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Click a tab below to learn about treating diffuse or localized TGCT.

DIFFUSE TGCT
(PVNS)
LOCALIZED TGCT
(GCT-TS)
Surgery

Diffuse TGCT may require multiple surgeries or may not be cured
with surgery6,8

Surgical removal of diffuse tumors can be difficult because they have poorly defined boundaries.3,6-8 Where possible, however, surgical resection of diffuse tumors in an open or arthroscopic procedure is the standard of care.6 Because consensus about the most appropriate type of surgery currently does not exist, cases should be evaluated individually.3,10,11

The NCCN Clinical Practice Guidelines In Oncology (NCCN Guidelines®) for Soft Tissue Sarcoma recommend that patients with TGCT be evaluated and managed by a multidisciplinary team.12 Because TGCT is rare,11 having multiple specialists on the team may help deliver the best patient care.2,12

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Recurrence rate

Complete resection can be challenging, and recurrence
is common3,6,8,13

Diffuse TGCT, which lacks definitive boundaries, can be difficult to remove completely.3,6-8 Recurrence rates are estimated to be up to 55% (up to 1 in 2 patients).13 Patients may require multiple surgeries, which can lead to substantial morbidity of
the joint and the need for future joint arthroplasty—or, in extremely rare cases, amputation.6,8,10 The potentially long-term course of the disease can be associated with loss of joint function and development of early secondary osteoarthritis.5

Up to 55%
Ongoing management

Management beyond surgery may include physical therapy to rehabilitate joint function.14 Additionally, palliative treatment, including pain relievers, anti-inflammatory drugs, or steroids, may be prescribed.1 In cases where the tumor has led to extensive bone destruction, joint repair surgery may be warranted.15

See the literature and NCCN Guidelines® for
Soft Tissue Sarcoma at NCCN.org for treatment recommendations.

Recovery takes tenacitya “He had a 40% deficit in his thigh muscle from being in the leg immobilizer, so he had a lot of work to do. But I was just so proud of him and just in awe at the tenacity to say, ‘Okay, Mom, we’re going to do this,’ and I could see the pain when he’s doing the physical therapy, but he stuck with it.” —Caregiver of patient with recurrent,
diffuse TGCT in knee

a The impact of TGCT is different for everyone; this information is not meant to be reflective of all patients with TGCT.

Surgery

For most patients with localized disease, surgery can be curative16

Surgical resection in an open or arthroscopic procedure is the standard of care where possible.6 Because consensus about the most appropriate type of surgery currently does not exist, cases should be evaluated individually.3,10,11

Recurrence rate

Recurrence rate after complete resection is up to 15% (up to
1 in 7 patients)13

Recurrent localized tumors usually are not destructive. Typically, localized tumors
are treated with repeated surgical removal.17

Up to 15%
Ongoing management

Management beyond surgery may include physical therapy to rehabilitate joint function.14
Additionally, palliative treatment, including pain relievers, anti-inflammatory drugs, or steroids,
may be prescribed.1

See the literature and NCCN Guidelines® for
Soft Tissue Sarcoma at NCCN.org for treatment recommendations.

