What is tenosynovial giant cell tumor (TGCT)? Tenosynovial Giant Cell Tumor (TGCT) is a rare, noncancerous, locally aggressive tumor that occurs in a joint, bursae, or tendon leading to functional impairment and damage to neighboring tissue. In 2013, the World Health Organization classified pigmented villonodular synovitis (PVNS) and giant cell tumor of the tendon sheath (GCT-TS) as TGCT. TGCT has two subtypes: localized (sometimes called nodular) and diffuse.
Is PVNS the same thing? TGCT is now an umbrella term for giant cell tumor of the tendon sheath (GCT-TS) and pigmented villonodular synovitis (PVNS). This was changed in 2013 to reflect the advanced understanding of disease such as that the cells grow and divide, steroids are ineffective, and inflammation is secondary to the tumors.
Is TGCT cancerous? TGCT is a type of non-cancerous tumor. TGCT is not life-threatening but can be life-limiting and lead to damage to surrounding joint tissue. In severe cases, it can invade healthy tissue. TGCT is treated at cancer centers by specialists who treat sarcoma. These specialists are trained to treat difficult tumors.
Will it spread? TGCT only occurs in a single joint and will not spread to any other part of the body. However, TGCT can worsen which can cause damage to surrounding regions of the joint.
Is this genetic? Is this hereditary? TGCT occurs sporadically. There is no occupational, dietary, genetic, or lifestyle risk that has been identified to predispose anyone to getting this disease. TGCT cannot be passed down.
How does the disease destroy the joint and bone? TGCT leads to swelling and can put pressure on bones leading to impairment and pain. Bone erosion can occur over time as the tumors grow. New research (Spierenberg et al., 2024) has demonstrated that 1 in 5 patients developed osteoarthritis following disease diagnosis.
What are the recurrence rates? TGCT symptoms and recurrence can vary by patient. TGCT can grow slowly or quickly. In localized TGCT, the recurrence is around 15%, whereas in diffuse TGCT, recurrence can be as high as 50-70%. Recurrence rates may increase following the first recurrence. It is important to be treated by an experienced multidisciplinary team at an expert center. Check out our specialists list to find a multidisciplinary team near you.
FAQ about Treatment
What are the treatment options? TGCT is often initially treated with surgery. In some cases, surgery can be curative, especially when the disease is localized. However, in diffuse TGCT, recurrence rates may be high. In these cases, drug options such as Turalio and imatinib may be used. There are also clinical trial options available globally. Active surveillance (also called active monitoring) is an important strategy for individuals who are managing symptoms of disease and may be discussed with your multidisciplinary team.
Does a joint replacement reduce recurrence? Joint replacements are used when there is bone and tissue damage that is beyond repair. It does not eliminate or lower recurrence and TGCT can still occur around the replacement. Joint replacements can distort MR imaging and reduce the visibility of TGCT. Additionally, TGCT can undermine the replacement and increase hardware failure rate. Joint replacements should not be used to compensate for incomplete removal of disease. Discuss a plan to monitor disease after replacement with your healthcare team.
What are the risks of surgery? Consult with your healthcare team about the risks associated with surgery. Risks vary depending on TGCT location, extent of disease, and surgical techniques. Multiple surgeries can lead to scar tissue buildup and recurrence risk increases after the first recurrence. The benefit of removing only part of the disease and not all of it (known as debulking or debridement) is controversial.
Is monitoring the tumors a treatment option? Depending on the patient, surgery is not always immediately needed. In patients that are experiencing mild symptoms and minimal surrounding damage, monitoring is a preferred approach. Consult with your healthcare provider about proper monitoring schedules to catch any growth.
Is radiotherapy effective? Due to the lack of effectiveness demonstrated in research studies and the risk of radiation-induced sarcoma, radiation should be avoided. Radiation can also lead to scar tissue formation, joint damage, and nerve pain.
FAQ about Living with Disease What symptoms are common? TGCT patients often report pain, stiffness, popping or locking, and limited range of motion. Some patients experience a lot of symptoms, others may have no symptoms at all. Each person may vary in their presentation. There is no symptom that is specific to TGCT and therefore, the disease is often misdiagnosed for more prevalent issues such as sport's injuries, Baker's cysts, lipoma, non-ossifying fibroma, and muscle sprain.
Can lifestyle or dietary changes help my disease? In general, research into the impact of lifestyle and diet is scarce in TGCT. More research is needed to determine how anti-inflammatory diets and low-impact exercise may influence TGCT. However, it is unlikely that diet will treat TGCT itself but patients report that some dietary changes may help symptoms associated with disease. Check out our Nutrition Page for more information.
How does TGCT affect exercising? High-impact exercise such as running is generally avoided following diagnosis to preserve the joint. However, exercise and movement will not change the growth of TGCT. Physical therapy can be used to strengthen the muscles surrounding the joint and promote joint stability. Consult with your healthcare team for the best exercises for you.
