title: Clinical presentation, treatment and outcome of anaplastic thyroid carcinoma: results of a multicenter study in Germany.
abstract: Anaplastic thyroid carcinoma (ATC) is an orphan disease and confers a dismal prognosis. Standard treatment is not established.
  
  
  
  The aim of this study is to describe clinical characteristics, current treatment regimens and outcome of ATC and to identify clinical prognostic markers and treatment factors associated with improved prognosis.
  
  
  
  Retrospective cohort study at five German tertiary care centers.
  
  
  
  Totally 100 ATC patients diagnosed between 2000 and 2015 were included in the analysis. Disease-specific overall survival (OS) was compared with the Kaplan-Meier method and log-rank test; Cox proportional hazard model was used to identify risk factors.
  
  
  
  The 6-month, 1-year and 5-year disease-specific OS rates were 37, 28 and 5%, respectively. Stage-dependent OS at 6 months was 78, 54 and 18% for stage IVA, B and C, respectively. 29% patients survived >1 year. Multivariate analysis of OS identified age ≥70 years, incomplete local resection status and the presence of distant metastasis as significant risk factors associated with shorter survival. Radical surgery (hazard ratio [HR] 2.20, 95% confidence interval (CI) 1.19-4.09, P = 0.012), external beam radiation therapy (EBRT) ≥40 Gy (HR = 0.34, 0.15-0.76, P = 0.008) and any kind of chemotherapy (CTX) (HR = 11.64, 2.42-60.39, P = 0.003) were associated with longer survival in multivariate analyses adjusted for age and tumor stage. A multimodal treatment regimen was significantly associated with a survival benefit (HR = 1.04, 1.01-1.08, P < 0.0001) only in IVC patients.
  
  
  
  Disease-specific OS is still poor in ATC. Treatment factors associated with improved OS provide a rationale to devise treatment pathways for routine care. Collaborative research structures should be aimed to advance treatment of ATC.
authors.affiliation: Department of Internal Medicine IDivision of Endocrinology and Diabetes, University of Würzburg, University Hospital Würzburg, Würzburg, Germany.
authors.forename: Julia
authors.initials: J
authors.lastname: Wendler
authors.affiliation: Department of Internal Medicine IDivision of Endocrinology and Diabetes, University of Würzburg, University Hospital Würzburg, Würzburg, Germany Kroiss_M@ukw.de vera.tiedje@uk-essen.de.
authors.forename: Matthias
authors.initials: M
authors.lastname: Kroiss
authors.affiliation: Department of Internal Medicine IIUniversity Hospital Munich, Ludwig-Maximilians-Universität Munich, München, Germany.
authors.forename: Katja
authors.initials: K
authors.lastname: Gast
authors.affiliation: Department of Nuclear MedicineCentral Hospital Augsburg, Augsburg, Germany.
authors.forename: Michael C
authors.initials: MC
authors.lastname: Kreissl
authors.affiliation: Division for Specific EndocrinologyUniversity Hospital Düsseldorf, Düsseldorf, Germany.
authors.forename: Stephanie
authors.initials: S
authors.lastname: Allelein
authors.affiliation: Department of Internal Medicine IVUniversity Hospital Munich, Ludwig-Maximilians-Universität München, München, Germany.
authors.forename: Urs
authors.initials: U
authors.lastname: Lichtenauer
authors.affiliation: Technical University MunichKlinikum rechts der Isar, Institute for Medical Statistics and Epidemiology, München, Germany.
authors.forename: Rainer
authors.initials: R
authors.lastname: Blaser
authors.affiliation: Department of Internal Medicine IIUniversity Hospital Munich, Ludwig-Maximilians-Universität Munich, München, Germany.
authors.forename: Christine
authors.initials: C
authors.lastname: Spitzweg
authors.affiliation: University of WürzburgComprehensive Cancer Center, Würzburg, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Fassnacht
authors.affiliation: Department of Internal Medicine IVUniversity Hospital Munich, Ludwig-Maximilians-Universität München, München, Germany.
authors.forename: Matthias
authors.initials: M
authors.lastname: Schott
authors.affiliation: Department of Endocrinology and MetabolismUniversity Hospital Essen, University Duisburg-Essen, Essen, Germany.
authors.forename: Dagmar
authors.identifier: 0000-0003-0357-4192
authors.initials: D
authors.lastname: Führer
authors.affiliation: Department of Endocrinology and MetabolismUniversity Hospital Essen, University Duisburg-Essen, Essen, Germany Kroiss_M@ukw.de vera.tiedje@uk-essen.de.
authors.forename: Vera
authors.initials: V
authors.lastname: Tiedje
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000970:Antineoplastic Agents / Q000008:administration & dosage; D015331:Cohort Studies; D005260:Female; D005858:Germany / Q000453:epidemiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging / Q000401:mortality / Q000639:trends; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D065646:Thyroid Carcinoma, Anaplastic / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D013964:Thyroid Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D013965:Thyroidectomy / Q000401:mortality / Q000639:trends; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents