title: Gastrointestinal stromal tumors of the esophagus: evaluation of a pooled case series regarding clinicopathological features and clinical outcome.
abstract: OBJECTIVE
  To elucidate diagnostic criteria, clinicopathological features and clinical outcome in patients with esophageal gastrointestinal stromal tumors (GIST), representing an extremely rare subform of GIST with an estimated incidence of about 0.1 to 0.3 per million people.
  
  
  METHODS
  Esophageal GIST cases from the Ulmer GIST registry consisting of 1077 cases were pooled with case reports and case series of esophageal GIST extracted from MEDLINE. Data were compared with those from 683 cases with gastric GIST from the Ulmer GIST registry.
  
  
  RESULTS
  In comparison to gastric GIST, esophageal GIST (n = 55) occurred significantly more frequent in men (p = 0.035) as well as in patients younger than 60 at diagnosis (p < 0.001). Primary tumor sizes were significantly larger (p < 0.001), thereby resulting more frequently in a high-risk classification (OR = 4.53, CI 95% 2.41-8.52, p < 0.001). The 5-year rates of disease-specific survival (DSS), disease-free survival (DFS), and overall survival (OS) were 50.9%, 65.3% and 48.3%, respectively. The prognosis of esophageal GIST was less favorable compared with gastric GIST (DSS: p < 0.001, HR = 0.158, 95% CI: 0.087-0.288; DFS: p = 0.023, HR 0.466, 95% CI: 0.241-0.901; OS p = 0.003, HR = 0.481, 95% CI: 0.294-0.785; univariate Cox model) after a median follow-up time of 28 months (range 1.9 to 202). Mutational analysis for KIT showed more frequently wild-type status in esophageal GIST (OR = 10.13, CI 95% 3.02-33.96, p < 0.001).
  
  
  CONCLUSIONS
  Esophageal GIST differ significantly from gastric GIST in respect to clinicopathological features and clinical outcome. To optimize treatment options further prospective data on patients with esophageal GIST are urgently warranted.
authors: Department of General and Visceral Surgery, University Hospital Ulm Germany.
authors: Simon
authors: S
authors: Lott
authors: Department of Internal Medicine, Alb-Fils-Kliniken Goeppingen, Germany.
authors: Michael
authors: M
authors: Schmieder
authors: Institute of Epidemiology and Medical Biometry, University of Ulm Germany.
authors: Benjamin
authors: B
authors: Mayer
authors: Department of General and Visceral Surgery, University Hospital Ulm Germany.
authors: Doris
authors: D
authors: Henne-Bruns
authors: Department of General and Visceral Surgery, University Hospital Ulm Germany.
authors: Uwe
authors: U
authors: Knippschild
authors: Institute of Pathology, University of Erlangen Germany.
authors: Abbas
authors: A
authors: Agaimy
authors: Dr Margarete Fischer-Bosch Institute of Clinical Pharmacology Stuttgart ; Department of Clinical Pharmacology, University Hospital Tuebingen Germany.
authors: Matthias
authors: M
authors: Schwab
authors: Department of General and Visceral Surgery, University Hospital Ulm Germany.
authors: Klaus
authors: K
authors: Kramer
keywords: GIST; esophagus; gastrointestinal stromal tumor; mutation analysis; outcome; prognosis
references: Diagn Cytopathol. 2007 Mar;35(3):167-70
references: 17415921
references: PLoS Med. 2007 Oct 16;4(10):e297
references: 17941715
references: Z Gastroenterol. 2007 Dec;45(12):1252-6
references: 18080227
references: Ann Thorac Surg. 2009 May;87(5):1569-71
references: 19379907
references: J Cardiothorac Surg. 2008 May 21;3:31
references: 18495011
references: Dis Esophagus. 2005;18(4):281-2
references: 16128787
references: Dis Esophagus. 2005;18(1):70-3
references: 15773848
references: Diagn Interv Radiol. 2010 Sep;16(3):217-20
references: 19838991
references: Arch Pathol Lab Med. 2007 Sep;131(9):1393-6
references: 17824795
references: Am J Surg Pathol. 2006 Apr;30(4):477-89
references: 16625094
references: World J Gastroenterol. 2011 Apr 28;17(16):2159-60
references: 21547139
references: World J Surg Oncol. 2008 Nov 05;6:116
references: 18986537
references: Am J Surg Pathol. 2005 Jan;29(1):52-68
references: 15613856
references: Saudi Med J. 2006 Aug;27(8):1236-9
references: 16883460
references: World J Gastroenterol. 2010 Sep 21;16(35):4374-6
references: 20845503
references: Hum Pathol. 2002 May;33(5):459-65
references: 12094370
references: Am J Surg Pathol. 2000 Feb;24(2):211-22
references: 10680889
references: World J Gastroenterol. 2013 Mar 28;19(12):2009-10
references: 23569350
references: Intern Med. 2010;49(8):781-5
references: 20424371
references: Clin Nucl Med. 2010 Jul;35(7):505-9
references: 20548142
references: Am J Pathol. 1998 May;152(5):1259-69
references: 9588894
references: J Laparoendosc Adv Surg Tech A. 2011 Sep;21(7):635-9
references: 21774702
references: Epidemiology. 2007 Nov;18(6):805-35
references: 18049195
references: Science. 1998 Jan 23;279(5350):577-80
references: 9438854
references: Surg Laparosc Endosc Percutan Tech. 2004 Apr;14(2):87-90
references: 15287607
references: Int J Surg Pathol. 2007 Oct;15(4):393-6
references: 17913949
references: Am J Gastroenterol. 2005 Jan;100(1):162-8
references: 15654796
references: Mod Pathol. 2008 Jan;21(1):46-53
references: 17917670
references: Eur J Surg Oncol. 2008 May;34(5):563-8
references: 17532173
references: Bull Cancer. 2010 Mar;97(3):E16-22
references: 20129875
references: Eur J Cardiothorac Surg. 2010 Aug;38(2):223-7
references: 20206541
references: Eur J Gastroenterol Hepatol. 2002 Apr;14(4):445-8
references: 11943962
references: Ann Intern Med. 2007 Oct 16;147(8):W163-94
references: 17938389
references: Cancer. 2005 Feb 15;103(4):821-9
references: 15648083
references: Ann Thorac Surg. 2007 Nov;84(5):1717-23
references: 17954092
references: J Natl Compr Canc Netw. 2010 Apr;8 Suppl 2:S1-41; quiz S42-4
references: 20457867
references: Ann Oncol. 2012 May;23(5):1335-40
references: 21994214
references: Ann Diagn Pathol. 2005 Feb;9(1):49-53
references: 15692952
references: Int Surg. 2008 Jul-Aug;93(4):209-13
references: 19731855
references: Dig Dis Sci. 2007 Sep;52(9):2249-53
references: 17420941
references: Arch Surg. 2009 Jul;144(7):670-8
references: 19620548
references: Dtsch Med Wochenschr. 2005 Oct 21;130(42):2380-3
references: 16235145
references: Hum Pathol. 1999 Oct;30(10):1213-20
references: 10534170
references: Ann Oncol. 2012 Oct;23 Suppl 7:vii49-55
references: 22997454
references: Am J Surg Pathol. 2005 Oct;29(10):1373-81
references: 16160481
references: Am J Clin Pathol. 2003 May;119(5):703-8
references: 12760289