Surgical resection for complete cure should be considered whenever possible, but may have associated limitations1
While there are no official guidelines for the surgical treatment of tenosynovial giant cell tumor (TGCT), surgical resection in an open or arthroscopic procedure is recommended when possible.1 There is no consensus about the most appropriate type of surgery; each case should be evaluated individually.2
Surgery can often be curative for patients with TGCT, especially for localized lesions that are completely resectable. However, complete surgical removal of diffuse TGCT lesions is not always achievable as lesions are typically infiltrative and locally aggressive.1,3,4
Management beyond surgery
In some instances, neoadjuvant or adjuvant approaches may be employed for the treatment of tumors that are not completely resectable or for recurrent tumors.5,6
There are currently no systemic therapies approved for the treatment of TGCT, although some systemic therapies with reported activity in TGCT are included in the literature and in the NCCN Clinical Practice Guidelines In Oncology (NCCN Guidelines®) for Soft Tissue Sarcoma.6,7
Physical therapy may be employed after surgery as part of the plan of care to rehabilitate joint function.8,9
Physicans may also use palliative treatment, including pain relievers, anti-inflammatory drugs, or steroids, to help patients with TGCT.4
Visit clinicaltrials.gov for information about TGCT clinical trials.
"If I'm taking out a sarcoma, it's a mass. I can draw a circle around it. For this disease [diffuse TGCT], it's hard to draw a circle around it. The only way you can often do that is to take the whole joint out."
“…diffuse TGCT is a difficult management problem. Complete removal is very challenging and often very morbid, and it can be debilitating, end up in joint replacements, and still have recurrences. I’ve seen that. That’s a lot of surgery.”
“Sometimes I see patients with TGCT who have very extensive disease, but they have rather limited symptoms. They actually have good quality of life. They are able to do their daily activities. They don’t use any pain medication. They don’t really have a big problem. When you look at their MRI scan, you would be scared by the extension of the disease.
At the same time, sometimes you can see patients with a relatively small amount of pathologic tissue, a small amount of TGCT, but with very debilitating disease. So they have a lot of pain, they are unhappy, they have stiffness…The amount of symptoms is not just a linear correlation with the amount of pathological tissue that you can see on the MRI scan.”
“I’ve been really surprised over the years to find out that, in spite of the benign definition of the disease, how badly this disease affects them [patients]. The difference is an important range of interrelated symptoms that go from pain to reduced function but also a change in social skills from a work point of view and then sports activities. The people with this disease–and I’m thinking for…lesions like knee and hips…even a lesion on an MRI that doesn’t seem gigantic–they really change the day of these individuals.”
“The first thing that comes to mind is just that this will be a recurrent tumor, that this is likely something that is going to become a chronic condition. …it could be something for which we do surgery and it comes back, and we try to counsel people that is going to be potentially a recurrent issue and something to deal with for years to come.”
TGCT can be a chronic disease
It is likely that patients with recurrent TGCT will need ongoing care3,10,11
Surgery can often be curative for patients with TGCT—especially for localized lesions (including giant cell tumor of the tendon sheath [GCT-TS]) that are completely resectable.3,12,13 However, complete surgical removal of diffuse TGCT lesions is not always achievable as lesions are typically infiltrative and locally aggressive.3,10,11
Diffuse TGCT, which includes pigmented villonodular synovitis (PVNS), frequently recurs after surgery and may result in the reduced functional ability of the affected joint.5
Diffuse, recurrent TGCT may lead to multiple surgeries, substantial morbidity of the joint, secondary osteoarthritis and, in some cases, joint arthroplasty.10,14
Consider working across specialties to manage some recurrent, diffuse, or difficult-to-treat TGCT cases7,15
While many TGCT cases can be resolved with one surgery, patients with recurrent, diffuse, or difficult-to-treat TGCT may benefit from team-based care.7,15
Academic centers may be a source of expertise in these rare tumors. Referring your patients with recurrent TGCT to a specialized oncology or sarcoma center can help support their potential long-term management needs.
Providing collaborative care for patients with TGCT, especially those with diffuse disease, which has a higher risk of recurrence, may help improve outcomes.15,16
The NCCN Guidelines® for Soft Tissue Sarcoma recommend that patients with TGCT/PVNS be evaluated and managed by a multidisciplinary team with experience and expertise in sarcoma.7
Every patient with TGCT is different, and several specialists may be involved throughout the disease course, including:
- Hand surgeon, orthopedic surgeon, or sports medicine
- Medical and surgical oncologists, as well as oncology nurses
- Radiologists and pathologists
- Physical and occupational rehabilitation therapists