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Gastric Gastrointestinal Stromal Tumors (GIST): a Case Series and Current State of the Art in the Workup and Treatment of This Rare Disease

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References

  1. Mucciarini C, Rossi G, Bertolini F, Valli R, Cirilli C, Rashid I, et al. Incidence and clinicopathologic features of gastrointestinal stromal tumors. A population-based study. BMC Cancer. 2007;7(1):230. https://doi.org/10.1186/1471-2407-7-230.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. Hirota S, Isozaki K, Moriyama Y, Hashimoto K, Nishida T, Ishiguro S, et al. Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science. 1998;279(5350):577–80. https://doi.org/10.1126/science.279.5350.577.

    Article  CAS  PubMed  Google Scholar 

  3. Miettinen M, Lasota J. Gastrointestinal stromal tumors: definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis. Virchows Arch. 2001;438(1):1–12. https://doi.org/10.1007/s004280000338.

    Article  CAS  PubMed  Google Scholar 

  4. Nishida T, Hirota S. Biological and clinical review of stromal tumors in the gastrointestinal tract. Histol Histopathol. 2000;15(4):1293–301.

    CAS  PubMed  Google Scholar 

  5. Sarlomo-Rikala M, Kovatich AJ, Barusevicius A, Miettinen M. CD117: a sensitive marker for gastrointestinal stromal tumors that is more specific than CD34. Mod Pathol. 1998;11(8):728–34.

    CAS  PubMed  Google Scholar 

  6. De Vogelaere K, Aerts M, Haentjens P, de Grève J, Delvaux G. Gastrointestinal stromal tumor of the stomach: progresses in diagnosis and treatment. Acta Gastroenterol Belg. 2013;76(4):403–6.

    PubMed  Google Scholar 

  7. Heinrich MC, Corless CL, Duensing A, McGreevey L, Chen CJ, Joseph N, et al. PDGFRA activating mutations in gastrointestinal stromal tumors. Science. 2003;299(5607):708–10. https://doi.org/10.1126/science.1079666.

    Article  CAS  PubMed  Google Scholar 

  8. DiMaio CJ, et al. Initial experience with a novel EUS-guided core biopsy needle (SharkCore): results of a large North American multicenter study. Endosc Int Open. 2016;4(9):E974–9.

    Article  PubMed  PubMed Central  Google Scholar 

  9. El Chafic AM, Loren D, et al. Comparison of FNA and fine-needle biopsy for EUS-guided sampling of suspected GI stromal tumors. Gastrointest Endosc. 2017;86(3):510–5. https://doi.org/10.1016/j.gie.2017.01.010.

    Article  PubMed  Google Scholar 

  10. Levy AD, Remotti HE, Thompson WM, Sobin LH, Miettinen M. Gastrointestinal stromal tumors: radiologic features with pathologic correlation. Radiographics. 2003;23(2):283–304. https://doi.org/10.1148/rg.232025146.

    Article  PubMed  Google Scholar 

  11. 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(1):51–8. https://doi.org/10.1097/00000658-200001000-00008.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  12. Zhang L, Smyrk TC, Young WF Jr, Stratakis CA, Carney JA. Gastric stromal tumors in Carney triad are different clinically, pathologically, and behaviorally from sporadic gastric gastrointestinal stromal tumors: findings in 104 cases. Am J Surg Pathol. 2010;34(1):53–64. https://doi.org/10.1097/PAS.0b013e3181c20f4f.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Miettinen M, Fetsch JF, Sobin LH, Lasota J. Gastrointestinal stromal tumors in patients with neurofibromatosis 1: a clinicopathologic and molecular genetic study of 45 cases. Am J Surg Pathol. 2006;30(1):90–6. https://doi.org/10.1097/01.pas.0000176433.81079.bd.

    Article  PubMed  Google Scholar 

  14. 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(1):162–8. https://doi.org/10.1111/j.1572-0241.2005.40709.x.

    Article  PubMed  Google Scholar 

  15. Kim KM, Kang DW, Moon WS, Park JB, Park CK, Sohn JH, et al. Gastrointestinal stromal tumors in Koreans: it’s incidence and the clinical, pathologic and immunohistochemical findings. J Korean Med Sci. 2005;20(6):977–84. https://doi.org/10.3346/jkms.2005.20.6.977.

