Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Gastric Gastrointestinal Stromal Tumors Workup

  • Author: Michael A Choti, MD, MBA, FACS; Chief Editor: N Joseph Espat, MD, MS, FACS  more...
 
Updated: Sep 15, 2015
 

Laboratory Studies

Laboratory studies are not diagnostic, and no identifiable tumor markers exist.

Next

Imaging Studies

See the list below:

  • Computed tomography scanning of the abdomen: Abdominal CT scanning with intravenous and oral contrast material is a necessary step in the evaluation of these patients. The gastric mass can be detected originating from the gastric wall, as depicted in the image below, but, at times, the organ site of origin is not clear on CT scan. CT scanning can also be used to evaluate tumor invasion to adjacent structures and the presence of intra-abdominal metastasis. The identification of distant disease is important as many as half of patients who initially present with a GIST have distant metastases (two-thirds of whom have hepatic involvement).
    CT scan of the abdomen with oral contrast in a 60- CT scan of the abdomen with oral contrast in a 60-year-old woman with a gastric gastrointestinal stromal tumor (GIST). A huge mass with central necrosis is observed originating from the gastric wall and narrowing its lumen. An ulcer crater can be identified within the mass (arrow).
  • Endoscopic ultrasonography: Endoscopic ultrasonography (EUS) can be a valuable tool in the diagnosis and preoperative assessment of gastric GISTs when the diagnosis or location is in doubt but is not generally required for preoperative workup. [15, 16]
    • If the location of the lesion is in doubt, the EUS can help plan the operative approach (eg, demonstrating that a proximal gastric lesion on CT scan is far enough away from the GE junction to allow local wedge resection as opposed to total gastrectomy). EUS can also demonstrate the submucosal location of the tumor and can define its size, borders, and echoic pattern.
    • In general, ultrasonic features of a mass suspicious for malignancy are large tumors, tumors with irregular extraluminal borders, and the presence of cystic spaces and echogenic foci.
    • All gastric GISTs should be considered to have malignant potential. Although, generally, gastric GISTs less than 2 cm tend to behave as "benign" masses, there have been rare reports of distant spread. EUS is generally the preferred modality to facilitate biopsy of the lesion in cases in which biopsy aids in the workup and management of the patient (see Preoperative biopsy).
Previous
Next

Procedures

Upper endoscopy

Upper endoscopy is often the first examination performed in the evaluation of patients with upper gastrointestinal symptoms, but endoscopy is not generally required in the workup of patients with lesions on CT suspicious for a gastric GIST. Gastroscopy may demonstrate a firm, smooth, yellowish submucosal mass, which can be ulcerated, as depicted in the image below.

Shown here is a gastric gastrointestinal stromal t Shown here is a gastric gastrointestinal stromal tumor (GIST). This is a gross specimen following partial gastrectomy. Note the submucosal tumor mass with the classic features of central umbilication and ulceration.

These tumors can be missed endoscopically because of their frequent submucosal and extraluminal growth. If the diagnosis is suspected prior to endoscopy, an endoscopic ultrasound can be performed to further characterize and help confirm the lesion's growth from the stomach (when the organ site of the tumor is not clearly evident on CT scan), even if not visible endoscopically.

Preoperative biopsy

While the diagnosis can often be made using ultrasonographic-guided biopsy, the use of biopsy is controversial in an otherwise primary, resectable lesion suspicious for GIST. Generally, unless a concern for an alternative diagnosis or use of neoadjuvant therapy is being entertained, the use of biopsy is not recommended in this setting (www.nccn.org).[17] The biopsy of a GIST, which tends to be soft and fragile, may cause intratumoral hemorrhage or even rupture and may increase the risk for tumor dissemination. Generally, irrespective of the biopsy results, surgical resection is required for treatment and for definitive diagnosis.

