title: Tailored Beta-catenin mutational approach in extra-abdominal sporadic desmoid tumor patients without therapeutic intervention.
abstract: The efficacy of the classical treatment modalities surgery and radiotherapy in the treatment of aggressive fibromatosis is presently disputed and there is a shift towards a more conservative approach. The aim of the present study is to objectify tumor growth in patients with extra-abdominal or abdominal wall aggressive fibromatosis, while adhering to a "watchful waiting" policy. Other objectives are to investigate quality of life and to identify factors associated with tumor growth, in particular the relation with the presence of a CTNNB1-gene mutation in the tumor.
  
  
  
  GRAFITI is a nationwide, multicenter, prospective registration trial. All patients with extra-abdominal or abdominal wall aggressive fibromatosis are eligible for inclusion in the study. Main exclusion criteria are: history of familiar adenomatous polyposis, severe pain, functional impairment, life/limb threating situations in case of progressive disease. Patients included in the study will be treated with a watchful waiting policy during a period of 5 years. Imaging studies with ultrasound and magnetic resonance imaging scan will be performed during follow-up to monitor possible growth: the first years every 3 months, the second year twice and the yearly. In addition patients will be asked to complete a quality of life questionnaire on specific follow-up moments. The primary endpoint is the rate of progression per year, defined by the Response Evaluation Criteria In Solid Tumors (RECIST). Secondary endpoints are quality of life and the rate of influence on tumor progression for several factors, such as CTNNB1-mutations, age and localization.
  
  
  
  This study will provide insight in tumor behavior, the effect on quality of life and clinicopathological factors predictive of tumor progression.
  
  
  
  The GRAFITI trial is registered in the Netherlands National Trial Register (NTR), number 4714 .
authors: Erasmus MC Cancer Institute, Rotterdam, The Netherlands. d.vanbroekhoven@erasmusmc.nl.
authors: Danique L M
authors: DL
authors: van Broekhoven
authors: Erasmus MC Cancer Institute, Rotterdam, The Netherlands.
authors: Dirk J
authors: DJ
authors: Grünhagenl
authors: University Medical Center Utrecht, Utrecht, The Netherlands.
authors: Thijs
authors: T
authors: van Dalen
authors: Netherlands Cancer Institute, Amsterdam, The Netherlands.
authors: Frits
authors: F
authors: van Coevorden
authors: Radboud University Medical Center, Nijmegen, The Netherlands.
authors: Han J
authors: HJ
authors: Bonenkamp
authors: University Medical Center Groningen, Groningen, The Netherlands.
authors: Lukas B
authors: LB
authors: Been
authors: Maastricht University Medical Center, Maastricht, The Netherlands.
authors: Marc H A
authors: MH
authors: Bemelmans
authors: Leiden University Medical Center, Leiden, The Netherlands.
authors: Sander D S
authors: SD
authors: Dijkstra
authors: Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
authors: Chiara
authors: C
authors: Colombo
authors: Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
authors: Alessandro
authors: A
authors: Gronchi
authors: Erasmus MC Cancer Institute, Rotterdam, The Netherlands.
authors: Cornelis
authors: C
authors: Verhoef
mesh_terms: D000008:Abdominal Neoplasms / Q000150:complications* / Q000235:genetics / Q000473:pathology*; D011125:Adenomatous Polyposis Coli / Q000150:complications* / Q000235:genetics / Q000473:pathology*; D000328:Adult; D000368:Aged; D004252:DNA Mutational Analysis; D018450:Disease Progression; D005260:Female; D018222:Desmoid Tumors / Q000150:complications* / Q000235:genetics / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009154:Mutation; D009426:Netherlands; D011788:Quality of Life; D012107:Research Design; D057832:Watchful Waiting; D051176:beta Catenin / Q000235:genetics
chemical_list: D051176:beta Catenin
keywords: Aggressive fibromatosis; Desmoid; Desmoid-type fibromatosis; Growth; Progression; Wait-and-see; Watchful waiting