Tenosynovial giant cell tumors (TGCTs) are rare tumors that grow in your joints, but they aren't life-threatening. Even though some conditions can show up later, TGCTs can often happen in younger adults. Here's what to know about living with TGCT.
What Is TGCT?
A TGCT can grow slowly (localized) inside a smaller joint or quickly (diffuse) in a large one, like your knee or hip. These tumors can affect your bones and impact your tendon sheath – the tissues covering your tendons. Tendons are rope-like structures that connect your muscles to your bones.
Research suggests a TGCT forms when the cells in your joint accidently swap part of chromosome 1 with chromosome 2, also called a translocation. These types of cells make up most of TGTC cells. Your chromosomes are made of DNA. The swapped chromosome parts cause high levels of a protein called colony stimulating factor-1 (CSF-1), which triggers tumor growth.
TGCT Symptoms
A TGCT may not cause pain at first but can grow larger to press on nearby structures and nerves. This can lead to pain and swelling, making you less able to move around. This condition used to be called PVNS, or pigmented villonodular synovitis.
TGCT symptoms can mimic those of other things, like a sprain or arthritis, which can make it harder to diagnose. Let your doctor know if you have symptoms:
- In one joint that doesn't move around
- That aren't triggered by movement, a time of day, or an injury
Two types of TGCT: What's the difference?
Localized or nodular TGCT tends to grow tumors in the smaller joints, like the ones in your fingers and toes. Your tumor likely has well-defined edges, which means it doesn't mix with your healthy tissues. So that makes this type of TGCT easier to remove with surgery.
Diffuse TGCT can grow and damage the bursae found next to large joints – small, fluid-filled sacs that cushion and help ease movement between your tendons and joints. These sacs are found in your shoulders, elbows, knees, and hips. The knee is most common, but you can get a diffuse TGCT in your hip or ankle. This type of TGCT is also more than twice as likely to return after surgery.
Who Gets TGCT?
Anyone can get TGCT, but you may have a higher risk if you are between 25 and 50 years old. The average age to develop a TGCT is 40, but your risk of diffuse TGCT is higher if you're under 40.
Experts aren't sure, but some research suggests that more:
- Women get localized TGCTs
- Adults get tumors more often than children
Ongoing studies will help doctors figure out if TGCTs happen more often in certain races, medical conditions, or adults. The studies will also help doctors know if the symptoms are different for 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 get TGCT more often. They still haven't found a gene change, environmental factor, or other medical cause for TGCT. It doesn't seem to run in families or be caused by certain types of jobs.
If you have TGCT, consider joining in a clinical trial. You can learn about trials that apply to you by visiting www.clinicaltrials.gov.
Build a Treatment Team for TGCT
You may wonder which doctors can help diagnose and treat a rare condition like TGCT. With diffuse TGCT, it's even more important to stay on top of your health, as things can change quickly. You can take action by putting a TGCT health care team together.
Here are some tips to help you find a doctor who has treated TGCTs before and build a care team.
Building your care team
You will likely see more than one doctor to help you manage TGCT. Your health care team may include:
- Rheumatologist (especially to rule out arthritis)
- Sports medicine doctor
- Orthopedic surgeon
- Orthopedic oncologist
- Medical oncologist
- Physical therapist
Treatment for TGCT in Young Adults
Surgery is often used to treat TGCTs when you are younger, but it's important to know about all your options. Some types of TGCTs are more likely to come back after treatment. Some options may make more sense, depending on the type of TGCT you have.
Localized TGCT treatments
This type of TGCT is often smaller, and surgery may be a good choice. Arthroscopy is a type of surgery using small cuts. They let your surgeon put a small camera into your joint and take out the tumor.
Your doctor may also talk about radiation therapy or chemotherapy – a good fit in rare cases. But experts don't recommend this treatment for TGCT anymore because of serious side effects. If you are unsure or would like another opinion, consider asking an orthopedic oncologist.
Diffuse TGCT treatments
There is more of a risk if you have diffuse TGCT. If not treated, the growth can damage the tissue around your joint to cause disability or even amputation. Removing the tumor is often a first line of defense, and some can also be treated another way.
If you're thinking about getting pregnant, talk to your doctor about your treatment options and the timing. Because diffuse TGCTs have a higher risk of returning, there are medicines that stop the colony stimulating factor-1 receptor (CSF-1R) to shrink the tumor.
If you have diffuse TGCT, your doctor may talk to you about:
- Active surveillance. If you don't have symptoms and surgery isn't an option, your doctor may watch you closely using a detailed check-in and testing plan.
- Pexidartinib (Turalio), an older CSF-1R inhibitor medicine used to treat diffuse TGCTs in adults
- Vimseltinib (Romvimza), a newer CSF-1R inhibitor medicine used to treat diffuse TGCTs in adults
- Imatinib (Gleevec), a leukemia medicine used "off-label" to treat TGCTs and commonly used in younger people
CSF-1R inhibitor side effects
These drugs may be given if you have significant symptoms. Clinical trials have shown significant shrinkage of tumors in 38% to over 60% of people. Your doctor will review the side effects of each option.
These medicines can come with serious side effects, including:
- Possible liver damage
- Rash
- Itching
If you decide to take a CSF-1R inhibitor, your doctor will order regular liver tests and monitor your liver health. If anything changes, they can adjust your medicine to prevent liver damage. They may also offer imatinib (also called Gleevec), a leukemia medicine used "off-label" to treat TGCT.
Living With TGCT
If you're in your 20s, 30s, or 40s, you may be growing a career, raising a family, or traveling. Managing pain can be difficult with TGCT because it can show up as a common symptom or after surgery.
Doctors are researching how pain impacts young adults. Studies suggest up to half of young adults deal with regular pain. TGCT can cause pain in your joints and throughout your body to impact your daily life.
Consider talking to a therapist
Studies show that living with a painful condition like TGCT can impact your mental health, too. Consider a counselor or therapist to help you manage the emotions that come with this condition. It can help to share your challenges of living with TGCT with others. A therapist can also help you find a support group. Talking to others, like your family and friends, can help you feel more connected.
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."
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