A prospective real-world study of the diffuse-type tenosynovial giant cell tumor patient journey: A 2-year observational analysis Journal Article


Authors: Bernthal, N. M.; Healey, J. H.; Palmerini, E.; Bauer, S.; Schreuder, H.; Leithner, A.; Martin-Broto, J.; Gouin, F.; Lopez-Bastida, J.; Gelderblom, H.; Staals, E. L.; Burke, Z. D.; Geiger, E. J.; Spierenburg, G.; Laeis, P.; Beyerlein, E.; Ye, X.; van de Sande, M.
Article Title: A prospective real-world study of the diffuse-type tenosynovial giant cell tumor patient journey: A 2-year observational analysis
Abstract: Background and Objectives: Diffuse-tenosynovial giant cell tumor (D-TGCT) is a rare, locally aggressive, typically benign neoplasm affecting mainly large joints, representing a wide clinical spectrum. We provide a picture of the treatment journey of D-TGCT patients as a 2-year observational follow-up. Methods: The TGCT Observational Platform Project registry was a multinational, multicenter, prospective observational study at tertiary sarcoma centers spanning seven European countries and two US sites. Histologically confirmed D-TGCT patients were categorized as either those who remained on initial treatment strategy (determined at baseline visit) or those who changed treatment strategy with specific changes documented (e.g., systemic treatment to surgery) at the 1-year and/or 2-year follow-up visits. Results: A total of 176 patients were assessed, mean diagnosis age was 38.4 (SD ± 14.6) years; most patients had a knee tumor (120/176, 68.2%). For the 2-year observation period, most patients (75.5%) remained on the baseline treatment strategy throughout, 54/79 patients (68.4%) remained no treatment, 30/45 patients (66.7%) remained systemic treatment, 39/39 patients (100%) remained surgery. Those who changed treatment strategy utilized multimodal treatment options. Conclusions: This is the first prospectively collected analysis to describe D-TGCT patient treatments over an extended follow-up and demonstrates the need for multidisciplinary teams to determine an optimal treatment strategy. © 2022 The Authors. Journal of Surgical Oncology published by Wiley Periodicals LLC.
Keywords: adult; controlled study; bone tumor; major clinical study; clinical trial; multimodality cancer therapy; systemic therapy; treatment planning; cancer patient; follow up; prospective study; prospective studies; treatment indication; clinical assessment; pathology; sarcoma; multicenter study; clinical evaluation; knee; recurrent disease; observational study; soft tissue neoplasms; soft tissue tumor; prospective; yttrium 90; pigmented villonodular synovitis; knee joint; knee disease; synovitis, pigmented villonodular; real-world; humans; human; male; female; article; synovectomy; pexidartinib; giant cell tumor of tendon sheath; diffuse-tenosynovial giant cell tumor (d-tgct); tgct observational platform project (topp); arthroscopic resection; diffuse tenosynovial giant cell tumor
Journal Title: Journal of Surgical Oncology
Volume: 126
Issue: 8
ISSN: 0022-4790
Publisher: Wiley Blackwell  
Date Published: 2022-12-15
Start Page: 1520
End Page: 1532
Language: English
DOI: 10.1002/jso.27067
PUBMED: 36006054
PROVIDER: scopus
PMCID: PMC9804718
DOI/URL:
Notes: Article -- Export Date: 1 December 2022 -- Source: Scopus
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  1. John H Healey
    547 Healey