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Published in: Digestive Diseases and Sciences 9/2007

01-09-2007 | Original Paper

Clinical Study on Gastrointestinal Stromal Tumors (GIST) in Iceland, 1990–2003

Authors: Geir Tryggvason, Þórarinn Kristmundsson, Kjartan Örvar, Jón G. Jónasson, Magnús K. Magnússon, Hjörtur G. Gíslason

Published in: Digestive Diseases and Sciences | Issue 9/2007

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Abstract

This is a whole population-based study on clinical symptoms, surgical treatment, and outcome of GIST. All mesenchymal tumors in the digestive tract diagnosed from 1990 to 2003 were identified. All reports were reviewed, all tumors were stained with antibodies to c-kit, and the diagnosis of GIST was confirmed. Clinical, pathological, treatment, and outcome data were analyzed. The study included 53 patients with GIST. The mean age at diagnosis was 65.8±13.6 years (SD). Tumor distribution included 62% in the upper, 32% in the middle, and 6% in the lower digestive tract. Mean tumor size was 4.9±4.4 cm (SD). Gastrointestinal (GI) bleeding was the main symptom in 53% (20/38) of symptomatic cases; most presented with acute gastrointestinal bleeding. Complete surgical resection was performed in 87% (46/53) of patients. Eight of the 53 tumors (15.1%) metastasized, 7 of which were nongastric. The disease-specific death rate at 5 years was 85%, and 5-year survival after complete resection was 64.1%. We conclude that GISTs are often found incidentally but GI bleeding is the most common presentation. Five-year survival is better than previously reported and gastric GIST seems to be more benign than nongastric. GIST seems to metastasize mainly intra-abdominally.
Literature
1.
go back to reference Kindblom L, Remotti H, Aldenborg F, Meis-Kindblom J (1998) Gastrointestinal pacemaker cell tumor (GIPACT): gastrointestinal stromal tumors show phenotypic characteristics of the interstitial cells of Cajal. Am J Pathol 152(5):1259–1269PubMed Kindblom L, Remotti H, Aldenborg F, Meis-Kindblom J (1998) Gastrointestinal pacemaker cell tumor (GIPACT): gastrointestinal stromal tumors show phenotypic characteristics of the interstitial cells of Cajal. Am J Pathol 152(5):1259–1269PubMed
2.
go back to reference Hirota S, Isozaki H, Moriyama Y, Hashimoto K, Nishida T, Ishiguro S, Kawano K, Hanada M, Kurata A, Takeda M, Muhammad Tunio G, Matsuzawa Y, Kanakura Y, Shinomura Y, Kitamura Y (1998) gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science 279:577–580PubMedCrossRef Hirota S, Isozaki H, Moriyama Y, Hashimoto K, Nishida T, Ishiguro S, Kawano K, Hanada M, Kurata A, Takeda M, Muhammad Tunio G, Matsuzawa Y, Kanakura Y, Shinomura Y, Kitamura Y (1998) gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science 279:577–580PubMedCrossRef
3.
go back to reference Rubin BP, Singer S, Tsao C, Duensing A, Lux ML, Ruiz R, Hibbard MK, Chen C-J, Xiao S, Tuveson D, Demetri GD, Fletcher CDM, Fletcher JA (2001) KIT activation is a ubiquitous feature of gastrointestinal stromal tumors. Cancer Res 61:8118–8121PubMed Rubin BP, Singer S, Tsao C, Duensing A, Lux ML, Ruiz R, Hibbard MK, Chen C-J, Xiao S, Tuveson D, Demetri GD, Fletcher CDM, Fletcher JA (2001) KIT activation is a ubiquitous feature of gastrointestinal stromal tumors. Cancer Res 61:8118–8121PubMed
