Background and Aims: Gastrointestinal stromal tumors (GISTs) are rare malignancies but the most common mesenchymal tumors of the digestive tract. Recent advances in diagnostic imaging and an increasing incidence will confront us more frequently with stromal tumors. This single center study aimed to characterize GIST patients in terms of tumor location, clinical presentation, metastasis formation, as well as associated secondary malignancies. Methods: In a retrospective study, 104 patients with a histologically confirmed diagnosis of GIST, collected between 1993 and 2011, were characterized for several clinical features. Results: The most common GIST location was the stomach (67.6%) followed by the small intestine (16.2%). Gastrointestinal bleeding (55.8%) and abdominal pain (38.5%) were the most frequently reported symptoms whereas about one-third of patients remained clinically asymptomatic (31.6%); 14.4% of patients had either synchronous or metachronous metastases and there was a significant prevalence also in the low risk group. The proportion of secondary malignant associated neoplasms was 31% in our GIST cohort, among which gastrointestinal, genitourinary tumors, and breast cancer were the most prevalent. Conclusion: There was a considerable risk for metastasis formation and the development of secondary neoplasias that should encourage discussion about the appropriate surveillance strategy after surgery for GIST.

1.
Pandurengan RK, Dumont AG, Araujo DM, Ludwig JA, Ravi V, Patel S, Garber J, Benjamin RS, Strom SS, Trent JC: Survival of patients with multiple primary malignancies: A study of 783 patients with gastrointestinal stromal tumor. Ann Oncol 2010; 21: 2107–2111.
2.
Min KW, Leabu M: Interstitial cells of cajal (icc) and gastrointestinal stromal tumor (gist): facts, speculations, and myths. J Cell Mol Med 2006; 10: 995–1013.
3.
Miettinen M, Lasota J: Gastrointestinal stromal tumors: Review on morphology, molecular pathology, prognosis, and differential diagnosis. Arch Pathol Lab Med 2006; 130: 1466–1478.
4.
Esposito I, Schlitter AM: Pathology of neuroendocrine neoplasias (nen) und gastrointestinal stromal tumors (gist) (article in german). Der Gastroenterologe 2015; 10: 394–409.
5.
Hirota S, Isozaki K, 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: Gain-of-function ­mutations of c-kit in human gastrointestinal stromal tumors. Science 1998; 279: 577–580.
6.
Group ESESNW: Gastrointestinal stromal tumours: Esmo clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol 2014; 25(suppl 3):iii21–iii26.
7.
Goettsch WG, Bos SD, Breekveldt-Postma N, Casparie M, Herings RM, Hogendoorn PC: Incidence of gastrointestinal stromal tumours is underestimated: results of a nation-wide study. Eur J Cancer 2005; 41: 2868–2872.
8.
Tran T, Davila JA, El-Serag HB: The epidemiology of malignant gastrointestinal stromal tumors: an analysis of 1,458 cases from 1992 to 2000. Am J Gastroenterol 2005; 100: 162–168.
9.
Joensuu H: Gastrointestinal stromal tumor (gist). Ann Oncol 2006; 17(suppl 10):x280–x286.
10.
Fletcher CD, Berman JJ, Corless C, Gorstein F, Lasota J, Longley BJ, Miettinen M, O’Leary TJ, Remotti H, Rubin BP, Shmookler B, Sobin LH, Weiss SW: Diagnosis of gastrointestinal stromal tumors: a consensus approach. Hum Pathol 2002; 33: 459–465.
11.
Joensuu H: Risk stratification of patients diagnosed with gastrointestinal stromal tumor. Hum Pathol 2008; 39: 1411–1419.
12.
TNM Classification of Malignant Tumors, (ed 7). Sobin LH (Ed.), Gospodarowicz MK (Ed.), Wittekind C (Ed.), Wiley-Blackwell, 2009.
13.
Miettinen M, Lasota J: Gastrointestinal stromal tumors: pathology and prognosis at different sites. Semin Diagn Pathol 2006; 23: 70–83.
14.
Joensuu H, Hohenberger P, Corless CL: Gastrointestinal stromal tumour. Lancet 2013; 382: 973–983.
15.
Yao KA, Talamonti MS, Langella RL, Schindler NM, Rao S, Small W Jr, Joehl RJ: Primary gastrointestinal sarcomas: analysis of prognostic factors and results of surgical management. Surgery 2000; 128: 604–612.
16.
