Answer a question about your experience with TGCT.
Tenosynovial giant cell tumor (TGCT), also known as pigmented villonodular synovitis (PVNS) or giant cell tumor of the tendon sheath (GCT-TS), is a group of rare tumors that typically arises from the
synovium of joints, bursae, and tendon sheaths.1-3 They are typically non-malignant neoplasms but
can be locally aggressive.1,4
TGCT encompasses 2 broad subtypes—diffuse and localized—that differ clinically but share a common etiology.1
A multidisciplinary team of experts can help create a plan to address the long-term management needs of patients with difficult-to-treat TGCT.3,5
Differences in TGCT by subtype
Diffusea (formerly PVNS)
Large tumors with ill-defined borders
that typically present in larger joints1,6,7
Surgical resection can be challenging,6-8
with a recurrence rate of up to 55%9
Symptoms include swelling, joint
locking, and pain1,10,11
Occurs most frequently in adults
under 40 years of age,1 although age
may vary widely2,12
Localizeda (formerly GCT-TS)
Small, well-circumscribed tumors that
typically present in small joints, like digits1,2,13,14
Can typically be treated with surgery,7,12,14,15
with a recurrence rate of up to 15%9
Symptoms include painless swelling and
Occurs most frequently in adults 30 to
50 years of age,1 although it can affect
people of any age2,12
a Subtypes as classified by the World Health Organization.
TGCT can be difficult to diagnose because it presents with symptoms of many other conditions.16 Once diagnosed, it may take a multidisciplinary team of experts to create a plan to address patients’ long-term management needs.3,5
Whether tumors are localized or diffuse can
affect the prognosis of patients.1,8,17
- 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.
- 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
- 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
- Mastboom MJ, Planje R, van de Sande M. The patient perspective on the impact of tenosynovial giant cell tumors on daily living: crowdsourcing study on physical function and quality of life. Interact J Med Res. 2018;7(1):e4. doi:10.2196/ijmr.9325
- 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.
- 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
- 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
- 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
- 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
- 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
- Myers BW, Masi AT. Pigmented villonodular synovitis and tenosynovitis: a clinical epidemiologic study of 166 cases and literature review. Medicine (Baltimore). 1980;59(3):223-238.
- Gouin F, Noailles T. Localized and diffuse forms of tenosynovial giant cell tumor (formerly giant cell tumor of the tendon sheath and pigmented villonodular synovitis). Orthop Traumatol Surg Res. 2017;103(1S):S91-S97. http://dx.doi.org/10.1016/j.otsr.2016.11.002
- Hu Y, Kuang B, Chen Y, Shu J. Imaging features for diffuse-type tenosynovial giant cell tumor of the temporomandibular joint: a case report. Medicine. 2017;96(26):e7383. doi:10.1097/MD.0000000000007383
- 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
- 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
- Illian C, Kortmann HR, Künstler HO, Poll LW, Schofer M. Tenosynovial giant cell tumors as accidental findings after episodes of distortion of the ankle: two case reports. J Med Case Rep. Published online December 15, 2009. 2009;3:9331. doi:10.1186/1752-1947-3-9331
- Ottaviani S, Ayral X, Dougados M, Gossec L. Pigmented villonodular synovitis: a retrospective single-center study of 122 cases and review of the literature. Semin Arthritis Rheum. 2011;40(6):539-546. doi:10.1016/j.semarthrit.2010.07.005
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