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Tenosynovial giant cell tumor (TGCT)

Includes giant cell tumor of the tendon sheath (GCT-TS) and pigmented villonodular synovitis (PVNS)7,8

These rare tumors can range in their impact on patients.9,10 Localized TGCT, which is the most common type, is often cured with surgery.7,11 Recurrence rates for localized TGCT are estimated to be up to ~15% following complete resection.12-15 Diffuse TGCT recurrence rates are estimated to be about 20% to 50% following complete resection.11,12,16 Additional surgeries for these more severe cases can lead to significant joint damage, debilitating functional impairments, and reduced quality of life.10,17,18

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Localized and diffuse TGCT
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Managing patients with TGCT?

Mechanism of disease

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TGCT growth is characterized by overexpression of colony-stimulating factor 1 (CSF-1) and recruitment and accumulation of colony-stimulating factor-1 receptor (CSF-1R) expressing cells in the synovium.22,23

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Localized TGCT fast facts

This handy card conveys key facts about localized TGCT and can be used to support your discussions with your patients about their condition.

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Diffuse TGCT fast facts

This handy card conveys key facts about diffuse TGCT and can be used to support your discussions with your patients about their condition.

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Footnote

aHypothetical numbers are based on diffuse TGCT literature but are not meant to represent population means. Although not all patients have the same experience, diffuse TGCT typically presents as a longstanding, painful mass that limits range of joint motion, and diagnosis can occur years after onset of disease.8,24

References

1. van der Heijden L, Mastboom MJL, Dijkstra PDS, van de Sande MAJ. Functional outcome and quality of life after the surgical treatment for diffuse-type giant-cell tumour around the knee: a retrospective analysis of 30 patients. Bone Joint J. 2014;96-B(1):1111-1118. doi:10.1302/0301-620X.96B8.33608.

2. de Saint Aubain Somerhausen N, Fletcher CDM. Diffuse-type giant cell tumor: clinicopathologic and immunohistochemical analysis of 50 cases with extraarticular disease. Am J Surg Pathol. 2000;24(4):479-492.

3. Rydholm U. How I do it: pigmented villonodular synovitis. Acta Orthop Scand. 1998;69(2):203-210.

4. Saxena A, Perez H. Pigmented villonodular synovitis about the ankle: a review of the literature and presentation in 10 athletic patients. Foot Ankle Int. 2004;25(11):819-826.

5. Mimura T, Kawasaki T, Yagi K, Mori K, Imai S, Matsusue Y. Pigmented villonodular synovitis causing osteonecrosis of the femoral head: a case report. Orthopedics. 2013;2013:756954. doi:10.1155/2013/756954.

6. 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.

7. de Saint Aubain Somerhausen N, van de Rijn M. Tenosynovial giant cell tumour, localized type. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, eds. WHO Classification of Tumours of Soft Tissue and Bone. Vol 5. 4th ed. Lyon, France: IARC Press; 2013:100-101.

8. de Saint Aubain Somerhausen N, van de Rijn M. Tenosynovial giant cell tumour, diffuse type. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, eds. WHO Classification of Tumours of Soft Tissue and Bone. Vol 5. 4th ed. Lyon, France: IARC Press; 2013:102-103.

9. Gelhorn HL, Tong S, McQuarrie K, et al. Patient-reported symptoms of tenosynovial giant cell tumors. Clin Ther. 2016;38(4):778-793. doi:10.1016/j.clinthera.2016.03.008.

10. 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.

11. 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.

12. 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. doi:10.1016/j.otsr.2016.11.002.

13. 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 Cancer. 2015;51(2):210-217. doi:10.1016/j.ejca.2014.11.001.

14. Rao AS, Vigorita VJ. Pigmented villonodular synovitis (giant-cell tumor of the tendon sheath and synovial membrane): a review of eighty-one cases. J Bone Joint Surg Am. 1984;66(1):76-94.

15. 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.

16. Xie G-P, Jiang N, Liang C-X, et al. Pigmented villonodular synovitis: a retrospective multicenter study of 237 cases. PLOS One. 2015;10(3):e0121451. doi:10.1371/journal.pone.0121451.

17. 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.

18. 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. 2016;17(2):10. doi:10.1007/s11864-015-0385-x.

19. 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.

20. 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.

21. Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Soft Tissue Sarcoma V.1.2019. © National Comprehensive Cancer Network, Inc. 2018.  All rights reserved.  Accessed December 19, 2018.  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.

22. West RB, Rubin BP, Miller MA, et al. A landscape effect in tenosynovial giant-cell tumor from activation of CSF1 expression by a translocation in a minority of tumor cells. Proc Natl Acad Sci U S A. 2006;103(3):690-695. doi:10.1073/pnas.0507321103.

23. Cupp JS, Miller MA, Montgomery KD, et al. Translocation and expression of CSF1 in pigmented villonodular synovitis, tenosynovial giant cell tumor, rheumatoid arthritis and other reactive synovitides. Am J Surg Pathol. 2007;31(6):970-976.

24. Akinci O, Akalin Y, Incesu M, Eren A. Long-term results of surgical treatment of pigmented villonodular synovitis of the knee. Acta Orthop Traumatol Turc. 2011;45(3):149-155. doi:10.3944/AOTT.2011.2442.

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