Joensuu H, Eriksson M, Sundby Hall K, Hartmann JT, Pink D, Schutte J, Ramadori G, Hohenberger P, Duyster J, Al-Batran SE, Schlemmer M, Bauer S, Wardelmann E, Sarlomo-Rikala M, Nilsson B, Sihto H, Monge OR, Bono P, Kallio R, Vehtari A, Leinonen M, Alvegard T, Reichardt P: One vs three years of adjuvant imatinib for operable gastrointestinal stromal tumor: a randomized trial. JAMA 2012; 307: 1265–1272.
17.
Demetri GD, van Oosterom AT, Garrett CR, Blackstein ME, Shah MH, Verweij J, McArthur G, Judson IR, Heinrich MC, Morgan JA, Desai J, Fletcher CD, George S, Bello CL, Huang X, Baum CM, Casali PG: Efficacy and safety of sunitinib in patients with advanced gastrointestinal stromal tumour after failure of imatinib: a randomised controlled trial. Lancet 2006; 368: 1329–1338.
18.
Demetri GD, Reichardt P, Kang YK, Blay JY, Rutkowski P, Gelderblom H, Hohenberger P, Leahy M, von Mehren M, Joensuu H, Badalamenti G, Blackstein M, Le Cesne A, Schoffski P, Maki RG, Bauer S, Nguyen BB, Xu J, Nishida T, Chung J, Kappeler C, Kuss I, Laurent D, Casali PG; GRID study investigators: Efficacy and safety of regorafenib for advanced gastrointestinal stromal tumours after failure of imatinib and sunitinib (grid): an international, multicentre, randomised, placebo-controlled, phase 3 trial. Lancet 2013; 381: 295–302.
19.
Beham AW, Schaefer IM, Schuler P, Cameron S, Ghadimi BM: Gastrointestinal stromal tumors. Int J Colorectal Dis 2012; 27: 689–700.
20.
Liu YJ, Yang Z, Hao LS, Xia L, Jia QB, Wu XT: Synchronous incidental gastrointestinal stromal and epithelial malignant tumors. World J Gastroenterol 2009; 15: 2027–2031.
21.
Caterino S, Lorenzon L, Petrucciani N, Iannicelli E, Pilozzi E, Romiti A, Cavallini M, Ziparo V: Gastrointestinal stromal tumors: correlation between symptoms at presentation, tumor location and prognostic factors in 47 consecutive patients. World J Surg Oncol 2011; 9: 13.
22.
Miettinen M: Gastrointestinal stromal tumors: an immunohistochemical study of cellular differentiation. Am J Clin Pathol 1988; 89: 601–610.
23.
Amt MVS: Veröffentlichungen – Statistisches Amt Mecklenburg-Vorpommern, 2015.
24.
Athey BD, Braxenthaler M, Haas M, Guo Y: Transmart: an open source and community-driven informatics and data sharing platform for clinical and translational research. AMIA Jt Summits Transl Sci Proc 2013; 2013: 6–8.
25.
Watson GA, Kelly D, Melland-Smith M, Gleeson J, McEntee G, Kelly CM, McCaffrey JA: Get the gist? An overview of gastrointestinal stromal tumours. Ir J Med Sci 2016; 185: 319–326.
26.
Nilsson B, Bumming P, Meis-Kindblom JM, Oden A, Dortok A, Gustavsson B, Sablinska K, Kindblom LG: Gastrointestinal stromal tumors: the incidence, prevalence, clinical course, and prognostication in the preimatinib mesylate era – a population-based study in western sweden. Cancer 2005; 103: 821–829.
27.
Tryggvason G, Gislason HG, Magnusson MK, Jonasson JG: Gastrointestinal stromal tumors in iceland, 1990–2003: the icelandic gist study, a population-based incidence and pathologic risk stratification study. Int J Cancer 2005; 117: 289–293.
28.
Antonescu CR, Sommer G, Sarran L, Tschernyavsky SJ, Riedel E, Woodruff JM, Robson M, Maki R, Brennan MF, Ladanyi M, DeMatteo RP, Besmer P: 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 2003; 9: 3329–3337.
29.
DeMatteo RP, Lewis JJ, Leung D, Mudan SS, Woodruff JM, Brennan MF: Two hundred gastrointestinal stromal tumors: recurrence patterns and prognostic factors for survival. Ann Surg 2000; 231: 51–58.
30.
Miettinen M, Sarlomo-Rikala M, Sobin LH, Lasota J: Gastrointestinal stromal tumors and leiomyosarcomas in the colon: a clinicopathologic, immunohistochemical, and molecular genetic study of 44 cases. Am J Surg Pathol 2000; 24: 1339–1352.
31.
Bundesamt S: Fachserie 1, Reihe 1.3, Bevölkerung und Erwerbstätigkeit. Wiesbaden, Statistisches Bundesamt, 2016.
32.
