Severity classification of Tenosynovial Giant Cell Tumours on MR imaging. (September 2018) - Mastboom, M.J.L.
Abstract: Aim: Current development of novel systemic agents requires identification and monitoring of extensive Tenosynovial Giant Cell Tumours (TGCT). This study defines TGCT extension on MR imaging to classify severity. Methods: In part one, six MR parameters were defined by field-experts to assess disease extension on MR images: type of TGCT, articular involvement, cartilage-covered bone invasion, and involvement of muscular/tendinous tissue, ligaments or neurovascular structures. Inter- and intra-rater agreement were calculated using 118 TGCT MR scans. In part two, the previously defined MR parameters were evaluated in 174 consecutive, not previously used, MR-scans. TGCT severity classification was established based on highest to lowest Hazard Ratios (HR) on first recurrence. Results: In part one, all MR parameters showed good inter- and intra-rater agreement (Kappa≥0.66). In part two, cartilage-covered bone invasion and neurovascular involvement were rarely appreciated (<13%) and therefore excluded for additional analyses. Univariate analyses for recurrent disease yielded positive associations for type of TGCT HR12.84(95%CI4.60–35.81), articular involvement HR6.00(95%CI2.14–16.80), muscular/tendinous tissue involvement HR3.50(95%CI1.75–7.01) and ligament-involvement HR4.59(95%CI2.23–9.46). With these, a TGCT severity classification was constructed with four distinct severity-stages. Recurrence free survival at 4 years (log rank p < 0.0001) was 94% in mild localized (n56, 1 recurrence), 88% in severe localized (n31, 3 recurrences), 59% in moderate diffuse (n32, 12 recurrences) and 36% in severe diffuse (n55, 33 recurrences). Conclusion: The proposed TGCT severity classification informs physicians and patients on disease extent and risk for recurrence after surgical treatment. Definition of the most severe subgroup attributes to a universal identification of eligible patients for systemic therapy or trials for novel agents. Highlights: TGCT describes a very heterogeneous entity with clear differences in clinical outcome related to recurrence free survival. A combination of four MR parameters, resulting in four TGCT severity subgroups, discriminates for TGCT severity on MR imaging. Definition of the most severe TGCT subgroup attributes to the identification of eligible patients for targeted therapy.