Multidisciplinary care in tenosynovial giant cell tumours.

Abstract

Tenosynovial giant cell tumour is a rare neoplastic condition of the joint that generally affects patients younger than 40 years. 1 Tap WD Wainberg ZA Antony SP et al. Structure-guided blockade of CSF1R kinase in tenosynovial giant-cell tumor. N Engl J Med. 2015; 373: 428-437 Crossref PubMed Scopus (325) Google Scholar It is not a malignancy per se, but for many, it is far from a benign disease and often causes substantial morbidity and disability due to pain, joint destruction, the need for repeated surgical intervention, and the use of analgesics and narcotics. Although not life-threatening, tenosynovial giant cell tumours can disrupt daily living and adversely alter an individual's life trajectory. Tenosynovial giant cell tumour is a complex disease with a wide breadth of clinical sequalae. Understanding of this disease has changed substantially in the past 10 years after causative genomic events were described. 2 West RB Rubin BP Millar MA et al. A landscape effect in tenosynovial giant-cell tumor from activation of CSF1 expression by a translocation in a minority of tumor cells. Proc Natl Acad Sci USA. 2006; 103: 690-695 Crossref PubMed Scopus (363) Google Scholar The discovery of these genomic events has allowed for the development and application of promising therapeutics targeting CSF1 signalling, which are further driving our knowledge base and greatly informing clinical practice. 1 Tap WD Wainberg ZA Antony SP et al. Structure-guided blockade of CSF1R kinase in tenosynovial giant-cell tumor. N Engl J Med. 2015; 373: 428-437 Crossref PubMed Scopus (325) Google Scholar , 3 Cassier PA Italiano A Gomez-Roca CA et al. CSF1R inhibition with emactuzumab in locally advanced diffuse-type tenosynovial giant cell tumours of the soft tissue: a dose-escalation and dose-expansion phase 1 study. Lancet Oncol. 2015; 16: 949-956 Summary Full Text Full Text PDF PubMed Scopus (236) Google Scholar The introduction of CSF1 receptor inhibitors came with the realisation that much needed to be learned about what patients with tenosynovial giant cell tumours go through and how the medical community could best serve them. This realisation prompted the medical and pharmaceutical community to partner with patients, patient advocacy, and patient support groups to learn more about the disease, to develop novel patient-reported outcome measures 4 Gelhorn HL Tong S McQuarrie K et al. Patient-reported symptoms of tenosynovial giant cell tumors. Clin Ther. 2016; 38: 778-793 Summary Full Text Full Text PDF PubMed Scopus (54) Google Scholar and potentially new imaging techniques, 1 Tap WD Wainberg ZA Antony SP et al. Structure-guided blockade of CSF1R kinase in tenosynovial giant-cell tumor. N Engl J Med. 2015; 373: 428-437 Crossref PubMed Scopus (325) Google Scholar and to review and scrutinise historical experiences. 5 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: 210-217 Summary Full Text Full Text PDF PubMed Scopus (79) Google Scholar This effort is paramount because the correct application of targeted therapies in tenosynovial giant cell tumours is not straightforward. Although active, many drugs have serious side-effects and associated financial costs. Much still needs to be learned about appropriate application, patient selection, timing of therapy, and length of use; decisions that need to be made in a multidisciplinary fashion, inclusive of the patient and their care team. Surgical outcomes of patients with diffuse-type tenosynovial giant-cell tumours: an international, retrospective, cohort studyThis study of patients with diffuse-type tenosynovial giant-cell tumour provides a comprehensive and up-to-date disease overview, assessing the clinical profile and management of the disease in multiple specialised referral centres. Surgical treatment of diffuse-type tenosynovial giant cell tumours is not a definitive treatment for every patient because it involves a high risk for local recurrent disease and a relatively high risk for postoperative complications. After surgical treatment in treatment-naive patients, risk factors for recurrent disease in individual patients were not identified in what we believe is the largest cohort to date. Full-Text PDF