You may have gone to your doctor because you were experiencing swelling, pain, and stiffness in a joint in your knee, finger, wrist, or other area of your body. Because these common TGCT symptoms are much like those of a number of joint conditions, including arthritis, it can be surprising to learn that you have this uncommon type of tumor, which is typically diagnosed through computerized tomography (CT) or magnetic resonance imaging (MRI) scans and a tissue biopsy. Now that you have a diagnosis, here are some key questions to ask your doctor to help you take control of your condition.
1. What exactly is a tenosynovial giant cell tumor and how did I get it?
These tumors grow in the soft connective tissue in your joint — the synovium, bursae, and tendon sheaths — according to the National Organization for Rare Disorders (NORD). According to Thomas J. Scharschmidt, MD, a member of the Molecular Biology and Cancer Genetics Program at The Ohio State University Comprehensive Cancer Center in Columbus, Ohio, there are two basic types: diffuse and localized (or nodular). Both can occur in tissues inside or outside the joint, says Dr. Scharschmidt. Localized TGCT is usually present in a specific area of the joint and can often be successfully treated with surgery to remove the tumor. Diffuse TGCT, on the other hand, means the tumor is widespread and can cause damage to the joint and surrounding areas. This type is less likely to be treated successfully with surgery, and the tumor can often grow back after removal. When it comes to TGCT, there are a lot of unknowns, including what causes it and what risk factors may make someone more susceptible to developing the disease. According to NORD, research has shown that chromosomal changes may lead to the formation of tumors, but it’s not yet clear what’s driving those changes that lead to TGCT. Doctors also don’t know why TGCT seems to occur most often in young adults 25 to 40 or why the incidence of the disease seems to be slightly higher in women than in men.
3. Is TGCT the same as a giant cell tumor of bone?
No. A giant cell tumor is an aggressive, benign tumor affecting the bone near a joint, according to Johns Hopkins Medicine, and in rare instances, it can metastasize to the lungs. While the two conditions sound extremely similar, “a giant cell tumor is a completely separate diagnosis than a TGCT,” says Scharschmidt. Knowing this distinction can save you a lot of fear and anxiety about what your future holds with TGCT.
4. Will my pain get worse, and can I expect the tumor to spread to other parts of my body?
Fortunately, once you get a diagnosis and receive the right treatment, your symptoms, including pain, can improve, particularly if you have localized TGCT. In cases where there is a delayed diagnosis and treatment, the joint may become progressively more painful and stiff and lose motion and function over time. While the TGCT-impacted joint itself is at higher risk for complications such as arthritis, there is no known link that predisposes you to inflammatory joint conditions such as rheumatoid arthritis, says Scharschmidt. As for whether it can spread to other areas of the body, Scharschmidt says that while there are isolated case reports of more than one joint being involved, you can typically expect TGCT to remain in the joint. There’s a chance that, if left untreated, the tumor can grow large enough to “extend outside of the joint,” but this does not mean that it’s spreading to other locations.
5. Is there a cure?
Whether your TGCT can be cured may depend on which type you have. If your tumor is localized, it means that it is in a limited portion of the joint and can be surgically removed. For those cases, Scharschmidt estimates that 90 percent are cured through surgery and the tumor does not grow back. Diffuse tumors, which are widespread throughout the joint, are more difficult to remove completely and 2.6 times more likely to grow back compared with localized tumors, according to research published in August 2017 in the journal Acta Orthopaedica. It’s not all bad news though: A study looking at surgical outcomes of 1,192 diffuse-type TGCT patients published in April 2019 in the journal The Lancet Oncology found that 40 percent of patients with diffuse tumors remained cured after 10 years. The study also revealed that surgery improved pain for 59 percent of patients and decreased swelling in 72 percent. In 2019 the FDA approved the drug pexidartinib as a treatment option for patients whose tumors continue to grow back or whose diffuse-type TGCT is difficult to treat with surgery, according to NORD.
6. How can I take care of myself after treatment?
Staying active — under the guidance of your doctor or physical therapist — will help the joint maintain range of motion and reduce inflammation in the area, says Scharschmidt. According to him, the inflammation from TGCT can eventually degrade the joint, and treatment aims to reduce that inflammation. “The large problem is from the inflammation that this condition causes within the joint,” he says. Anti-inflammatories, physical therapy, and surgery to remove the TGCT can all be helpful in treating inflammation, says Scharschmidt. People who undergo joint surgery may be at increased risk of developing joint stiffness, which can restrict your ability to stay active, says Scharschmidt. Stiffness from surgery should be temporary and can be improved with physical therapy and anti-inflammatories, he notes. And while there’s no diet that’s been shown to be helpful for TGCT specifically, it may be a good idea to choose foods that have been shown to reduce inflammation and help keep joints healthy. According to the Arthritis Foundation, eating an anti-inflammatory diet rich in fruits, vegetables, nuts, grains, and fish and avoiding foods that can increase inflammation, such as red meat, dairy products, and foods high in refined sugar or flour, may be beneficial for joint health.
7. How can I stay positive after diagnosis?
Because patients tend to be younger adults, a condition that takes away the ability to stay active can be particularly challenging. According to Scharschmidt, how patients feel after a diagnosis can vary dramatically depending on the severity of the disease and if the tumor is localized or diffuse. Because diffuse TGCT has a higher likelihood of coming back, it can contribute to chronic pain and disability, which can significantly impact quality of life. Because this condition can be disruptive, a multidisciplinary team is key in treating the patient and improving outcomes, notes another study published in April 2019 study in the journal The Lancet Oncology. “At our hospital, we view this as a team sport, with a variety of specialists, orthopedic surgeons, physical therapists, pain medicine physicians, and psychologists that can be brought in as part of the treatment team,” says Scharschmidt. This type of comprehensive management helps treat someone both physically and mentally, so you can have the best chance at recovery by learning to cope with the disease, practice practical strategies for functioning in daily life, and manage pain.