4.
go back to reference Demetri GD, von Mehren M, Blanke CD, Van den Abbeele AD, Eisenberg B, Roberts PJ, Heinrich MC, Tuveson DA, Singer S, Janicek M, Fletcher JA, Silverman SG, Silberman SL, Capdeville R, Kiese B, Peng B, Dimitrijevic S, Druker BJ, Corless C, Fletcher CD, Joensuu H (2002) Efficacy and safety of imatinib mesylate in advanced gastrointestinal stromal tumors. N Engl J Med 347:472–480PubMedCrossRef Demetri GD, von Mehren M, Blanke CD, Van den Abbeele AD, Eisenberg B, Roberts PJ, Heinrich MC, Tuveson DA, Singer S, Janicek M, Fletcher JA, Silverman SG, Silberman SL, Capdeville R, Kiese B, Peng B, Dimitrijevic S, Druker BJ, Corless C, Fletcher CD, Joensuu H (2002) Efficacy and safety of imatinib mesylate in advanced gastrointestinal stromal tumors. N Engl J Med 347:472–480PubMedCrossRef
5.
go back to reference Tryggvason G, Gislason HG, Magnusson MK, Jonasson JG (2005) Gastrointestinal stromal tumors in Iceland 1990–2003: The Icelandic GIST Study, population based incidence and pathologic risk stratification study. Int J Cancer 117(1):289–293PubMedCrossRef Tryggvason G, Gislason HG, Magnusson MK, Jonasson JG (2005) Gastrointestinal stromal tumors in Iceland 1990–2003: The Icelandic GIST Study, population based incidence and pathologic risk stratification study. Int J Cancer 117(1):289–293PubMedCrossRef
6.
go back to reference Fletcher CDM, Berman JJ, Corless CL, Gorstein F, Lasota J, Longley BJ, Miettinen M, O’Leary TJ, Remotti H, Rubin BP, Shmookler B, Sobin LH, Weiss SW (2002) Diagnosis of gastrointestinal stromal tumors: A consensus approach. Hum Pathol 33:459–465PubMedCrossRef Fletcher CDM, Berman JJ, Corless CL, Gorstein F, Lasota J, Longley BJ, Miettinen M, O’Leary TJ, Remotti H, Rubin BP, Shmookler B, Sobin LH, Weiss SW (2002) Diagnosis of gastrointestinal stromal tumors: A consensus approach. Hum Pathol 33:459–465PubMedCrossRef
7.
go back to reference Dematteo RP, Lewis JJ, Leung D, Mudan SS, Woodruff JM, Brennan MF (2000) Two hundred gastrointestinal stromal tumors: recurrence patterns and prognostic factors for survival. Ann Surg 231(1):51–58PubMedCrossRef Dematteo RP, Lewis JJ, Leung D, Mudan SS, Woodruff JM, Brennan MF (2000) Two hundred gastrointestinal stromal tumors: recurrence patterns and prognostic factors for survival. Ann Surg 231(1):51–58PubMedCrossRef
8.
go back to reference Crosby JA, Catton CN, Davis A, Couture J, O’Sullivan B, Kandel R, Swallow CJ (2001) Malignant gastrointestinal stromal tumors of the small intestine: A review of 50 cases from a prospective database. Ann Surg Oncol 8(1):50–59PubMedCrossRef Crosby JA, Catton CN, Davis A, Couture J, O’Sullivan B, Kandel R, Swallow CJ (2001) Malignant gastrointestinal stromal tumors of the small intestine: A review of 50 cases from a prospective database. Ann Surg Oncol 8(1):50–59PubMedCrossRef
9.
go back to reference Pierie J-PEN, Choudry U, Muzikansky A, Yong Yeap B, Souba WW, Ott MJ (2001) The effect of surgery and grade on outcome of gastrointestinal stromal tumors. Arch Surg 136:383–389PubMedCrossRef Pierie J-PEN, Choudry U, Muzikansky A, Yong Yeap B, Souba WW, Ott MJ (2001) The effect of surgery and grade on outcome of gastrointestinal stromal tumors. Arch Surg 136:383–389PubMedCrossRef
10.