Volzke H, Alte D, Schmidt CO, Radke D, Lorbeer R, Friedrich N, Aumann N, Lau K, Piontek M, Born G, Havemann C, Ittermann T, Schipf S, Haring R, Baumeister SE, Wallaschofski H, Nauck M, Frick S, Arnold A, Junger M, Mayerle J, Kraft M, Lerch MM, Dorr M, Reffelmann T, Empen K, Felix SB, Obst A, Koch B, Glaser S, Ewert R, Fietze I, Penzel T, Doren M, Rathmann W, Haerting J, Hannemann M, Ropcke J, Schminke U, Jurgens C, Tost F, Rettig R, Kors JA, Ungerer S, Hegenscheid K, Kuhn JP, Kuhn J, Hosten N, Puls R, Henke J, Gloger O, Teumer A, ­Homuth G, Volker U, Schwahn C, Holtfreter B, Polzer I, Kohlmann T, Grabe HJ, Rosskopf D, Kroemer HK, Kocher T, Biffar R, John U, Hoffmann W: Cohort profile: the study of health in pomerania. Int J Epidemiol 2011; 40: 294–307.
33.
Grabe HJ, Assel H, Bahls T, Dorr M, Endlich K, Endlich N, Erdmann P, Ewert R, Felix SB, Fiene B, Fischer T, Flessa S, Friedrich N, Gadebusch-Bondio M, Salazar MG, Hammer E, Haring R, Havemann C, Hecker M, Hoffmann W, Holtfreter B, Kacprowski T, Klein K, Kocher T, Kock H, Krafczyk J, Kuhn J, Langanke M, Lendeckel U, Lerch MM, Lieb W, Lorbeer R, Mayerle J, Meissner K, zu Schwabedissen HM, Nauck M, Ott K, Rathmann W, Rettig R, Richardt C, Salje K, Schminke U, Schulz A, Schwab M, Siegmund W, Stracke S, Suhre K, Ueffing M, Ungerer S, Volker U, Volzke H, Wallaschofski H, Werner V, Zygmunt MT, Kroemer HK: Cohort profile: greifswald approach to individualized medicine (gani_med). J Transl Med 2014; 12: 144.
34.
Lerch MM, Braun J, Harder M, Hofstadter F, Schumpelick V, Matern S: Postoperative adaptation of the small intestine after total colectomy and j-pouch-anal anastomosis. Dis Colon Rectum 1989; 32: 600–608.
35.
Cichoz-Lach H, Kasztelan-Szczerbinska B, Slomka M: Gastrointestinal stromal tumors: Epidemiology, clinical picture, diagnosis, prognosis and treatment. Pol Arch Med Wewn 2008; 118: 216–221.
36.
Liu Q, Wang Y, Kong L, Kan Y: Study on clinicopathological features of gastrointestinal stromal tumor and relevant prognostic factors. Cell Biochem Biophys 2015; 73: 743–747.
37.
Al-Thani H, El-Menyar A, Rasul KI, Al-Sulaiti M, El-Mabrok J, Hajaji K, Elgohary H, Tabeb A: Clinical presentation, management and outcomes of gastrointestinal stromal tumors. Int J Surg 2014; 12: 1127–1133.
38.
Wang M, Xu J, Zhang Y, Tu L, Qiu WQ, Wang CJ, Shen YY, Liu Q, Cao H: Gastrointestinal stromal tumor: 15-years’ experience in a single center. BMC Surg 2014; 14: 93.
39.
Joensuu H, Vehtari A, Riihimaki J, Nishida T, Steigen SE, Brabec P, Plank L, Nilsson B, Cirilli C, Braconi C, Bordoni A, Magnusson MK, Linke Z, Sufliarsky J, Federico M, Jonasson JG, Dei Tos AP, Rutkowski P: Risk of recurrence of gastrointestinal stromal tumour after surgery: an analysis of pooled population-based cohorts. Lancet Oncol 2012; 13: 265–274.
40.
Wronski M, Ziarkiewicz-Wroblewska B, Gornicka B, Cebulski W, Slodkowski M, Wasiutynski A, Krasnodebski IW: Synchronous occurrence of gastrointestinal stromal tumors and other primary gastrointestinal neoplasms. World J Gastroenterol 2006; 12: 5360–5362.
41.
Agaimy A, Wunsch PH, Sobin LH, Lasota J, Miettinen M: Occurrence of other malignancies in patients with gastrointestinal stromal tumors. Semin Diagn Pathol 2006; 23: 120–129.
42.
Miettinen M, Lasota J: Gastrointestinal stromal tumors – definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis. Virchows Arch 2001; 438: 1–12.
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