Highlights
- •Although benign, diffuse-type tenosynovial giant cell tumour (D-TGCT) can cause significant pain and impaired quality of life.
- •Surgery is the mainstay of treatment for D-TGCT, yet relapse is common.
- •Current treatments include surgery, external beam radiotherapy, radiosynovectomy.
- •New therapeutic strategies include immunotherapy and targeted therapy.
Abstract
At present, the optimal treatment strategy in patients with diffuse-type tenosynovial
giant cell tumour (D-TGCT) is unclear. The purpose of this review was to describe
current treatment options, and to highlight recent developments in the knowledge of
the molecular pathogenesis of D-TGCT as well as related therapeutic implications.
Epidemiology, clinical features, and the pathogenesis of D-TGCT and the most widely
used treatment modalities are described. D-TGCT is a benign clonal neoplastic proliferation
arising from the synovium. Patients are often symptomatic and require multiple surgical
procedures during their lifetime. Currently, surgery is the main treatment for patients
with D-TGCT, with relapse rates ranging from 14% to 55%. Radiosynovectomy and external
beam radiotherapy have been used in combination with surgical excision or as single
modalities. The finding that D-TGCT cells overexpress colony-stimulating factor 1
(CSF1), resulting in recruitment of CSF1 receptor (CSF1R)-bearing macrophages that
are polyclonal and make up the bulk of the tumour, has led to clinical trials with
CSF1R inhibitors. These inhibitors include small molecules such as imatinib, nilotinib,
PLX3397, and the monoclonal antibody RG7155. In conclusion, D-TGCT impairs patients’
quality of life significantly. The evidence that the pathogenetic loop of D-TGCT can
be inhibited could potentially change the therapeutic armamentarium for this condition.
Clinical trials of agents that target D-TGCT are currently ongoing. In the meantime,
international registries should be activated in order to provide useful information
on this relatively rare tumour.
Keywords
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Article info
Publication history
Published online: June 04, 2016
Accepted:
April 26,
2016
Received in revised form:
April 18,
2016
Received:
November 17,
2015
Identification
Copyright
© 2016 Elsevier Ltd. All rights reserved.
