Skip to main content

Advertisement

SpringerLink
Log in

Presentation and Management of Gastrointestinal Stromal Tumors of the Duodenum: A Multi-Institutional Analysis

  • Bone and Soft Tissue Sarcomas
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript

Abstract

Background

Duodenal gastrointestinal stromal tumors (GISTs) are a small subset of GISTs, and their management is poorly defined. We evaluated surgical management and outcomes of patients with duodenal GISTs treated with pancreaticoduodenectomy (PD) versus local resection (LR) and defined factors associated with prognosis.

Methods

Between January 1994 and January 2011, 96 patients with duodenal GISTs were identified from five major surgical centers. Perioperative and long-term outcomes were compared based on surgical approach (PD vs LR).

Results

A total of 58 patients (60.4 %) underwent LR, while 38 (39.6 %) underwent PD. Patients presented with gross bleeding (n = 25; 26.0 %), pain (n = 23; 24.0 %), occult bleeding (n = 19; 19.8 %), or obstruction (n = 3; 3.1 %). GIST lesions were located in first (n = 8, 8.4 %), second (n = 47; 49 %), or third/fourth (n = 41; 42.7 %) portion of duodenum. Most patients (n = 86; 89.6 %) had negative surgical margins (R0) (PD, 92.1 vs LR, 87.9 %) (P = 0.34). Median length of stay was longer for PD (11 days) versus LR (7 days) (P = 0.001). PD also had more complications (PD, 57.9 vs LR, 29.3 %) (P = 0.005). The 1-, 2-, and 3-year actuarial recurrence-free survival was 94.2, 82.3, and 67.3 %, respectively. Factors associated with a worse recurrence-free survival included tumor size [hazard ratio (HR) = 1.09], mitotic count >10 mitosis/50 HPF (HR = 6.89), AJCC stage III disease (HR = 4.85), and NIH high risk classification (HR = 4.31) (all P < 0.05). The 1-, 3-, and 5-year actuarial survival was 98.3, 87.4, and 82.0%, respectively. PD versus LR was not associated with overall survival.

Conclusions

Recurrence of duodenal GIST is dependent on tumor biology rather than surgical approach. PD was associated with longer hospital stays and higher risk of perioperative complications. When feasible, LR is appropriate for duodenal GIST and PD should be reserved for lesions not amenable to LR.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

Reference

  1. Miettinen M, Lasota J. Gastrointestinal stromal tumors: definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis. Virchows Arch. 2001;438:1–12.

    Article  PubMed  CAS  Google Scholar 

  2. Fletcher CD, Berman JJ, Corless C, Gorstein F, Lasota J, Longley BJ, et al.. Diagnosis of gastrointestinal stromal tumors: a consensus approach. Hum Pathol. 2002;33:459–65.

    Article  PubMed  Google Scholar 

  3. Joensuu H, Fletcher C, Dimitrijevic S, Silberman S, Roberts P, Demetri G, et al. Management of malignant gastrointestinal stromal tumours. Lancet Oncol. 2002;3:655–64.

    Article  PubMed  CAS  Google Scholar 

  4. Connolly EM, Gaffney E, Reynolds JV. Gastrointestinal stromal tumours. Br J Surg. 2003;90:1178–86.

    Article  PubMed  CAS  Google Scholar 

  5. Miettinen M, Kopczynski J, Makhlouf HR, Sarlomo-Rikala M, Gyorffy H, Burke A, et al. Gastrointestinal stromal tumors, intramural leiomyomas, and leiomyosarcomas in the duodenum: a clinicopathologic, immunohistochemical, and molecular genetic study of 167 cases. Am J Surg Pathol. 2003;27:625–41.

    Article  PubMed  Google Scholar 

  6. Winfield RD, Hochwald SN, Vogel SB, Hemming AW, Liu C, Cance WG, et al. Presentation and management of gastrointestinal stromal tumors of the duodenum. Am Surg. 2006;72:719–22; discussion 722–3.

