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Imaging of Gastrointestinal Stromal Tumor

  • Chapter
Oncology

Abstract

Gastrointestinal stromal tumor (GIST) is a rare stromal neoplasm that accounts for 5% of all soft tissue sarcomas.1 It is the most common mesenchymal neoplasm of the gastrointestinal tract. Before the advent of immunohistologic methods, most spindle cell sarcomas of the gastrointestinal tract were considered to be leiomyomas or leiomyosarcomas, with occasional examples of neurogenic tumors. GIST defines a distinct group of gastrointestinal tumors that originate from the intestinal cells of Cajal. These cells act as regulators of bowel peristalsis and therefore are also called pacemaker cells. Cajal cells normally express cKIT (CD 117), which is a tyrosine kinase growth factor receptor. This cKIT immunoreactivity is the best defining feature of GISTs, distinguishing them from true smooth muscle tumors (i.e., leiomyomas and leiomyosarcomas) and tumors arising from neural crest derivatives (i.e., schwannomas and neurofibromas); this is considered the most specific criterion for the diagnosis of GIST. In addition, targeting the cKIT receptor with a cKIT tyrosine kinase inhibitor [STI-571, imatinib (gleevec); Novartis, Basel, Switzerland] has been successfully utilized in treating patients with GIST

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References

  1. 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.

    Article  PubMed  CAS  Google Scholar 

  2. Stelow EB, Stanley MW, Mallery S, Lai R, Linzie BM, Bardales RH. Endoscopic ultrasound-guided fine-needle aspiration findings of gastrointestinal leiomyomas and gastrointestinal stromal tumors. Am J Clin Pathol 2003;119:703–708.

    Article  PubMed  Google Scholar 

  3. Miettinen M, Sobin LH, Sarlomo-Rikala M. Immunohisto-chemical spectrum of GISTs at different sites and their differential diagnosis with a reference to CD117 (KIT). Mod Pathol 2000;13:1134–1142.

    Article  PubMed  CAS  Google Scholar 

  4. Kindblom LG, Remotti HE, Aldenborg F, Meis-Kindblom JM. Gastrointestinal pacemaker cell tumor (GIPACT): gastrointestinal stromal tumors show phenotypic characteristics of the interstitial cells of Cajal. Am J Pathol 1998;152:1259–1269.

    PubMed  CAS  Google Scholar 

  5. Sircar K, Hewlett BR, Huizinga JD, Chorneyko K, Berezin I, Riddell RH. Interstitial cells of Cajal as precursors of gastrointestinal stromal tumors. Am J Surg Pathol 1999;23:377–389.

    Article  PubMed  CAS  Google Scholar 

  6. 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;11:728–734.

    PubMed  CAS  Google Scholar 

  7. Miettinen M, Virolainen M, Maarit Sarlomo R. Gastrointestinal stromal tumors—value of CD34 antigen in their identification and separation from true leiomyomas and schwannomas. Am J Surg Pathol 1995;19:207–216.

    Article  PubMed  CAS  Google Scholar 

  8. Bauer S, Corless CL, Heinrich MC, et al. Response to imatinib mesylate of a gastrointestinal stromal tumor with very low expression of KIT. Cancer Chemother Pharmacol 2003;51:261–265.

    PubMed  Google Scholar 

  9. Dagher R, Cohen M, Williams G, et al. Approval summary: imatinib mesylate in the treatment of metastatic and/or unresectable malignant gastrointestinal stromal tumors. Clin Cancer Res 2002;8:3034–3038.

    PubMed  CAS  Google Scholar 

  10. 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 

  11. Miettinen M, Sarlomo-Rikala M, Lasota J. Gastrointestinal stromal tumors: recent advances in understanding of their biology. Hum Pathol 1999;30:1213–1220.

    Article  PubMed  CAS  Google Scholar 

  12. Greenson JK. Gastrointestinal stromal tumors and other mesenchymal lesions of the gut. Mod Pathol 2003;16:366–375.

    Article  PubMed  Google Scholar 

  13. Miettinen M, Sarlomo-Rikala M, Sobin LH, Lasota J. Esophageal stromal tumors: a clinicopathologic, immunohistochemical, and molecular genetic study of 17 cases and comparison with esophageal leiomyomas and leiomyosarcomas. Am J Surg Pathol 2000;24:211–222.

