Abstract

AimCurrent 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. MethodsIn 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. ResultsIn 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). ConclusionThe 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

  • Tenosynovial Giant Cell Tumour (TGCT) affecting large joints is an orphan, mono-articular, potentially locally aggressive disease with high recurrence rates

  • Using case discussions in expert meetings with two dedicated musculoskeletal radiologists and three oncological orthopaedic surgeons, six magnetic resonance (MR) parameters were selected in relation to anatomical or surgical landmarks

  • Since inter- and intra-rater agreements were good [5] for these six MR features, all parameters were considered viable to use for Tenosynovial Giant Cell Tumours (TGCT) subgroup analyses

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Summary

Introduction

Tenosynovial Giant Cell Tumour (TGCT) affecting large joints is an orphan, mono-articular, potentially locally aggressive disease with high recurrence rates. According to the 2013 WHO classification of tumours of soft tissue and bone, at the base of growth pattern, a radiological distinction is made between single nodule (localized-TGCT) and multiple lesions (diffuse-TGCT). These types differ in their clinical presentation, response to treatment and prognosis, but histologically, they seem identical [6,7,8,16]. Reported recurrence rates of diffuse-TGCT following open synovectomy are 14% up till 67% and after arthroscopic synovectomy 40% up till 92% [26]. Frequently requiring multiple, sometimes mutilating operations, may result in total joint

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