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Treating TGCT

Surgical resection for complete cure should be considered whenever possible, but may have associated limitations1

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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


Clinical trials

Visit clinicaltrials.gov for information about TGCT clinical trials.

TGCT can be a chronic disease

It is likely that patients with recurrent TGCT will need ongoing care3,10,11

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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

Team-based approach

Consider working across specialties to manage some recurrent, diffuse, or difficult-to-treat TGCT cases7,15

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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
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References

1. 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.

2. Mastboom MJL, Verspoor FGM, Verschoor AJV, et al; TGCT study group. Higher incidence rates than previously known in tenosynovial giant cell tumors: a nationwide study in The Netherlands [published online August 8, 2017]. Acta Orthop. doi:10.1080/17453674.2017.1361126.

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. 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.

5. 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.

6. van der Heijden L, Gibbons CLMH, Hassan AB, et al. A multidisciplinary approach to giant cell tumors of tendon sheath and synovium: a critical appraisal of literature and treatment proposal. J Surg Oncol. 2013;107(4):433-435. doi:10.1002/jso.23220.

7. 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.

8. 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.

9. Jabalameli M, Jamshidi K, Radi M, Hadi H, Bagherifard A. Surgical outcomes of 26 patients with pigmented villonodular synovitis (PVNS) of the knee at a mean follow-up of 4 years: introducing a novel technique. Med J Islam Repub Iran. 2014;28:123.

10. 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.

11. Lucas DR. Tenosynovial giant cell tumor. Arch Pathol Lab Med. 2012;136(8):901-906. doi:10.5858/arpa.2012-0165-CR.

12. 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.

13. 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.

14. 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.

15. 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.

16. Hao Y, Krohe M, Yaworsky A, et al. Clinical trial patient-reported outcomes data: going beyond the label in oncology. Clin Ther. 2016;38(4):811-820. doi:10.1016/j.clinthera.2016.03.010.

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