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are your patientsTrapped By TGCT?

UNTANGLE THE TRUTH OF TENOSYNOVIAL GIANT CELL TUMOR (TGCT) AND THE STRUGGLES YOUR PATIENTS MAY BE FACING

Surgery every year is exhausting. We want more options to surgery. We want more proactive medicine rather than just waiting for significant regrowth.

— Person living with TGCT

TGCT, previously called pigmented villonodular synovitis (PVNS) or giant cell tumor of the tendon sheath (GCT-TS) is a locally aggressive neoplasm which can cause permanent joint damage, significant pain, swelling, decreased range of motion (ROM), and stiffness.1-3 While some cases of TGCT can be surgically resected and cured, others can be difficult to live with and challenging to treat.4

Truth is, TGCT can be harrowing1,5

Pain, joint stiffness, and physical limitations can be debilitating and life‑altering.5 Surgical resection is considered the standard of care. However, non‑surgical treatment options do exist and many patients could benefit from learning about them sooner in their journey.6-8

There are two types of TGCT1,2

Localized/nodular TGCT

  • Icon depicting the outline of multiple people

    More common9

  • Icon of the outline of a nodular tumor

    Normally a single, well-defined tumor, usually nodular1,10

  • Icon of a small bi-directional arrow pointing diagonally

    Typically smaller, ~2 cm in size1,11

  • Icon of a human joint

    Often does not cause pain or joint dysfunction1,2

  • Icon of crosshairs

    Tumor location:Tends to impact smaller joints like hands, fingers, wrist, foot1,12

silhouette of the human body highlighting areas where tgcts can occur such as wrists, fingers, and feet

Diffuse TGCT

  • Icon depicting the outline of one person

    Less common9

  • Icon of an ill-defined tumor

    Ill-defined tumors that develop outside of the joint13,14

  • Icon of bi-directional arrow pointing diagonally

    Large tumors, >5 cm in size13

  • Icon of a human joint being damaged

    Can extend out of the joint and damage surrounding bone1,14

  • Icon of crosshairs

    Tumor location:Tends to impact larger joints like knee, hip, ankle2,4

silhouette of the human body highlighting areas where tgcts can occur such as hips, knees, and ankles

In a qualitative interview study of 20 participants with histologically confirmed TGCT in the knee, hip, or ankle:

80

%

reported pain6

85

%

reported swelling6

Occurring in a young patient population, TGCT can have major impact on Quality of Life (QoL) and physical function15

TGCT can be a lifelong condition with long‑term impacts resulting from both the disease and treatments.1,15 TGCT affects a relatively young population with the average age of diagnosis being 33 years old.5

The physical and emotional burdens of TGCT can be significant and potentially lifelong1,5

40%or more of
TGCT patients

reported using opioids and NSAIDs in their treatment history3

The TGCT patient journey can be long, frustrating, and fraught with delays15

Once diagnosed, understanding all of the options and getting appropriate treatment can be difficult.1 Surgical resection, usually the first approach, does not treat the hypothesized underlying genetic driver of the disease.6,15

DIAGNOSIS

It can take up to 3-4 years to get a TGCT diagnosis15

PRIMARY TREATMENT

Typically surgery—but it is not a cure for many patients with TGCT and tumors often return3,15

TGCT RECURRENCE

A lifetime risk for both diffuse-type and localized/nodular-type TGCT16

TREATMENT AFTER RECURRENCES

  • Repeated surgical resections. This may not be an option or effective for all TGCT patients and can be debilitating3,15
  • Systemic treatment is often not discussed until a patient has had multiple recurrences and surgical resections15

Patients often get stuck in a surgery loop and may not be aware of other treatment options.3,8

Truth is, the majority of patients with diffuse TGCT will experience recurrence16

Due to the locally aggressive nature of the tumor, complete resection is difficult to achieve and often results in residual disease, recurrence, and multiple surgical resections.3,4,7 Diffuse TGCT is often resected incompletely, resulting in high recurrence rates and worse clinical outcomes.17,18

LIFETIME RECURRENCE RATES16

diffuse
TGCT
up to

55%

localized/
nodular
TGCT up to

15%

With each recurrence, TGCT becomes increasingly difficult to manage.3,4 Repetitive surgical resections can lead to increasingly significant morbidity, including joint destruction and secondary osteoarthritis.3,4 Because of its tendency to recur, diffuse TGCT can become a chronic, lifelong condition for some.1,15

Morbidity risk increases with each subsequent resection, adding to the DIFFICULTY of managing TGCT7,8

The prolonged course of the disease and the need for multiple resections has been reported to contribute to further joint destruction and, in some cases, lead to premature joint replacement or amputation.4

Over

40%of
patients

who had surgery for TGCT underwent 2 or more surgeries3

Joint replacement is not a cure for TGCT and carries the risk of recurrence and complications1,7,19

Truth is, current TGCT treatment options are limited7

The standard of care for symptomatic TGCT is surgical resection, when it can be accomplished without significant morbidity and depending on tumor location and the extent of the disease.1,20 Resection of diffuse TGCT is associated with a high risk of recurrence, postoperative complications, and joint damage.1,4

Repeated surgical resections can lead to increased morbidity, including acceleration of secondary osteoarthritis, permanent joint damage, and other lifelong consequences.3,4

~50 %
of patients
with DIFFUSE TGCT
remain symptomatic, even after SURGERY1,5

Patients with difficult-to-manage, symptomatic disease, or moderate or severe functional impairment, may be candidates for targeted systemic treatment if the expected benefits of resection are outweighed by the morbidity risk.1

The potential benefits of any medication or systemic treatment need to be carefully weighed against the risks and expected impacts on QOL.1

Tyrosine kinase inhibitors (TKIs) targeting the colony stimulating factor 1 (CSF1) pathway have shown a wide range of efficacy, but may be limited by toxicity.20

In TGCT, both the disease and the current treatment options can impact QOL.4 With TGCT being a potentially lifelong and challenging disease to manage, symptomatic and functional improvement are essential parts of treatment decision making.1

What can be done to help improve outcomes if TGCT recurs?

