Symptoms of Chordoma

A chordoma is a rare type of bone cancer that develops from remnants of the notochord, a structure in the developing embryo that eventually helps to form the spinal column. Chordomas typically occur near the top or bottom of the spine, such as the skull base, the sacrum (triangular bone at the base of the spine), or the coccyx (tailbone). Symptoms can vary depending on the location and size of the tumor.  

One of the most common symptoms of chordoma is pain that is either confined to one location (localized) or spreads to a different location (radiating). Localized pain tends to be dull and constant, whereas radiating pain is typically sharp and can be felt to travel down the limbs. This can happen as the tumor grows and begins to press on nerves that carry sensory information to the brain, resulting in the perception of pain. Since these symptoms can be mistaken for other conditions such as a herniated disc or osteoarthritis, consult your physician who can determine if further testing is needed.

If the chordoma has grown big enough and is close to the skin, you may be able to feel a swelling or lump. A chordoma lump may feel firm or hard to the touch. In the case of a tailbone chordoma, you may be able to feel a lump in the lowermost part of your back. In many cases, the tumor is more deep-seated, or far from the body surface, and cannot be felt through the skin. This is especially true for skull base chordomas. In such cases, a chordoma may be detected through imaging studies such as computed tomography (CT) or magnetic resonance imaging (MRI) scans.

Sacral chordomas appear at the triangular bone located at the base of the spine, just above the tailbone. Pain that localizes to the lower back or radiates down the legs can occur and mimic other medical conditions. However, sacral chordomas can also be associated with the following additional symptoms.

• Weakness in the legs and difficulty walking
• Problems with bowel movements, such as incontinence or constipation
• Urinary problems, such as incontinence, difficulty urinating, and chronic urinary tract infections
• Sexual dysfunction, including difficulty with arousal, achieving or maintaining an erection, and ejaculation

Skull base chordomas, such as clival chordomas, are often difficult to diagnose because symptoms usually do not appear until the condition has progressed. These chordomas can cause a variety of symptoms based on their location and size:

• Headaches that can be severe and persistent
• Double vision, loss of vision, or other visual disturbances
• Hearing loss or ringing in the ears (tinnitus)
• Speech difficulty and swallowing problems
• Facial asymmetry
• Nasal blockage, nasal bleeding, and occasionally clear watery discharge from the nose
• Dizziness and vertigo
• Difficulty breathing or hoarseness of voice

If you or someone you know is experiencing any of these symptoms, see a doctor promptly. Your doctor may recommend imaging studies such as CT or MRI scans to confirm the diagnosis.

When a chordoma has reached an advanced stage, the initial symptoms such as pain may intensify. The pain can be constant and difficult to manage with over-the-counter pain medications. In such cases, your healthcare team will provide you with prescription medications to effectively manage and control your pain. However, as the tumor continues to grow and spread, everyday activities such as walking, standing, or sitting for long periods can become increasingly challenging.

Compression of structures around the tumor may lead to difficulties breathing, swallowing, and controlling bowel or bladder movements. Fatigue and general weakness from both the tumor and its treatments can make it difficult to enjoy activities and even the company of family and friends. Aggressive tumors can also spread to other parts of the body, leading to the formation of new tumors (metastases).

Despite advances in cancer therapeutics, the treatment options for end-stage chordoma are currently limited. While surgery, radiation therapy, and sometimes chemotherapy are available, a tumor that has spread to multiple locations will be highly unlikely to be able to be removed completely. When this happens, some patients may prefer an approach to care that prioritizes comfort and quality of life over curative treatment (palliative care).

Opting for comfort care can allow individuals to focus on making the most of the time they have left, rather than pursuing a cure that may no longer be feasible. Alternatively, some patients may choose to explore clinical trial opportunities.

These trials can provide access to experimental treatments and potential breakthroughs in chordoma management. However, the risks associated with such treatments are being investigated during the trial and may not be completely known.

Deciding on the next steps after a diagnosis of an advanced chordoma can be a challenging and complex process. However, you are not alone. Your healthcare team will discuss the available options and considerations for each potential plan. Together, you will determine the path that best fits your health goals and preferences. 

• Chordomas can cause a range of symptoms based the size and location of the tumor.
• Pain is the most obvious symptom: Skull base chordomas tend to cause headaches, while sacral or spinal chordomas can cause back pain with sharp radiating pain to the legs.
• Bowel and bladder problems may occur depending on the location of the chordoma.
• End-stage chordoma symptoms are mainly managed with palliative care, though clinical trials may also be explored.

• National Cancer Institute: Chordoma
• Chordoma Foundation
• NIH Genetic & Rare Diseases (GARD) Center: Chordoma
• Cancer Net
• American Cancer Society