    Article  PubMed  CAS  Google Scholar 

  14. Miettinen M, Furlong M, Sarlomo-Rikala M, Burke A, Sobin LH, Lasota J. Gastrointestinal stromal tumors, intramural leiomyomas, and leiomyosarcomas in the rectum and anus: a clinicopathologic, immunohistochemical, and molecular genetic study of 144 cases. Am J Surg Pathol 2001;25:1121–1133.

    Article  PubMed  CAS  Google Scholar 

  15. 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.

    Article  PubMed  CAS  Google Scholar 

  16. Logrono R, Jones DV, Faruqi S, Bhutani MS. Recent advances in cell biology, diagnosis, and therapy of gastrointestinal stromal tumor (GIST). Cancer Biol Ther 2004;3:251–258.

    PubMed  Google Scholar 

  17. DeMatteo RP. The GIST of targeted cancer therapy: a tumor (gastrointestinal stromal tumor), a mutated gene (c-kit), and a molecular inhibitor (STI571). Ann Surg Oncol 2002;9:831–839.

    Article  PubMed  Google Scholar 

  18. Fuller CE, Williams GT. Gastrointestinal manifestations of type 1 neurofibromatosis (von Recklinghausen’s disease). Histopathology (Oxf) 1991;19:1–11.

    Article  PubMed  CAS  Google Scholar 

  19. Boldorini R, Tosoni A, Leutner M, et al. Multiple small intestinal stromal tumours in a patient with previously unrecognized neurofibromatosis type 1: immunohistochemical and ultrastructural evaluation. Pathology 2001;33:390–395.

    PubMed  CAS  Google Scholar 

  20. Carney JA. The triad of gastric epithelioid leiomyosarcoma, pulmonary chondroma, and functioning extra-adrenal paraganglioma: a five-year review. Medicine (Baltim) 1983;62:159–169.

    Article  PubMed  CAS  Google Scholar 

  21. Carney JA. Gastric stromal sarcoma, pulmonary chondroma, and extra-adrenal paraganglioma (Carney Triad): natural history, adrenocortical component, and possible familial occurrence. Mayo Clin Proc 1999;74:543–552.

    PubMed  CAS  Google Scholar 

  22. Ueyama T, Guo KJ, Hashimoto H, Daimaru Y, Enjoji M. A clinicopathologic and immunohistochemical study of gastrointestinal stromal tumors. Cancer (Phila) 1992;69:947–955.

    Article  PubMed  CAS  Google Scholar 

  23. Crosby JA, Catton CN, Davis A, et al. Malignant gastrointestinal stromal tumors of the small intestine: a review of 50 cases from a prospective database. Ann Surg Oncol 2001;8:50–59.

    Article  PubMed  CAS  Google Scholar 

  24. Burkill GJ, Badran M, Al-Muderis O, et al. Malignant gastrointestinal stromal tumor: distribution, imaging features, and pattern of metastatic spread. Radiology 2003;226:527–532.

    Article  PubMed  Google Scholar 

  25. Licht JD, Weissmann LB, Antman K. Gastrointestinal sarcomas. Semin Oncol 1988;15:181–188.

    PubMed  CAS  Google Scholar 

  26. Lau S, Tam KF, Kam CK, et al. Imaging of gastrointestinal stromal tumour (GIST). Clin Radiol 2004;59:487–498.

    Article  PubMed  CAS  Google Scholar 

  27. Levy AD, Remotti HE, Thompson WM, Sobin LH, Miettinen M. Gastrointestinal stromal tumors: radiologic features with pathologic correlation. Radiographics 2003;23:283–304, 456; quiz 532.

    Article  PubMed  Google Scholar 

  28. Suster S. Gastrointestinal stromal tumors. Semin Diagn Pathol 1996;13:297–313.

    PubMed  CAS  Google Scholar 

  29. Franquemont DW. Differentiation and risk assessment of gastrointestinal stromal tumors. Am J Clin Pathol 1995;103:41–47.

    PubMed  CAS  Google Scholar 

  30. Fletcher CD, Berman JJ, Corless C, et al. Diagnosis of gastrointestinal stromal tumors: a consensus approach. Hum Pathol 2002;33:459–465.