    Article  PubMed  PubMed Central  Google Scholar 

  16. 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(18):2868–72. https://doi.org/10.1016/j.ejca.2005.09.009.

    Article  PubMed  Google Scholar 

  17. Miettinen M, Lasota J. Gastrointestinal stromal tumors (GISTs): definition, occurrence, pathology, differential diagnosis and molecular genetics. Pol J Pathol. 2003;54(1):3–24.

    PubMed  Google Scholar 

  18. Novitsky YW, Kercher KW, Sing RF, Heniford BT. Long-term outcomes of laparoscopic resection of gastrointestinal stromal tumors. Ann Surg. 2006;243(6):738–745; discussion 745-7. https://doi.org/10.1097/01.sla.0000219739.11758.27.

    Article  PubMed  PubMed Central  Google Scholar 

  19. Marano L, Torelli F, Schettino M, Porfidia R, Reda G, Grassia M, et al. Combined laparoscopic-endoscopic “rendezvous” procedure for minimally invasive resection of gastrointestinal stromal tumor of the stomach. Am Surg. 2011;77(8):1100–1102(3).

    PubMed  Google Scholar 

  20. Wilhelm D, von Delius S, Burian M, Schneider A, Frimberger E, Meining A, et al. Simultaneous use of laparoscopy and endoscopy for minimally invasive resection of gastric subepithelia masses—analysis of 93 interventions. World J Surg. 2008;32(6):1021–8. https://doi.org/10.1007/s00268-008-9492-1.

    Article  CAS  PubMed  Google Scholar 

  21. Davila JS, Mombian D, Gines A, Sanchez-Montes C, Araujo I, Saavendra-Perez D, et al. Endoscopic-assisted laparoscopic resection for gastric subepithelial tumors. Surg Endosc. 2016;30(1):199–203. https://doi.org/10.1007/s00464-015-4183-0.

    Article  PubMed  Google Scholar 

  22. Kim HH. Endoscopic treatment for gastrointestinal stromal tumor: advantages and hurdles. World J Gastrointest Endosc. 2015;7(3):192–205.

    Article  PubMed  PubMed Central  Google Scholar 

  23. Andalib I, Yeoun D, Reddy R, Xie S, Iqbal S. Endoscopic resection of gastric gastrointestinal stromal tumors rising from the muscularis propria layer in North America: methods and feasibility data. Surg Endosc. 2017; https://doi.org/10.1007/s00464-017-5862-9.

  24. Ping-Hong Z, et al. Endoscopic full thickness resection without laparoscopic assistance for gastric submucosal tumors originated from the mucosal propria. Surg Endosc. 2011;25(9):2926–31.

    Article  Google Scholar 

  25. Nishida T. The role of endoscopy in the diagnosis of gastric gastrointestinal tumors. Ann Surg Oncol. 2015;22(9):2810–1. https://doi.org/10.1245/s10434-015-4520-5.

    Article  PubMed  PubMed Central  Google Scholar 

  26. Pappo AS, Janeway KA. Pediatric gastrointestinal stromal tumors. Hematol Oncol Clin North Am. 2009;23(1):15–34. https://doi.org/10.1016/j.hoc.2008.11.005.

    Article  PubMed  Google Scholar 

  27. Fletcher CD, Berman JJ, Corless C, et al. Diagnosis of gastrointestinal stromal tumors: a consensus approach. Hum Pathol. 2002;33(5):459–65. https://doi.org/10.1053/hupa.2002.123545.

    Article  PubMed  Google Scholar 

  28. Kawanowa K, Sakuma Y, Sakurai S, Hishima T, Iwasaki Y, Saito K, et al. High incidence of microscopic gastrointestinal stromal tumors in the stomach. Hum Pathol. 2006;37(12):1527–35. https://doi.org/10.1016/j.humpath.2006.07.002.

