People with a family history of chordoma are at a higher risk of developing the disease.
- Age: Chordoma is more common in people over the age of 30, with the highest incidence in people in their 50s and 60s.
- Gender: Men are slightly more likely to develop chordoma than women.
- Race: Chordoma is more common in people of European descent.
- Location: Chordomas tend to occur in the bones of the skull and spine.
- Physical trauma: People who have experienced physical trauma, such as a fracture or dislocation of the spine, may be at a higher risk of developing chordoma.
- Smoking: There is some evidence to suggest that smoking may increase the risk of developing chordoma.
- Alcohol consumption: Heavy alcohol consumption may also increase the risk of developing chordoma.
- Obesity: Obesity has been linked to an increased risk of chordoma.
- Hormonal imbalances: Hormonal imbalances, such as those that occur in people with acromegaly, may increase the risk of developing chordoma.
- Paget’s disease: People with Paget’s disease, a condition that causes the bones to become weak and misshapen, may be at a higher risk of developing chordoma.
- Neurofibromatosis: People with neurofibromatosis, a genetic disorder that causes tumors to grow on nerves, may be at a higher risk of developing chordoma.
- Ollier disease: People with Ollier disease, a rare disorder that causes benign cartilage tumors to grow in bones, may be at a higher risk of developing chordoma.
- Maffucci syndrome: People with Maffucci syndrome, a rare genetic disorder that causes benign tumors to grow on bones and skin, may be at a higher risk of developing chordoma.
- Li-Fraumeni syndrome: People with Li-Fraumeni syndrome, a genetic disorder that increases the risk of developing several types of cancer, including chordoma, may be at a higher risk of developing chordoma.
- Gardner syndrome: People with Gardner syndrome, a genetic disorder that causes the growth of non-cancerous tumors in the colon and rectum, may be at a higher risk of developing chordoma.
- Multiple endocrine neoplasia type 2: People with multiple endocrine neoplasia type 2, a genetic disorder that causes tumors to grow in the endocrine glands, may be at a higher risk of developing chordoma.
- Von Hippel-Lindau syndrome: People with Von Hippel-Lindau syndrome, a genetic disorder that causes tumors to grow in various parts of the body, may be at a higher risk of developing chordoma.
Symptoms
Common symptoms of chordoma and discuss their causes and management.
- Pain
Pain is one of the most common symptoms of chordoma. It can be localized to the area of the tumor or may radiate to other parts of the body. Pain can be mild or severe, depending on the size and location of the tumor.
- Stiffness
Stiffness is another symptom that can be caused by chordoma. It can affect the neck or back, making it difficult to move the head or body. Stiffness can be caused by the tumor pressing on nerves or other tissues.
- Numbness
Numbness is a sensation of loss of feeling or tingling in the affected area. It can be caused by the compression of nerves by the tumor.
- Weakness
Weakness can be caused by the compression of nerves or muscles by the tumor. It can affect the ability to move the affected area or perform certain tasks.
- Difficulty walking
Chordoma in the spine can cause difficulty in walking. The tumor can press on the spinal cord, causing problems with balance and coordination.
- Difficulty swallowing
Chordoma in the skull base can cause difficulty in swallowing. The tumor can compress the structures in the throat, making it difficult to eat or drink.
- Hoarseness
Hoarseness is a change in the voice, making it sound rough or scratchy. It can be caused by the tumor pressing on the nerves that control the vocal cords.
- Fatigue
Fatigue is a feeling of exhaustion or lack of energy. It can be caused by the body’s response to the tumor or by the treatment for chordoma.
- Loss of appetite
Loss of appetite is a common symptom of chordoma. It can be caused by the tumor or by the treatment for chordoma.
- Weight loss
Weight loss can be caused by the loss of appetite or by the body’s response to the tumor.
- Headaches
Chordoma in the skull base can cause headaches. The tumor can press on the brain or the blood vessels in the head, causing pain.
- Dizziness
Dizziness can be caused by the tumor pressing on the nerves that control balance and coordination.
- Vision problems
Chordoma in the skull base can cause vision problems. The tumor can compress the structures in the eye or the optic nerve, causing blurred vision or double vision.
- Hearing problems
Chordoma in the skull base can cause hearing problems. The tumor can compress the structures in the ear or the auditory nerve, causing hearing loss or ringing in the ears.
- Nasal obstruction
Chordoma in the skull base can cause nasal obstruction. The tumor can compress the structures in the nose, making it difficult to breathe.
- Nosebleeds
Nosebleeds can be caused by the tumor pressing on the blood vessels in the nose.