References

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  1. Gelhorn HL, Tong S, McQuarrie K, et al. Patient-reported symptoms of tenosynovial giant cell tumors. Clin Ther. Published online April 1, 2016. 2016;38(4):778-793. doi:10.1016/j.clinthera.2016.03.008
  2. van der Heijden L, Gibbons CLMH, Dijkstra PDS, et al. The management of diffuse-type giant cell tumour (pigmented villonodular synovitis) and giant cell tumour of tendon sheath (nodular tenosynovitis). J Bone Joint Surg Br. 2012;94(7):882-888. doi:10.1302/0301-620X.94B7.28927
  3. Ravi V, Wang W-L, Lewis VO. Treatment of tenosynovial giant cell tumor and pigmented villonodular synovitis. Curr Opin Oncol. 2011;23(4):361-366. doi:10.1097/CCO.0b013e328347e1e3
  4. Dines JS, DeBerardino TM, Wells JL, et al. Long-term follow-up of surgically treated localized pigmented villonodular synovitis of the knee. Arthroscopy. 2007;23(9):930-937. doi:10.1016/j.arthro.2007.03.012
  5. Verspoor FGM, Zee AAG, Hannink G, van der Geest ICM, Veth RPH, Schreuder HWB. Long-term follow-up results of primary and recurrent pigmented villonodular synovitis. Rheumatology (Oxford). 2014;53(11):2063-2070. doi:10.1093/rheumatology/keu230
  6. Brahmi M, Vinceneux A, Cassier PA. Current systemic treatment options for tenosynovial giant cell tumor/pigmented villonodular synovitis: targeting the CSF1/CSF1R axis. Curr Treat Options Oncol. Published online January 28, 2016. 2016;17(2):10. doi:10.1007/s11864-015-0385-x
  7. Lucas DR. Tenosynovial giant cell tumor: case report and review. Arch Pathol Lab Med. 2012;136(8):901-906. doi:10.5858/arpa.2012-0165-CR
  8. Palmerini E, Staals EL, Maki RG, et al. Tenosynovial giant cell tumour/pigmented villonodular synovitis: outcome of 294 patients before the era of kinase inhibitors. Eur J Canc. 2015;51(2):210-217. doi:10.1016/j.ejca.2014.11.001
  9. Verspoor FGM, van der Geest ICM, Vegt E, Veth RPH, van der Graaf WT, Schreuder HWB. Pigmented villonodular synovitis: current concepts about diagnosis and management. Future Oncol. 2013;9(10):1515-1531. doi:10.2217/fon.13.124
  10. Griffin AM, Ferguson PC, Catton CN, et al. Long-term outcome of the treatment of high-risk tenosynovial giant cell tumor/pigmented villonodular synovitis with radiotherapy and surgery. Cancer. 2012;118(19):4901-4909. doi:10.1002/cncr.26529
  11. Mastboom MJL, Verspoor FGM, Verschoor AJ, et al; TGCT study group. Higher incidence rates than previously known in tenosynovial giant cell tumors: a nationwide study in The Netherlands. Acta Orthop. 2017;88(6):688-694. doi:10.1080/17453674.2017.1361126
  12. Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Soft Tissue Sarcoma V.1.2021. © National Comprehensive Cancer Network, Inc. 2020. All rights reserved. Accessed November 9, 2020. To view the most recent and complete version of the guideline, go online to NCCN.org. NCCN makes no warranties of any kind whatsoever regarding their content, use or application and disclaims any responsibility for their application or use in any way.
  13. Ehrenstein V, Andersen SL, Qazi I, et al. Tenosynovial giant cell tumor: incidence, prevalence, patient characteristics, and recurrence. A registry-based cohort study in Denmark. J Rheumatol. 2017;44(10):1476-1483. doi:10.3899/jrheum.160816
  14. Hegedus EJ, Theresa K. Postoperative management of pigmented villonodular synovitis in a single subject. J Orthop Sports Phys Ther. 2008;38(12):790-797. doi:10.2519/jospt.2008.2934
  15. Sharma H, Jane MJ, Reid R. Pigmented villonodular synovitis: diagnostic pitfalls and management strategy. Curr Orthop. 2005;19(3):215-222. doi:10.1016/j.cuor.2005.02.013
  16. Mastboom MJL, Verspoor FGM, Hanff DF, et al. Severity classification of tenosynovial giant cell tumours on MR imaging. Surg Oncol. 2018;27(3):544-550. doi:10.1016/j.suronc.2018.07.002
  17. So-called fibrohistiocytic tumours. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, eds.  WHO Classification of Tumours of Soft Tissue and Bone. 4th ed. International Agency for Research on Cancer; 2013:99-108. Bosman FT, Jaffe ES, Lakhani SR, Ohgaki H, eds. World Health Organization Classification of Tumours; vol 5.