    Article  PubMed  Google Scholar 

  29. Agaimy A, Wiinsch PH, Hofstaedter F, et al. Minute gastric sclerosing stromal tumors (GIST tumorlets) are common in adults and frequently show c-KIT mutations. Am J Surg Pathol. 2007;31(1):113–20. https://doi.org/10.1097/01.pas.0000213307.05811.f0.

    Article  PubMed  Google Scholar 

  30. Yan BM, Kaplan GG, Urbanski S, Nash CL, Beck PL. Epidemiology of gastrointestinal stromal tumors in a defined Canadian Health Region: a population-based study. Int J Surg Pathol. 2008;16(3):241–50. https://doi.org/10.1177/1066896907306967.

    Article  PubMed  Google Scholar 

  31. Demetri GD, Benjamin RS, Blanke CD, Blay JY, Casali P, Choi H, et al. NCCN Task Force report: management of patients with gastrointestinal stromal tumor (GIST)—update of the NCCN clinical practice guidelines. J Natl Compr Canc Netw. 2007;5(Suppl 2):S1–29. quiz S30

    Article  PubMed  Google Scholar 

  32. Blay JY, Bonvalot S, Casali P, Choi H, Debiec-Richter M, Dei Tos AP, et al. Consensus meeting for the management of gastrointestinal stromal tumors. Report of the GIST consensus conference of 20-21 March 2004, under the auspices of ESMO. Ann Oncol. 2005;16(4):566–78. https://doi.org/10.1093/annonc/mdi127.

    Article  PubMed  Google Scholar 

  33. Benjamin RS, Choi H, Macapinlac HA, Burgess MA, Patel SR, Chen LL, et al. We should desist using RECIST, at least in GIST. J Clin Oncol. 2007;25(13):1760–4. https://doi.org/10.1200/JCO.2006.07.3411.

    Article  PubMed  Google Scholar 

  34. Agaimy A. Gastrointestinal stromal tumors (GIST) from risk stratification systems to the new TNM proposal: more questions than answers? A review emphasizing the need for a standardized GIST reporting. Int J Clin Exp Pathol. 2010;3(5):461–71.

    PubMed  PubMed Central  Google Scholar 

  35. Hong HS, Ha HK, Won HJ, Byun JH, Shin YM, Kim AY, et al. Gastric schwannomas: radiological features with endoscopic and pathological correlation. Clin Radiol. 2008;63(5):536–42. https://doi.org/10.1016/j.crad.2007.05.026.

    Article  CAS  PubMed  Google Scholar 

  36. ESMO/European Sarcoma Network Working Group. Gastrointestinal stromal tumours: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2014;25(Suppl 3):iii21–6.

    Article  Google Scholar 

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Funding

This research was supported by The Mirro Center for Research and Innovation and the Parkview Research Center.

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Correspondence to Christina Zelt.

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Research Key Highlights

• Gastrointestinal stromal tumors (GISTs) are uncommon intramural tumors of the GI tract. They are most often seen in the stomach and arise from the muscular layers of the visceral wall.

• The clinical manifestations of GISTs vary depending on the tumor size and location, but are often asymptomatic.

• Advances in endosonography (EUS) and immunohistochemical staining can distinguish these lesions from other intramural and GI tract tumors and are an essential part of the diagnostic and staging workup.

• The advent of EUS-guided fine needle biopsy (FNB) allows for large core tissue acquisition. This has replaced FNA and allowed for histologic analysis of large specimens, increased IHC confirmation, and personalized medicine.

• Treatment involves surgical, endoscopic or hybrid endoscopic-laparoscopic resection. Imatinib may be given, and lesions less than 2 cm may be observed.

• The cases presented in this report represent pathology-confirmed GISTs diagnosed at a large volume, community, tertiary referral GI oncology program during July 2012 through May 2016. Follow-ups for each patient have discovered no recurrences to date.

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Sharma, N.R., Gopakumar, H., Harrison, S. et al. Gastric Gastrointestinal Stromal Tumors (GIST): a Case Series and Current State of the Art in the Workup and Treatment of This Rare Disease. J Gastrointest Canc 50, 548–555 (2019). https://doi.org/10.1007/s12029-017-0034-7

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