  • Reasons to perform a biopsy: A biopsy is important and required in the setting of suspected metastatic disease or when neoadjuvant treatment of borderline resectable GIST is being entertained.
    • The initial treatment of metastatic GIST should generally be tyrosine-kinase inhibitor therapy with imatinib. Patients with GISTs that appear to involve critical structures or are in challenging locations (eg, duodenal requiring Whipple) may benefit from neoadjuvant therapy. Prior to initiation of imatinib for either metastatic disease or in the neoadjuvant setting, a pretreatment biopsy is generally required to confirm the diagnosis prior to initiation of such treatment.
    • Biopsy may also be important when the diagnosis of GIST in is question, such as when the submucosal nature of this tumor is in doubt or when tumor characteristics as demonstrated by upper endoscopy and endoscopic ultrasonography are not typical. In specific patients, such as high-risk operative patients with small benign-appearing lesions and minimal or no symptoms, tissue diagnosis may help in further decision-making.
  • The 2 ways to obtain a preoperative histologic diagnosis are as follows:
    • Endoscopic biopsy: Preoperative endoscopic biopsy may be taken with or without EUS guidance. When taken without the help of EUS, endoscopic biopsy is not accurate and leads to a correct diagnosis in less than 50% of patients. Biopsies may miss the tumor and show only mucosal tissue. In addition, samples from the tumor itself are often too small to establish malignant nature. EUS-guided biopsy is more accurate. This technique can achieve a correct histologic diagnosis in more than 80% of cases and should be performed whenever preoperative histology seems necessary.
    • Percutaneous biopsy: Tumor biopsy can be obtained percutaneously under CT scanning or ultrasonographic guidance.[18] Consider this procedure in selected patients when endoscopic biopsy is impossible to perform or the results are negative.
Previous
Next

Histologic Findings

Cellular morphology as visualized by light microscopy can be variable. Most often, the tumors are highly cellular and composed of spindle-shaped cells that resemble smooth-muscle tissue, as depicted in the 1st image below. However, this histologic appearance is not uniform. A similar tumor with a predominant epithelioid component was historically diagnosed as leiomyoblastoma, as depicted in the 2nd image below. This variant is occasionally associated with a well-defined condition called Carney syndrome.

Photomicrograph of gastrointestinal stromal tumor Photomicrograph of gastrointestinal stromal tumor (GIST) stained with hematoxylin and eosin (H&E) and magnified 40X. Note the solid sheet of spindle cells.
Photomicrograph of gastric gastrointestinal stroma Photomicrograph of gastric gastrointestinal stromal tumor (GIST) stained with hematoxylin and eosin (H&E) and magnified 400X. This stromal tumor demonstrates spindle cells with epithelioid features.

Important histologic factors to consider in evaluating these tumors are mitotic index, cellularity, necrosis, nuclear atypia and nuclear-cytoplasmic ratio, cell shape, amount of stroma, and vascularity.

Investigations of GISTs by immunohistochemistry and electron microscopy (ultrastructural parameters) reveal phenotype variability that includes myoid, neural, and indeterminate characteristics.[19] Study of GISTs by immunohistochemistry methods reveals expression of CD117 and other various antigens, such as nestin (90-100% positivity), CD34 (70% positivity), CD44,[20] vimentin, desmin, muscle-specific actin, smooth-muscle actin, S-100 protein, neurofilament, neuron-specific enolase, and PGP9.5. CD117 plays an important role in the latest specific diagnostic criteria for GISTs. CD117 (c-kit protein) is a growth factor receptor with tyrosine-kinase activity and is a product of the proto-oncogene c-kit. CD117, although not tumor-specific, is expressed in all GISTs but not in true smooth muscle tumors and neural tumors.

CD117 has become a very important tool in the differentiation of GIST from other GI mesenchymal tumors.[21, 22] Positive CD117 staining in a spindle-shaped cell GI tumor is diagnostic for GIST, as depicted in the image below. CD34 is another important diagnostic marker. It is detected in approximately 70% of GISTs, and its presence may indicate a higher probability for a malignant phenotype. CD44 is variably expressed by GISTs, but its expression has been demonstrated to correlate with a better prognosis.

Photomicrograph of gastrointestinal stromal tumor Photomicrograph of gastrointestinal stromal tumor (GIST) with immunohistochemical staining for CD117. Note the strong positive staining of tumor cells with negative staining of the adjacent vessel. Positive stain for CD117 is diagnostic of GIST.

Recent studies suggest that GISTs may originate from the interstitial cells of Cajal. These cells are distributed along the GI tract and play a role in the control of gut motility. The interstitial cells of Cajal exhibit both myeloid and neural features and express the c-kit proto-oncogene receptor. However, the fact that GISTs are detected (although very rarely) outside of the GI tract (ie, omentum, mesentry, retroperitoneum) argues against this hypothesis.

Previous
Next

Staging

No consensus has been reached regarding a uniform staging system, and none of the currently used classifications is fully satisfactory. Most staging systems employ the 3 most important survival predictors—tumor size, histologic grade, and presence or absence of distant metastatic disease.

