Many diseases like dementia and certain cancers tend to show up in older ages. Some conditions, like one called TGCT, can impact younger adults. Here’s what to know about living with TGCT.
What Is TGCT?
Tenosynovial giant cell tumors (TGCTs) are rare tumors that cover your joints. It is a noncancerous disease and also not life-threatening. These tumors can affect your bones and impact your tendon sheath (tissues that cover tendons). Tendons are rope-like structures that connect your muscles to your bones.
TGCT can also damage your bursa – small, fluid-filled sacs that provide cushion and ease of movement between your tendons and joints. These sacs are located next to large joints like in your shoulders, elbows, knees, and hips. TGCT can impact small joints like your toes or larger joints such as those in your knee.
When any of these areas are affected, it can cause pain and swelling and limit your ability to move around. This condition used to be called PVNS or pigmented villonodular synovitis.
The symptoms of TGCT can mimic other things like a sprain or arthritis, so it is important to talk to your doctor.
Two types of TGCT
Tumors for TGCT fall into two types: localized or diffused. What’s the difference?
- Localized or nodular TGCT tends to grow tumors in the smaller joints like in your fingers and toes. Localized TGCT mainly has one tumor with well-defined edges. These are easier to remove with surgery.
- Diffuse TGCT tumors are often found in larger joints and are more than twice as likely to return even after surgery. The knee is the most common for diffused TGCT, but it can also show up in your hips or ankles. Diffuse TGCT has several tumors, which tend to be larger.
Who Gets TGCT?
Anyone can get TGCT, but it is more common in certain groups. These include:
- Women and people assigned female assigned at birth (AFAB)
- Adults ages 20 to 50
The average age is 40. Young adults (under age 40) tend to get diffuse TGCT more often than localized.
Females and males equally get diffused giant cell tumors, while women are more likely to have localized TGCTs. They can affect all races, but studies show a higher rate in Asian populations. More research is needed on how TGCT impacts certain races.
More adults than children get TGCT, but children can be diagnosed. Experts are still learning how TGCT impacts children, but so far, the symptoms and repeat tumors mirror those of adults.
Why are young adults more at risk?
Experts are unsure why young adults, and women and people AFAB especially, are more at risk for getting TGCT. It doesn’t seem to run in families or be caused by certain types of jobs. More research is needed. If you have TGCT, consider participating in a clinical trial. You can learn about trials that apply to you by visiting www.clinicaltrials.gov.
Treating TGCT in Young Adults
Surgery is often used to treat TGCT. But the tumors can come back. There is more of a risk if you have diffuse TGCT. If not treated, your affected joints and the tissue around them can be damaged enough to cause a disability. In rare cases and if left untreated, TGCT can lead to amputation.
While removing the tumor(s) is the first line of defense, some tumors are also treated with radiation or chemotherapy. Normally, chemotherapy is used to treat cancerous tumors, but it can remove noncancerous ones like with TGCT also.
Young adults of child-bearing age will want to discuss all their treatment options with their doctors as they make their treatment decisions and timing.
Because young adults are more likely to have diffuse TGCT, it’s important to stay on top of your health as things can change quickly. Find someone who has treated the disease previously. If you have diffuse TGCT, keep the following in mind:
- Get a clear diagnosis. Studies show that TGCT is often misdiagnosed or has a late diagnosis. Because the tumors can grow, it’s important to advocate for yourself if you aren’t getting answers for your pain.
- Ask about options. Ask your doctor about radiation treatment. This is sometimes used for repeat tumors.
- Know your rights. Many patients with TGCT (either localized or diffuse) say it can be hard to perform their jobs. If pain or symptoms are making it tough for you to keep a job, talk to the Department of Labor about your options to take leave or to request changes at your job.
- Find support. Patient accounts suggest TGCT is “a lonely disease.” Some say friends and family discount their symptoms because “it’s not cancer.”
- Find the right doctor. TGCT is rare, so it can be hard to find a doctor with experience in it. Ask your primary doctor for suggestions, and don’t be afraid to ask specialists if they’ve treated TGCT before.
Building your care team
You will likely see more than one doctor to help you manage TGCT. Your health care team may include a rheumatologist (especially to rule out arthritis), a sports medicine doctor, an orthopedic surgeon, an orthopedic oncologist, a medical oncologist, and a physical therapist.
Studies show living with a painful condition like TGCT can impact your mental health. Consider a counselor or therapist to help you manage the emotions that come with illness and the challenges of living with a chronic condition.
Living With TGCT
If you’re in your 20s, 30s or 40s, you may be growing a career, raising a family, or traveling. Managing your life while managing pain can be difficult. For people with TGCT, pain can show up as a common symptom or after surgery. Pain in young adults needs more research. Studies suggest as many as 20% to 50% of young adults deal with regular pain. Conditions like TGCT also involve pain in the joints and throughout the body. Several things impact your ability to manage pain as a young adult. These include:
- Smoking
- Poor sleep quality and quantity
- Alcohol use
- Obesity
Here are seven tips to help you better manage your pain while living with TGCT.
- Exercise regularly.
- Stay at a healthy weight.
- Try an anti-inflammatory diet.
- Take your vitamins.
- Don’t ignore your pain.
- Talk to your doctor if TGCT is impacting your life.
Hope on the Horizon
While surgery has been the main treatment for patients with TGCT for years, a new drug offers hope for other options.
Research suggests TGCT forms from a transition of a certain chromosome. This produces a high level of a certain protein called colony stimulating factor-1 or CSF-1. These types of cells make up most of TGTC cells.
Scientists believe if you could block CSF-1, you could treat TGCT. This research led to the recently FDA-approved drug called pexidartinib (Turalio). In clinical trials, 50% of cases showed positive results. For 18% of patients, the tumor disappeared completely. Side effects noted include possible liver damage, rash or itching.
Show Sources
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SOURCES:
Cancer Care: “Improving Resources and Support for Patients with Tenosynovial Giant Cell Tumors.”
City of Hope: “New Drug Tackles Teens’ Leg Tumor Without Surgery.”
Cleveland Clinic: “Tendons,” “Tenosynovial Giant Cell Tumor.”
Everyday Health: “Lifestyle Changes.”
Family Doctor.org “Pigmented Villonodular Synovitis.”
National Council on Aging: “The Top 10 Most Common Chronic Conditions in Older Adults.”
National Institutes of Health: “Characterizing chronic pain in late adolescence and early adulthood: prescription opioids, marijuana use, obesity, and predictors for greater pain interference.”
Radiological Society of North America: “Giant Cell Tumor of Bone: Review, Mimics, and New Developments in Treatment.”
TGCTsupport.org: “What Is A Tenosynovial Giant Cell Tumor?” “Types of TGCT.”