- Facial swelling
Chordoma in the skull base can cause facial swelling. The tumor can compress the structures in the face, causing swelling and deformity.
- Seizures
Seizures can be caused by the tumor pressing on the brain or the blood vessels in the head.
- Weakness or numbness in the arms or legs
Chordoma in the spine can cause weakness or numbness in the arms or legs. The tumor can press on the nerves that control movement and sensation.
Diagnosis
Most common diagnosis and tests for chordoma in simple, SEO-optimized language.
- Physical Exam: The first step in diagnosing chordoma is a physical exam. During the exam, your doctor will ask about your symptoms and medical history, and may perform a physical examination to check for any signs of chordoma.
- Imaging Tests: Imaging tests are used to create detailed images of the bones and tissues inside your body. Common imaging tests used to diagnose chordoma include X-rays, CT scans, MRI scans, and PET scans.
- Biopsy: A biopsy is a procedure that involves taking a small sample of tissue from the affected area for examination under a microscope. This can help your doctor determine if the tissue is cancerous or not.
- Blood Tests: Blood tests can be used to check for certain markers that may indicate the presence of chordoma. However, blood tests are not always reliable and are usually used in combination with other diagnostic tests.
- Bone Scan: A bone scan is a type of imaging test that uses a radioactive tracer to create images of the bones. This test can help your doctor determine if the cancer has spread to other areas of the body.
- Lumbar Puncture: A lumbar puncture, also known as a spinal tap, involves taking a sample of cerebrospinal fluid from the spine. This test can help your doctor determine if the cancer has spread to the brain or spinal cord.
- CT-Guided Biopsy: A CT-guided biopsy is a type of biopsy that is performed using CT imaging to guide the needle to the affected area. This can help your doctor take a more accurate sample of tissue for examination.
- MRI-Guided Biopsy: An MRI-guided biopsy is similar to a CT-guided biopsy, but uses MRI imaging to guide the needle to the affected area.
- Endoscopy: An endoscopy is a procedure that involves inserting a thin, flexible tube with a camera on the end into the affected area. This can help your doctor examine the area and take a tissue sample if needed.
- Fine Needle Aspiration: Fine needle aspiration involves inserting a thin needle into the affected area to remove a small sample of tissue for examination.
- Ultrasound: Ultrasound uses sound waves to create images of the inside of the body. It can be used to examine the affected area and determine if there are any abnormalities.
- Molecular Testing: Molecular testing involves analyzing the DNA of the cancer cells to determine the best treatment options.
- Immunohistochemistry: Immunohistochemistry is a type of test that uses antibodies to identify specific proteins in the tissue sample. This can help your doctor determine if the tissue is cancerous or not.
- Cytogenetic Analysis: Cytogenetic analysis involves examining the chromosomes of the cancer cells to determine if there are any abnormalities. This can help your doctor determine the best treatment options.
- Flow Cytometry: Flow cytometry is a type of test that can be used to analyze the properties of the cancer cells. This can help your doctor determine the best treatment options.
- Electromyography (EMG): Electromyography is a type of test that measures the electrical activity of the muscles and nerves. It can be used to determine if the cancer has spread to the nerves.
- Spinal Angiography: Spinal angiography is a type of test that uses dye and X-rays to create images of the blood vessels in the spinal cord.
Treatment
Different treatments for chordoma in detail.
- Surgery – Surgery is the primary treatment option for chordomas. The goal of surgery is to remove as much of the tumor as possible while preserving neurological function. The type of surgery depends on the location and size of the tumor. Some chordomas are accessible through minimally invasive techniques, while others require more extensive procedures. Surgery is often followed by radiation therapy to kill any remaining cancer cells.
- Radiation – Therapy Radiation therapy uses high-energy rays to kill cancer cells. It is often used after surgery to kill any remaining cancer cells. Radiation therapy can also be used to shrink the tumor before surgery to make it easier to remove. There are different types of radiation therapy, including external beam radiation therapy, proton therapy, and brachytherapy.
- Proton – Therapy Proton therapy is a type of radiation therapy that uses protons instead of x-rays to deliver radiation to the tumor. Protons have less effect on healthy tissue than x-rays, making it a good option for tumors that are close to critical structures.
- Brachytherapy – Brachytherapy is a type of radiation therapy that involves placing radioactive material directly into the tumor. This allows for a high dose of radiation to be delivered to the tumor while minimizing exposure to healthy tissue.
- Chemotherapy – Chemotherapy uses drugs to kill cancer cells. It is not typically used as the primary treatment for chordomas but may be used in combination with other treatments. Chemotherapy can be given orally or through an IV.