Since TGCT can be challenging to control with surgery alone, a multimodal approach, including systemic treatments should be considered.17 Collaborative care involving a multidisciplinary team including an orthopedic surgeon, medical oncologist, and other sarcoma experts can help improve patient outcomes.1

A multidisciplinary team is critical early in the patient journey especially for patients with symptomatic, recurrent, or unresectable TGCT—or simply for patients who want to understand all of their options.1,15

A page with a corner folded down representing management guidelinesGet the latest TGCT management guidelinesInternational Consensus Recommendations for the Management of TGCT

With ongoing research

there ARE REASONS to be hopeful7

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References: 1. Stacchiotti S, Durr HR, Schaefer IM, et al. Best clinical management of tenosynovial giant cell tumour (TGCT): a consensus paper from a community of experts. Cancer Treat Rev. 2023;112:102491. 2. Spierenburg G, Ballesteros CS, Stoel BC, et al. MRI of diffuse-type tenosynovial giant cell tumour in the knee: a guide for diagnosis and treatment response assessment. Insights Imaging. 2023;14(1):22. 3. Lin F, Kwong WJ, Shi S, Pivneva I, Wu EQ, Abraham JA. Surgical treatment patterns, healthcare resource utilization, and economic burden in patients with tenosynovial giant cell tumor who underwent joint surgery in the United States. J Health Econ Outcomes Res. 2022;9(1):68-74. 4. Verspoor FGM, Mastboom MJL, Hannink RG, et al. Long‑term efficacy of imatinib mesylate in patients with advanced tenosynovial giant cell tumor. Sci Rep. 2019;9(1):14551. 5. Mastboom MJ, Planje R, van de Sande MA. 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. 6. Gelhorn HL, Tong, S, McQuarrie, K, et al. Patient-reported symptoms of tenosynovial giant cell tumors. Clin Ther. 2016;38(4):778-793. 7. Brahmi M, Vinceneux A, Cassier PA. Current systemic treatment options for tenosynovial giant cell tumor/pigmented villonodular synovitis: targeting the CSF1/CSSF1R axis. Curr Treat Options Oncol. 2016;17(2):10. 8. Bernthal NM, Healy JH, Palmerini E, et al. A prospective real-world study of the diffuse-type tenosynovial giant cell tumor patient journey: a 2-year observational analysis. J Surg Oncol. 2022;126(8):1520-1532. 9. Lopez-Bastida J, Aranda-Reneo I, Rodríguez-Sánchez B, et al. Economic burden and health-related quality of life in tenosynovial giant-cell tumour patients in Europe: an observational disease registry. Orphanet J Rare Dis. 2021;16(1):294. 10. Mastboom MJL, Verspoor FGM, Gelderblom H, van de Sande MAJ. Limb amputation after multiple treatments of tenosynovial giant cell tumour: series of 4 Dutch cases. Case Rep Orthop. 2017;2017:7402570. 11. Robert M, Farese H, Miossec P. Update on tenosynovial giant cell tumor, an inflammatory arthritis with neoplastic features. Front Immunol. 2022;13:820046. 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. 13. Ramteke P, Iver VK, Madan K, Gamangatti S, Mridha AR. Fine needle aspiration cytology diagnosis of metastatic malignant diffuse type tenosynovial giant cell tumor. J Cytol. 2017;34(3):174-176. 14. Wang C, Song R-R, Kuang P-D, Wang L-H, Zhang M-M. Giant cell tumor of the tendon sheath: magnetic resonance imaging findings in 38 patients. Oncol Lett. 2017;13(6):4459-4462. 15. Bernthal NM, Spierenburg G, Healey JH, et al. The diffuse-type tenosynovial giant cell tumor (dt-TGCT) patient journey: a prospective multicenter study. Orphanet J Rare Dis. 2021;16(1):191. 16. 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. 17. Spierenburg G, van der Heijden L, Mastboom MJL, et al. Surgical management of 144 diffuse-type TGCT patients in a single institution: a 20-year cohort study. J Surg Oncol. 2022;126(6):1087-1095. 18. Van IJizendoorn DGP, Matusiak M, Charville GW, et al. Interactions in CSF1-driven tenosynovial giant cell tumors. Clin Cancer Res. 2022;28(22):4934-4946. 19. Gómez-Barrena E, García-Rey G. Complications in total joint arthroplasties. J Clin Med. 2019;8(11):1891. 20. Bernthal NM, Ishmael CR, Burke ZDC. Management of pigmented villonodular synovitis (PVNS): an orthopedic surgeon’s perspective. Curr Oncol Rep. 2020;22(6):63.
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