    PubMed  Google Scholar 

  7. Emory TS, Sobin LH, Lukes L, Lee DH, O’Leary TJ. Prognosis of gastrointestinal smooth-muscle (stromal) tumors: dependence on anatomic site. Am J Surg Pathol. 1999;23:82–7.

    Article  PubMed  CAS  Google Scholar 

  8. Wasag B, Debiec-Rychter M, Pauwels P, Stul M, Vranckx H, Ooosterom AV, et al. Differential expression of KIT/PDGFRA mutant isoforms in epithelioid and mixed variants of gastrointestinal stromal tumors depends predominantly on the tumor site. Mod Pathol. 2004;17:889–94.

    Article  PubMed  CAS  Google Scholar 

  9. Miettinen M, Sobin LH, Sarlomo-Rikala M. Immunohistochemical spectrum of GISTs at different sites and their differential diagnosis with a reference to CD117 (KIT). Mod Pathol. 2000;13:1134–42.

    Article  PubMed  CAS  Google Scholar 

  10. Nilsson B, Bümming P, Meis-Kindblom JM, Odén A, Dortok A, Gustavsson B, et al. Gastrointestinal stromal tumors: the incidence, prevalence, clinical course, and prognostication in the preimatinib mesylate era: a population-based study in western Sweden. Cancer. 2005;103:821–9.

    Article  PubMed  Google Scholar 

  11. Blay JY, Bonvalot S, Casali P, Choi H, Debiec-Richter M, De 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;16:566–78.

    Google Scholar 

  12. Goh BK, Chow PK, Kesavan S, Yap WM, Wong WK. Outcome after surgical treatment of suspected gastrointestinal stromal tumors involving the duodenum: is limited resection appropriate? J Surg Oncol. 2008;97:388–91.

    Article  PubMed  Google Scholar 

  13. Singer S, Rubin BP, Lux ML, Chen CJ, Demetri GD, Fletcher CD, et al. Prognostic value of KIT mutation type, mitotic activity, and histologic subtype in gastrointestinal stromal tumors. J Clin Oncol. 2002;20:3898–905.

    Article  PubMed  CAS  Google Scholar 

  14. Lanuke K, Bathe OF, Mack LA. Local excision of duodenal gastrointestinal stromal tumor. J Surg Oncol. 2007;95:267–9.

    Article  PubMed  Google Scholar 

  15. Chung JC, Chu CW, Cho GS, Shin EJ, Lim CW, Kim HC, et al. Management and outcome of gastrointestinal stromal tumors of the duodenum. J Gastrointest Surg. 2010;14:880–3.

    Article  PubMed  Google Scholar 

  16. Mennigen R, Wolters HH, Schulte B, Pelster FW. Segmental resection of the duodenum for gastrointestinal stromal tumor (GIST). World J Surg Oncol. 2008;6:105.

    Article  PubMed  Google Scholar 

  17. Gervaz P, Huber O, Morel P. Surgical management of gastrointestinal stromal tumours. Br J Surg. 2009;96:567–78.

    Article  PubMed  CAS  Google Scholar 

  18. Yang WL, Yu R, Wu YJ, Zhu KK, Ding W, Gao Y, et al. Duodenal gastrointestinal stromal tumor: clinical, pathologic, immunohistochemical characteristics, and surgical prognosis. J Surg Oncol. 2009;100:606–10.

    Article  PubMed  Google Scholar 

  19. Bucher P, Egger JF, Gervaz P, Ris F, Weintraub D, Villiger P, et al. An audit of surgical management of gastrointestinal stromal tumours (GIST). Eur J Surg Oncol. 2006;32:310–4.

    Article  PubMed  CAS  Google Scholar 

  20. Cameron JL, Riall TS, Coleman J, Belcher KA. One thousand consecutive pancreaticoduodenectomies. Ann Surg. 2006;244:10–5.

    Article  PubMed  Google Scholar 

  21. 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;3:461–71.

    PubMed  Google Scholar 

  22. Clavien PA, Barkun J, de Oliveira ML, Vauthey JN, Dindo D, Schulick RD, et al. The Clavien–Dindo classification of surgical complications: five-year experience. Ann Surg. 2009;250:187–96.