    Article  PubMed  Google Scholar 

  31. Dematteo RP, Maki RG, Antonescu C, Brennan MF. Targeted molecular therapy for cancer: the application of STI571 to gastrointestinal stromal tumor. Curr Probl Surg 2003;40:144–193.

    Article  PubMed  Google Scholar 

  32. Kamel IR, Fishman EK. Recent advances in CT imaging of liver metastases. Cancer J 2004;10:104–120.

    Article  PubMed  Google Scholar 

  33. Kamel IR, Choti MA, Horton KM, et al. Surgically staged focal liver lesions: accuracy and reproducibility of dual-phase helical CT for detection and characterization. Radiology 2003;227:752–757.

    Article  PubMed  Google Scholar 

  34. Ludwig DJ, Traverso LW. Gut stromal tumors and their clinical behavior. Am J Surg 1997;173:390–394.

    Article  PubMed  CAS  Google Scholar 

  35. Pidhorecky I, Cheney RT, Kraybill WG, Gibbs JF. Gastrointestinal stromal tumors: current diagnosis, biologic behavior, and management. Ann Surg Oncol 2000;7:705–712.

    Article  PubMed  CAS  Google Scholar 

  36. McGrath PC, Neifeld JP, Lawrence W Jr, Kay S, Horsley JS III, Parker GA. Gastrointestinal sarcomas. Analysis of prognostic factors. Ann Surg 1987;206:706–710.

    Article  PubMed  CAS  Google Scholar 

  37. Kamel IR, Lawler LP, Corl FM, Fishman EK. Patterns of collateral pathways in extrahepatic portal hypertension as demonstrated by multidetector row computed tomography and advanced image processing. J Comput Assist Tomogr 2004;28:469–477.

    Article  PubMed  Google Scholar 

  38. Johnson PT, Heath DG, Hofmann LV, Horton KM, Fishman EK. Multidetector-row computed tomography with three-dimensional volume rendering of pancreatic cancer: a complete preoperative staging tool using computed tomography angiography and volume-rendered cholangiopancreatography. J Comput Assist Tomogr 2003;27:347–353.

    Article  PubMed  Google Scholar 

  39. Nino-Murcia M, Tamm EP, Charnsangavej C, Jeffrey RB Jr. Multidetector-row helical CT and advanced postprocessing techniques for the evaluation of pancreatic neoplasms. Abdom Imaging 2003;28:366–377.

    Article  PubMed  CAS  Google Scholar 

  40. Springer P, Dessl A, Giacomuzzi SM, et al. Virtual computed tomography gastroscopy: a new technique. Endoscopy 1997;29:632–634.

    PubMed  CAS  Google Scholar 

  41. Raptopoulos V, Davis MA, Davidoff A, et al. Fat-density oral contrast agent for abdominal CT. Radiology 1987;164:653–656.

    PubMed  CAS  Google Scholar 

  42. Horton KM, Fishman EK. Current role of CT in imaging of the stomach. Radiographics 2003;23:75–87.

    Article  PubMed  Google Scholar 

  43. Matsuoka Y, Masumoto T, Koga H, et al. Positive and negative oral contrast agents for combined abdominal and pelvic helical CT: first iodinated agent and second water. Radiat Med 2000;18:213–216.

    PubMed  CAS  Google Scholar 

  44. Thoeni RF, Filson RG. Abdominal and pelvic CT: use of oral metoclopramide to enhance bowel opacification. Radiology 1988;169:391–393.

    PubMed  CAS  Google Scholar 

  45. Horton KM, Fishman EK. Multidetector-row computed tomography and 3-dimensional computed tomography imaging of small bowel neoplasms: current concept in diagnosis. J Comput Assist Tomogr 2004;28:106–116.

    Article  PubMed  Google Scholar 

  46. Tateishi U, Hasegawa T, Satake M, Moriyama N. Gastrointestinal stromal tumor. Correlation of computed tomography findings with tumor grade and mortality. J Comput Assist Tomogr 2003;27:792–798.