- Immunotherapy – Immunotherapy is a type of cancer treatment that uses the body’s immune system to fight cancer. It works by stimulating the immune system to attack cancer cells. Immunotherapy is not yet a standard treatment for chordoma, but clinical trials are underway to test its effectiveness.
- Targeted Therapy – Targeted therapy is a type of cancer treatment that targets specific molecules that are involved in the growth and spread of cancer cells. It is not yet a standard treatment for chordoma, but clinical trials are underway to test its effectiveness.
- Cryotherapy – Cryotherapy is a treatment that uses extreme cold to destroy cancer cells. It is not a standard treatment for chordoma but may be used in certain situations.
- Laser Ablation – Laser ablation is a minimally invasive treatment that uses a laser to destroy cancer cells. It is not a standard treatment for chordoma but may be used in certain situations.
- Radiofrequency – Ablation Radiofrequency ablation is a minimally invasive treatment that uses heat to destroy cancer cells. It is not a standard treatment for chordoma but may be used in certain situations.
- Photodynamic – Therapy Photodynamic therapy is a treatment that uses a special light and a photosensitizing agent to destroy cancer cells. It is not a standard treatment for chordoma but may be used in certain situations.
- Stereotactic Radiosurgery – Stereotactic radiosurgery is a non-invasive treatment that delivers high doses of radiation to the tumor. It is not a standard treatment for chordoma but may be used in certain situations.
- Radioimmunotherapy – Radioimmunotherapy is a type of cancer treatment that combines radiation therapy and immunotherapy. It is not yet a standard treatment for chordoma, but clinical trials are underway to test its effectiveness.
Medications
Drugs treatments for chordoma, including their mechanisms of action, side effects, and efficacy.
- Imatinib: Imatinib is a tyrosine kinase inhibitor that blocks the activity of a protein called KIT, which is often overexpressed in chordoma. Imatinib can slow down the growth of chordoma cells and reduce tumor size. The most common side effects of imatinib are fatigue, nausea, and diarrhea.
- Pazopanib: Pazopanib is another tyrosine kinase inhibitor that targets multiple receptors involved in angiogenesis (the formation of new blood vessels). By inhibiting angiogenesis, pazopanib can prevent tumor growth and metastasis. The most common side effects of pazopanib are high blood pressure, diarrhea, and fatigue.
- Sorafenib: Sorafenib is a multikinase inhibitor that targets several pathways involved in tumor growth and proliferation. Sorafenib can induce apoptosis (programmed cell death) in chordoma cells and inhibit angiogenesis. The most common side effects of sorafenib are diarrhea, hand-foot skin reaction, and fatigue.
- Sunitinib: Sunitinib is a tyrosine kinase inhibitor that targets multiple receptors involved in angiogenesis and tumor growth. Sunitinib can induce apoptosis in chordoma cells and inhibit angiogenesis. The most common side effects of sunitinib are fatigue, nausea, and diarrhea.
- Regorafenib: Regorafenib is a multikinase inhibitor that targets several pathways involved in tumor growth and proliferation. Regorafenib can inhibit angiogenesis and induce apoptosis in chordoma cells. The most common side effects of regorafenib are fatigue, hand-foot skin reaction, and diarrhea.
- Cabozantinib: Cabozantinib is a tyrosine kinase inhibitor that targets multiple receptors involved in angiogenesis and tumor growth. Cabozantinib can induce apoptosis in chordoma cells and inhibit angiogenesis. The most common side effects of cabozantinib are diarrhea, fatigue, and nausea.
- Temsirolimus: Temsirolimus is an mTOR inhibitor that blocks a protein called mTOR, which plays a crucial role in cell growth and proliferation. Temsirolimus can induce apoptosis in chordoma cells and reduce tumor size. The most common side effects of temsirolimus are fatigue, rash, and mucositis.
- Everolimus: Everolimus is another mTOR inhibitor that blocks the activity of mTOR. Everolimus can induce apoptosis in chordoma cells and reduce tumor size. The most common side effects of everolimus are fatigue, mouth sores, and diarrhea.
- Bevacizumab: Bevacizumab is a monoclonal antibody that targets vascular endothelial growth factor (VEGF), a protein involved in angiogenesis. By inhibiting angiogenesis, bevacizumab can prevent tumor growth and metastasis. The most common side effects of bevacizumab are high blood pressure, fatigue, and proteinuria.
- Cetuximab: Cetuximab is a monoclonal antibody that targets epidermal growth factor receptor (EGFR), a protein involved in cell growth and proliferation. Cetuximab can induce apoptosis in chordoma cells and inhibit tumor growth.
