Abstract
Anaplastic thyroid carcinoma (ATC) is one of the most lethal neoplasms in humans, with a disease-specific mortality approaching 100%. ATC is uncommon and represents only 1-2% of all thyroid carcinomas. The aggressive nature and rarity of this disease make it difficult to improve the quality of evidence with regard to ATC. To date, most existing knowledge about ATC is derived from single-institution studies with limited numbers of cohorts and short-term follow-up. To obtain further insights into this “orphan disease”, we established the ATC Research Consortium of Japan (ATCCJ) in January 2009 as a multi-center registry that accumulates, analyzes and reviews all information on ATC available in Japan. As of July 2011, the ATCCJ database includes 721 cases with ATC from 38 institutions. Using the database, members of the ATCCJ have conducted several retrospective studies, including prognostic factors, therapeutic strategies, characteristic calcification patterns, pathologic features, implications of super-radical surgery for ATC and characteristics of incidental ATC. Moreover, investigator-initiated prospective trials using weekly-paclitaxel have been conducted since May 2012 as a multi--center collaboration to identify details of beneficial treatment, with the ultimate purpose of improving survival for patients with ATC.