    Article  PubMed  Google Scholar 

  47. Chen MY, Bechtold RE, Savage PD. Cystic changes in hepatic metastases from gastrointestinal stromal tumors (GISTs) treated with Gleevec (imatinib mesylate). AJR Am J Roentgenol 2002;179:1059–1062.

    PubMed  Google Scholar 

  48. Joensuu H, Fletcher C, Dimitrijevic S, Silberman S, Roberts P, Demetri G. Management of malignant gastrointestinal stromal tumours. Lancet Oncol 2002;3:655–664.

    Article  PubMed  CAS  Google Scholar 

  49. Berman J, O’Leary TJ. Gastrointestinal stromal tumor workshop. Hum Pathol 2001;32:578–582.

    Article  PubMed  CAS  Google Scholar 

  50. Pope TL Jr, Buschi AJ, Brenbridge AN. Leiomyosarcoma of the duodenum simulating pancreatic carcinoma: case report. Va Med 1982;109:398–400.

    PubMed  Google Scholar 

  51. Lawler LP, Horton KM, Fishman EK. Peripancreatic masses that simulate pancreatic disease: spectrum of disease and role of CT. Radiographics 2003;23:1117–1131.

    Article  PubMed  Google Scholar 

  52. Tworek JA, Goldblum JR, Weiss SW, Greenson JK, Appelman HD. Stromal tumors of the anorectum: a clinicopathologic study of 22 cases. Am J Surg Pathol 1999;23:946–954.

    Article  PubMed  CAS  Google Scholar 

  53. Miettinen M, El-Rifai W, L HLS, Lasota J. Evaluation of malignancy and prognosis of gastrointestinal stromal tumors: a review. Hum Pathol 2002;33:478–483.

    Article  PubMed  CAS  Google Scholar 

  54. Miettinen M, Monihan JM, Sarlomo-Rikala M, et al. Gastrointestinal stromal tumors/smooth muscle tumors (GISTs) primary in the omentum and mesentery: clinicopathologic and immunohistochemical study of 26 cases. Am J Surg Pathol 1999;23:1109–1118.

    Article  PubMed  CAS  Google Scholar 

  55. Zighelboim I, Henao G, Kunda A, Gutierrez C, Edwards C. Gastrointestinal stromal tumor presenting as a pelvic mass. Gynecol Oncol 2003;91:630–635.

    Article  PubMed  Google Scholar 

  56. Joensuu H, Roberts PJ, Sarlomo-Rikala M, et al. Effect of the tyrosine kinase inhibitor STI571 in a patient with a metastatic gastrointestinal stromal tumor. N Engl J Med 2001;344:1052–1056.

    Article  PubMed  CAS  Google Scholar 

  57. Ng EH, Pollock RE, Munsell MF, Atkinson EN, Romsdahl MM. Prognostic factors influencing survival in gastrointestinal leiomyosarcomas. Implications for surgical management and staging. Ann Surg 1992;215:68–77.

    PubMed  CAS  Google Scholar 

  58. Lehnert T. Gastrointestinal sarcoma (GIST): a review of surgical management. Ann Chir Gynaecol 1998;87:297–305.

    PubMed  CAS  Google Scholar 

  59. Mudan SS, Conlon KC, Woodruff JM, Lewis JJ, Brennan MF. Salvage surgery for patients with recurrent gastrointestinal sarcoma: prognostic factors to guide patient selection. Cancer (Phila) 2000;88:66–74.

    Article  PubMed  CAS  Google Scholar 

  60. Demetri GD, von Mehren M, Blanke CD, et al. Efficacy and safety of imatinib mesylate in advanced gastrointestinal stromal tumors. N Engl J Med 2002;347:472–480.

    Article  PubMed  CAS  Google Scholar 

  61. van Oosterom AT, Judson I, Verweij J, et al. Safety and efficacy of imatinib (STI571) in metastatic gastrointestinal stromal tumours: a phase I study. Lancet 2001;358:1421–1423.

    Article  PubMed  Google Scholar 

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Kamel, I.R., Fishman, E.K. (2006). Imaging of Gastrointestinal Stromal Tumor. In: Chang, A.E., et al. Oncology. Springer, New York, NY. https://doi.org/10.1007/0-387-31056-8_30

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  • DOI: https://doi.org/10.1007/0-387-31056-8_30

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