    Article  PubMed  Google Scholar 

  23. Goldblum JR, Appelman HD. Stromal tumors of the duodenum. A histologic and immunohistochemical study of 20 cases. Am J Surg Pathol. 1995;19:71–80.

    Article  PubMed  CAS  Google Scholar 

  24. Tien YW, Lee CY, Huang CC, Hu RH, Lee PH. Surgery for gastrointestinal stromal tumors of the duodenum. Ann Surg Oncol. 2010;17:109–14.

    Article  PubMed  Google Scholar 

  25. Miki Y, Kurokawa Y, Hirao M, Fujitani K, Iwasa Y, Mano M, et al. Survival analysis of patients with duodenal gastrointestinal stromal tumors. J Clin Gastroenterol. 2010;44:97–101.

    Article  PubMed  Google Scholar 

  26. Asakawa M, Sakamoto Y, Kajiwara T, Nara S, Esaki M, Shimada K, et al. Simple segmental resection of the second portion of the duodenum for the treatment of gastrointestinal stromal tumors. Langenbecks Arch Surg. 2008;393:605–9.

    Article  PubMed  Google Scholar 

  27. Rutkowski P, Nowecki ZI, Michej W, Debiec-Rychter M, Wozniak A, Limon J, et al. Risk criteria and prognostic factors for predicting recurrences after resection of primary gastrointestinal stromal tumor. Ann Surg Oncol. 2007;14:2018–27.

    Article  PubMed  Google Scholar 

  28. Pierie JP, Choudry U, Muzikansky A, Yeap BY, Souba WW, Ott MJ. The effect of surgery and grade on outcome of gastrointestinal stromal tumors. Arch Surg. 2001;136:383–9.

    Article  PubMed  CAS  Google Scholar 

  29. Tryggvason G, Gíslason HG, Magnússon MK, Jónasson 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;117:289–93.

    Article  PubMed  CAS  Google Scholar 

  30. Wu PC, Langerman A, Ryan CW, Hart J, Swiger S, Posner MC. Surgical treatment of gastrointestinal stromal tumors in the imatinib (STI-571) era. Surgery. 2003;134:656–65; discussion 665–6.

    Article  PubMed  Google Scholar 

  31. 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;7:230.

    Article  PubMed  Google Scholar 

  32. Takahashi T, Nakajima K, Nishitani A, Souma Y, Hirota S, Sawa Y, et al. An enhanced risk-group stratification system for more practical prognostication of clinically malignant gastrointestinal stromal tumors. Int J Clin Oncol. 2007;12:369–74.

    Article  PubMed  CAS  Google Scholar 

  33. Hassan I, You YN, Shyyan R, Dozois EJ, Smyrk TC, Okuno SH, et al. Surgically managed gastrointestinal stromal tumors: a comparative and prognostic analysis. Ann Surg Oncol. 2008;15:52–9.

    Article  PubMed  Google Scholar 

  34. Huang HY, Li CF, Huang WW, Hu TH, Lin CN, Uen YH, et al. A modification of NIH consensus criteria to better distinguish the highly lethal subset of primary localized gastrointestinal stromal tumors: a subdivision of the original high-risk group on the basis of outcome. Surgery. 2007;141:748–56.

    Article  PubMed  Google Scholar 

  35. Pidhorecky I, Cheney RT, Kraybill WG, Gibbs JF. Gastrointestinal stromal tumors: current diagnosis, biologic behavior, and management. Ann Surg Oncol. 2000;7:705–12.

    Article  PubMed  CAS  Google Scholar 

  36. Machado NO, Chopra PJ, Al-Haddabi IH, Al-Qadhi H. Large duodenal gastrointestinal stromal tumor presenting with acute bleeding managed by a Whipple resection. A review of surgical options and the prognostic indicators of outcome. JOP. 2011;12:194–9.

    PubMed  Google Scholar 

  37. Cohen MH, Cortazar P, Justice R, Pazdur R. Approval summary: pemetrexed maintenance therapy of advanced/metastatic nonsquamous, non-small cell lung cancer (NSCLC). Oncologist. 2010;15:1352–8.

