Patient-reported Symptoms of Tenosynovial Giant Cell Tumors.

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Abstract

[Indexed for MEDLINE] Conflict of interest statement: CONFLICTS OF INTEREST G. Maclaine and X. Ye are employees of Daiichi Sankyo Development Ltd, which provided financial support for this research. S. Tong and P. Lin are employees of Plexxikon Inc, which provided financial support for this research. J.H. Healey is a paid consultant of Daiichi Sankyo Development Ltd. H. Hsu is a paid consultant of Plexxikon Inc. S. Anthony is a paid member of the Paradigm Medical Evidence Team and a paid consultant of Zymeworks Biopharmaceuticals. W. Taq is a paid consultant of Plexxikon and Daiichi Sankyo Development Ltd. The institutions of S. Bukata, D. Von Hoff, and V. Keedy received funding from Plexxikon for conducting the study for this work. D. Von Hoff is a paid consultant of Five Prime Therapeutics. H. Gelhorn, K. McQuarrie, C. Vernon, J. Hanlon, W. Lenderking, and R. Speck participated in this project as employees of Evidera, a company which performs work for hire for multiple pharmaceutical and device companies in outcomes research. K. McQuarrie is currently employed by Janssen. A. Wagner, A. Singh, C. Becerra, J. Hanlon, and R. Lackman have no conflicts of interest related to this work to report. 3. J Clin Oncol. 2018 Jan 10;36(2):202-209. doi: 10.1200/JCO.2017.75.8482. Epub 2017 Dec 8. Locally Aggressive Connective Tissue Tumors. Gounder MM(1), Thomas DM(1), Tap WD(1). Author information: (1)Mrinal M. Gounder and William D. Tap, Memorial Sloan Kettering Cancer Center and Weil Cornell Medical School, New York, NY; and David M. Thomas, Garvan Institute of Medical Research, Darlinghurst, Australia. Comment in J Clin Oncol. 2018 Jun 1;36(16):1643-1644. doi: 10.1200/JCO.2018.78.0478. J Clin Oncol. 2018 Jun 1;36(16):1642-1643. doi: 10.1200/JCO.2018.77.9819. In this review, we highlight the complexities of the natural history, biology, and clinical management of three intermediate connective tissue tumors: desmoid tumor (DT) or aggressive fibromatosis, tenosynovial giant cell tumor (TGCT) or diffuse-type pigmented villonodular synovitis (dtPVNS), and giant cell tumor of bone (GCTB). Intermediate histologies include tumors of both soft tissue and bone origin and are locally aggressive and rarely metastatic. Some common aspects to these tumors are that they can be locally infiltrative and/or impinge on critical organs, which leads to disfigurement, pain, loss of function and mobility, neurovascular compromise, and occasionally life-threatening consequences, such as mesenteric, bowel, ureteral, and/or bladder obstruction. DT, PVNS, and GCTB have few and recurrent molecular aberrations but, paradoxically, can have variable natural histories. A multidisciplinary approach is recommended for optimal management. In DT and PVNS, a course of observation may be appropriate, and any intervention should be guided by symptoms and/or disease progression. A surgical approach should take into consideration the infiltrative nature, difficulty in obtaining wide margins, high recurrence rates, acute and chronic surgical morbidities, and impact on quality of life. There are similar concerns with radiation, which especially relate to optimal field and transformation to high-grade radiation-associated sarcomas. Systemic therapies must be considered carefully in light of acute and chronic toxicities. Although standard and novel therapies are promising, many unanswered questions, such as duration of therapy and optimal end points to evaluate efficacy of drugs in clinical practice and trials, exist. Predictive biomarkers and novel clinical trial end points, such as volumetric measurement, magnetic resonance imaging T2 weighted mapping, nuclear imaging, and patient-reported outcomes, are in development and will require validation in prospective trials. DOI: 10.1200/JCO.2017.75.8482 PMCID: PMC6804876