Tenosynovial giant cell tumors (TGCTs) happen when the soft tissue around your joints overgrow and thicken. Although they're called tumors, this extra tissue isn't cancer but a benign growth. That means your TGCT won't spread to other parts of your body. But the growth can press on nearby structures to cause stiffness, pain, and joint damage.
Because TGCTs are so rare, many doctors don't know about them. Localized TGCT is typically found as a smaller bump (nodule) in the small joints of your fingers and toes. Diffuse TGCT can grow faster, often in the larger joints, like your hip or knee. So getting the right diagnosis and treatment is important.
"Oftentimes, people are diagnosed with TGCTs by orthopedic specialists, who then recommend surgery," says Sydney Stern, PhD, director of Giant Cell Tumor Programs with TGCT Support, a nonprofit patient advocacy organization.
Surgery may not be necessary, especially with new treatments. Exploring your options and getting a second opinion can be a great choice. Here's a look at TGCT diagnosis and the latest therapies. Find out when and how to look for a second opinion so you can make sure that you're getting the right care.
When to Get a Second Opinion About a TGCT Diagnosis
It can be tough to get a diagnosis for TGCT because the growth can look like other common health problems. In fact, it can take three to four years to get diagnosed.
"Since TGCT usually causes joint swelling and pain, its symptoms are often thought to be due to something else, like a sports injury," says Stern.
One study showed a delay of almost three years to diagnose TGCTs in the knee and ankle. But you can shorten that time by finding a specialist and seeing a team at specialized centers. Find out which doctors can help, which symptoms to watch for, and what tests can diagnose your TGCT.
Doctors who can help
Not everyone with a tenosynovial giant cell tumor gets symptoms at first. But if you have common signs like pain and swelling around your joint, you might first see:
- Your regular doctor
- An orthopedic specialist, a doctor who treats muscles and bones
- A sports medicine specialist, a doctor who treats sports injuries
If your symptoms don't get better after a few weeks of treatment such as physical therapy, ask your doctor about more testing.
Key symptoms to watch
Some TGCT symptoms can give your doctor a clue that it's another problem. Let your doctor know if you notice:
- Pain, swelling, or stiffness in one joint, because arthritis often happens in more than one joint
- Symptoms that aren't from a sports injury or trauma
- A firm bump in your joint that grows and is probably not painful
- Without a reason, your joint pops, locks up, or doesn't feel stable, "especially if it's not triggered by activity or time of day," says Stern.
- Symptoms that get worse and don't get better after rest, ice, and other treatments
- If you're younger, under the age of 40
Your doctor may start by ordering imaging tests to look inside your joint.
Is it time for another opinion?
For you to choose the best treatment and prevent problems, it's important to get the right diagnosis. Here are a few things to consider when you look for a second opinion.
Tests for diagnosis. An X-ray or ultrasound picture of your joint may point to a TGCT, but these tests can also miss one. To be sure, you'll need an MRI or synovial biopsy.
Type of doctor. Stern says an orthopedic specialist can do the tests and make the diagnosis for you. If they are unsure, try an orthopedic oncologist. They're an orthopedic surgeon who also treats benign and cancerous tumors in the bone and soft tissues.
Using another name for TGCT. You may also want to see a specialist if your doctor calls your TGCT by an older name like:
- Pigmented villonodular synovitis (PVNS)
- Giant cell tumor of the tendon sheath (GCTTS)
In 2013, the older names were combined into one: tenosynovial giant cell tumors.
"Health care providers who still refer to TGCT by these names may also have outdated understanding of the condition," says Stern. "They may not be aware of new drug treatments, and even promote older treatments such as steroids that haven't been proven effective."
When Should I Get a Second Opinion About My TGCT Treatment Plan?
Here are some reasons to look for a second opinion about your TGCT treatment.
Surgery is the treatment
Consider another opinion anytime surgery is the only treatment offered. There are many new therapies.
"Surgery has traditionally been the first-line treatment for all forms of TGCT," says Stern. "But now there are new medicines available that may be more effective."
Your doctor isn't sure about your TGCT type
The best treatment plan can depend on your type of TGCT, localized or diffuse. Most people have localized TGCT (80%), often found in small joints like your hands and toes.
Localized TGCT responds best to surgery. But diffuse TGCT has a higher chance of coming back again.
You aren't offered medicine for diffuse TGCT
Newer medicines target a substance in your body that helps the tumor form, called colony stimulating factor-1 (CSF-1). This is very helpful if you have diffuse TGCT because there's a higher risk that your tumor will come back after treatment.
Your doctor may offer a medicine such as:
Imatinib (Gleevec). This medicine was approved to treat a form of leukemia, and some doctors also use it to treat TGCT. "It works to target a protein that's in the same family as CSF-1," says Stern. One study found about a third of the TGCT tumors got smaller and the medicine lowered symptoms in most people with TGCT.
Pexidartinib (Turalio). It lowers CSF-1 levels and blocks certain immune cells to help shrink your tumor and ease pain and swelling. Pexidartinib helped ease symptoms in over half of the people with diffuse TGCT.
Romvimza (Vimseltinib). This also lowers CSF-1 levels and blocks immune cells to help treat your TGCT. Vimseltinib shrank the tumor by at least 30% after six months in just under half the people with TGCT.
Your doctor only gives you one option
Weighing the pros and cons of each medication can help you make the best choice. Here are some differences about TGCT treatments to consider.
The common choice. "Pexidartinib is considered more 'tried and true,' because it's been FDA approved for the longest and has the most robust outcome data," says Stern.
Twice a day. You take pexidartinib twice a day with 11 to 14 grams of fat. That can mean a lot of time and effort. "It's very specific. You can't consume 16 grams, which is about two tablespoons of peanut butter," says Stern. "So you really need to calculate your fat precisely."
Twice weekly. Romvimza may be an easier choice for some people. You take it twice a week, at least three days apart.
Under 18. "Imatinib is sometimes prescribed for kids and teenagers, since it's very effective and relatively cheap," says Stern.
You're not referred to a specialist
Stern suggests seeing an orthopedic oncologist after your TGCT diagnosis, even if you need surgery. They're a doctor that removes soft tissue tumors like TGCTs.
Sometimes they may need to remove a small growth. But "it's best for your orthopedic oncologist to refer you to someone," says Stern. "So both doctors can sit with you at the same table to discuss treatment options."
Questions to Ask a New TGCT Doctor
Where to Find Providers
Here are some tips to find doctors who treat TGCT.
Know your team. Medical oncologists diagnose tumors like TGCT and treat them with medications, but orthopedic oncologists treat them with surgery. Your team may include doctors like:
- Musculoskeletal radiologist
- Physical therapist
- Orthopedic surgeon
Look on the TGCT Support website to find an oncologist nearby. If there isn't one, TGCT Support can help you find a specialist in your area.
"Oftentimes, we try to connect patients to one of the larger TGCT centers via telehealth, then help them find someone local to manage their care," says Stern.
Another great place to find a specialist is the Musculoskeletal Tumor Society or TGCT Support websites.
Show Sources
Photo Credit: iStock/Getty Images
SOURCES:
Sydney Stern, PhD, director, Giant Cell Tumor Programs, TGCT Support, Baltimore.
UCSF Health: "Synovial biopsy."
National Organization for Rare Disorders: "Tenosynovial Giant Cell Tumor – Symptoms, Causes, Treatment."
Orphanet Journal of Rare Diseases: "The diffuse-type tenosynovial giant cell tumor (dt-TGCT) patient journey: a prospective multicenter study."
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TGCT Support: "Turalio," "FAQs."
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