go back to reference Ng EH, Pollock RE, Munsell MF, Atkinson EN, Romsdahl MM (1992) Prognostic factors influencing survival in gastrointestinal leiomyosarcomas. Implications for surgical management and staging. Ann Surg 215(1):68–77PubMedCrossRef Ng EH, Pollock RE, Munsell MF, Atkinson EN, Romsdahl MM (1992) Prognostic factors influencing survival in gastrointestinal leiomyosarcomas. Implications for surgical management and staging. Ann Surg 215(1):68–77PubMedCrossRef
11.
go back to reference Emory TS, Sobin LH, Lukes L, Lee DH, O’Leary TJ (1999) Prognosis of gastrointestinal smooth-muscle (stromal) tumors: Dependence on anatomic site. Am J Surg Pathol 23(1):82–87PubMedCrossRef Emory TS, Sobin LH, Lukes L, Lee DH, O’Leary TJ (1999) Prognosis of gastrointestinal smooth-muscle (stromal) tumors: Dependence on anatomic site. Am J Surg Pathol 23(1):82–87PubMedCrossRef
12.
go back to reference Nilsson B, Bumming P, Kindblom JM, Odén A, Dortok A, Gustavsson B, Sablinska K, Kindblom L-G (2005) Gastrointestinal stromal tumors: The incidence, prevalence, clinical course and prognostication in the preimatinib mesylate era. Cancer 103:821–829PubMedCrossRef Nilsson B, Bumming P, Kindblom JM, Odén A, Dortok A, Gustavsson B, Sablinska K, Kindblom L-G (2005) Gastrointestinal stromal tumors: The incidence, prevalence, clinical course and prognostication in the preimatinib mesylate era. Cancer 103:821–829PubMedCrossRef
13.
go back to reference Antonescu CR, Sommer G, Sarran L, Tschernyavsky SJ, Riedel E, Woodruff JM, Robson M, Maki R, Brennan MF, Ladanyi M, Dematteo RP, Besmer P (2003) Association of KIT exon 9 mutations with nongastric primary site and aggressive behavior: KIT mutation analysis and clinical correlates of 120 gastrointestinal stromal tumors. Clin Cancer Res 9:3329–3337PubMed Antonescu CR, Sommer G, Sarran L, Tschernyavsky SJ, Riedel E, Woodruff JM, Robson M, Maki R, Brennan MF, Ladanyi M, Dematteo RP, Besmer P (2003) Association of KIT exon 9 mutations with nongastric primary site and aggressive behavior: KIT mutation analysis and clinical correlates of 120 gastrointestinal stromal tumors. Clin Cancer Res 9:3329–3337PubMed
14.
go back to reference Lasota J, Dansonka-Miesakowska A, Sobin LH, Miettinen M (2004) A great majority of GISTs with PDGFRA mutations represent gastric tumors of low or no malignant potential. Lab Invest 84:874–883PubMedCrossRef Lasota J, Dansonka-Miesakowska A, Sobin LH, Miettinen M (2004) A great majority of GISTs with PDGFRA mutations represent gastric tumors of low or no malignant potential. Lab Invest 84:874–883PubMedCrossRef
15.
go back to reference McGrath PC, Neifeld JP, Lawrence WJ, Kay S, Horsley JS, Parker GA (1987) Gastrointestinal sarcomas. Analysis of prognostic factors. Ann Surg 206(6):706–710PubMedCrossRef McGrath PC, Neifeld JP, Lawrence WJ, Kay S, Horsley JS, Parker GA (1987) Gastrointestinal sarcomas. Analysis of prognostic factors. Ann Surg 206(6):706–710PubMedCrossRef
Metadata
Title
Clinical Study on Gastrointestinal Stromal Tumors (GIST) in Iceland, 1990–2003
Authors
Geir Tryggvason
Þórarinn Kristmundsson
Kjartan Örvar
Jón G. Jónasson
Magnús K. Magnússon
Hjörtur G. Gíslason
Publication date
01-09-2007
Publisher
Springer US
Published in
Digestive Diseases and Sciences / Issue 9/2007
Print ISSN: 0163-2116
Electronic ISSN: 1573-2568
DOI
https://doi.org/10.1007/s10620-006-9248-4

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