    Article  PubMed  CAS  Google Scholar 

  38. Demetri GD, von Mehren M, Blanke CD, Van den Abbeele AD, Eisenberg B, Roberts PJ, et al. Efficacy and safety of imatinib mesylate in advanced gastrointestinal stromal tumors. N Engl J Med. 2002;347:472–80.

    Article  PubMed  CAS  Google Scholar 

  39. Cohen MH, Cortazar P, Justice R, Pazdur R, et al. Approval summary: imatinib mesylate in the adjuvant treatment of malignant gastrointestinal stromal tumors. Oncologist. 2010;15:300–7.

    Article  PubMed  CAS  Google Scholar 

  40. Mohiuddin K, Nizami S, Munir A, Memon B, Memon MA. Metastatic duodenal GIST: role of surgery combined with imatinib mesylate. Int Semin Surg Oncol. 2007;4:9.

    Article  PubMed  Google Scholar 

  41. Gold JS, van der Zwan SM, Gönen M, Maki RG, Singer S, Brennan MF, et al. Outcome of metastatic GIST in the era before tyrosine kinase inhibitors. Ann Surg Oncol. 2007;14:134–42.

    Article  PubMed  Google Scholar 

  42. Eisenberg BL, Judson I. Surgery and imatinib in the management of GIST: emerging approaches to adjuvant and neoadjuvant therapy. Ann Surg Oncol. 2004;11:465–75.

    Article  PubMed  Google Scholar 

  43. Goh BK, Chow PK, Chuah KL, Yap WM, Wong WK. Pathologic, radiologic and PET scan response of gastrointestinal stromal tumors after neoadjuvant treatment with imatinib mesylate. Eur J Surg Oncol. 2006;32:961–3.

    Article  PubMed  CAS  Google Scholar 

  44. Dematteo RP, Ballman KV, Antonescu CR, Maki RG, Pisters PW, Demetri GD, et al. Adjuvant imatinib mesylate after resection of localised, primary gastrointestinal stromal tumour: a randomised, double-blind, placebo-controlled trial. Lancet. 2009;373:1097–104.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Surgery, The Johns Hopkins University, Baltimore, MD, USA

    Fabian M. Johnston MD, Peter J. Kneuertz MD, John L. Cameron MD, Omar Hyder MD, Michael A. Choti MD, Christopher L. Wolfgang MD, PhD & Timothy M. Pawlik MD, MPH, PhD

  2. Department of Surgery, Washington University School of Medicine, St. Louis, MO, USA

    Dominic Sanford MD & William G. Hawkins MD

  3. Department of Surgery, Emory University School of Medicine, Atlanta, GA, USA

    Sarah Fisher MD, David A. Kooby MD & Shishir K. Maithel MD

  4. Department of Surgery, Duke University Medical Center, Durham, NC, USA

    Ryan Turley MD & Dan Blazer III MD

  5. Department of Surgery, Medical College of Wisconsin, Milwaukee, WI, USA

    Ryan Groeschl MD & T. Clark Gamblin MD

Authors
  1. Fabian M. Johnston MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Peter J. Kneuertz MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. John L. Cameron MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Dominic Sanford MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Sarah Fisher MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Ryan Turley MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Ryan Groeschl MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Omar Hyder MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. David A. Kooby MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Dan Blazer III MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Michael A. Choti MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Christopher L. Wolfgang MD, PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

  13. T. Clark Gamblin MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  14. William G. Hawkins MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  15. Shishir K. Maithel MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  16. Timothy M. Pawlik MD, MPH, PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Timothy M. Pawlik MD, MPH, PhD.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Johnston, F.M., Kneuertz, P.J., Cameron, J.L. et al. Presentation and Management of Gastrointestinal Stromal Tumors of the Duodenum: A Multi-Institutional Analysis. Ann Surg Oncol 19, 3351–3360 (2012). https://doi.org/10.1245/s10434-012-2551-8

Download citation

  • Received:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1245/s10434-012-2551-8

Keywords

Search

Navigation