Journal
ANTI-CANCER DRUGS
Volume 31, Issue 1, Pages 80-84Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/CAD.0000000000000844
Keywords
chemotherapy; imatinib; malignant tenosynovial giant cell tumour; pazopanib; pigmented villonodular synovitis; tenosynovial giant cell tumour
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Tenosynovial giant cell tumour (TGCT) is a group of rare soft tissues neoplasia affecting synovial joints, bursae and tendon sheaths and is classified as localized type or diffuse type. The diffuse type (TGCT-D), also known as 'pigmented villonodular (teno)synovitis' is characterized by local aggressivity, with invasion and destruction of adjacent soft-tissue structures, and high local recurrence rate. Radical surgery remains the standard therapy while adjuvant radiotherapy may help to control local spread. Malignant TGCT is characterized by high rate of local recurrences and distant metastasis. Few cases of malignant TGCT and very few evidences on systemic therapies are described in the literature, so, to date, no systemic treatment is approved for this rare disease. We report the case of a malignant TGCT patient treated with many different systemic therapies, including chemotherapy and tyrosine-kinase inhibitors, and performed a review of the literature on the systemic treatment options of this rare tumour. Copyright (C) 2019 The Author(s). Published by Wolters Kluwer Health, Inc.
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