The new criteria for risk stratification of primary GIST have been incorporated into the AJCC staging seen below. A study by Rutkowski et al found that the AJCC risk classification after resection of primary GIST is reliable in terms of disease-free survival and overall survival.[23] The study concluded that patients with primary gastric GISTs had better prognosis than those with nongastric GISTs. Primary tumor size and mitotic activity were the most important prognostic factors in terms of disease-free survival in both groups.

The stratification is by mitotic index (5 or less or more than 5 per 50 HPF) and then further divided by tumor size, nodal disease, and tumor metastasis.[24] Gastric GISTs larger than 10 cm but 5 or less per 50 HPF mitotic index have only a 10% risk of progressive disease despite 34-57% risk of progressive disease in the other tumor locations. Gastric GISTs greater than 10 cm and a high mitotic index (>5 per 50 HPF), however, have an equally high risk of progressive disease (86%) as the other tumor locations.

Many studies have shown that tumor diameter greater than 5 cm is associated with increased risk for malignancy. However, relation of size to malignant potential may be gradual, with no clear cut-off point.

The number of mitotic figures is the most accepted index for grade classification, although other histologic parameters, such as cellularity, atypia, and necrosis, are also taken into consideration. A high mitotic index of more than 5 mitoses per 10 HPF usually signifies highly malignant disease. However, a low mitotic index is not always associated with benign course. As many as 25% of tumors with mitotic index of less than 5 mitoses per 10 HPF may manifest an aggressive biological behavior.

Tumor size

See the list below:

  • TX - Primary tumor cannot be assessed
  • T0 - No evidence of primary tumor
  • T1- Tumor smaller than 2 cm, localized
  • T2 - Tumor 2-5 cm
  • T3 - Tumor 5-10 cm
  • T4 - Tumor rupture more than 10 cm in greatest dimension

Regional lymph node

See the list below:

  • N0 - No regional lymph node metastasis
  • N1 - Regional lymph node metastasis

Metastasis

See the list below:

  • M0 - No distant metastasis
  • M1 - Distant metastases

Table 1. Staging System for Malignant Gastrointestinal Stromal Tumors (Open Table in a new window)

Group Tumor Size Regional Lymph Node Metastasis Mitosis
Stage IA T1 or T2 N0 M0 Low
Stage IB T3 N0 M0 High
  T1 N0 N0 High
Stage II T2 N0 M0 High
  T4 N0 M0 Low
Stage IIIA T3 N0 M0 High
Stage IIIB T4 N0 M0 High
  Any T N1 M0 Any rate
Stage IV Any T Any N M1 Any rate
Previous
 
 
Contributor Information and Disclosures
Author

Michael A Choti, MD, MBA, FACS Hall and Mary Lucile Shannon Professor and Chair, Department of Surgery, University of Texas Southwestern Medical Center

Michael A Choti, MD, MBA, FACS is a member of the following medical societies: American Association for the Study of Liver Diseases, American Surgical Association, International Hepato-Pancreato-Biliary Association, Americas Hepato-Pancreato-Biliary Association, American Society of Clinical Oncology, American College of Surgeons, Association for Academic Surgery, Society for Surgery of the Alimentary Tract, Society of Surgical Oncology, Society of University Surgeons

Disclosure: Nothing to disclose.

Coauthor(s)

Fabian M Johnston, MD, MHS, FACS Assistant Professor, Department of Surgery, Johns Hopkins University School of Medicine

Fabian M Johnston, MD, MHS, FACS is a member of the following medical societies: American Medical Association, National Medical Association, Society of Black Academic Surgeons, Society of Surgical Oncology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

N Joseph Espat, MD, MS, FACS Harold J Wanebo Professor of Surgery, Assistant Dean of Clinical Affairs, Boston University School of Medicine; Chairman, Department of Surgery, Director, Adele R Decof Cancer Center, Roger Williams Medical Center

N Joseph Espat, MD, MS, FACS is a member of the following medical societies: Alpha Omega Alpha, American Association for Cancer Research, American College of Surgeons, American Medical Association, American Society for Parenteral and Enteral Nutrition, American Society of Clinical Oncology, Americas Hepato-Pancreato-Biliary Association, Association for Academic Surgery, Central Surgical Association, Chicago Medical Society, International Hepato-Pancreato-Biliary Association, Pancreas Club, Sigma Xi, Society for Leukocyte Biology, Society for Surgery of the Alimentary Tract, Society of American Gastrointestinal and Endoscopic Surgeons, Society of Surgical Oncology, Society of University Surgeons, Southeastern Surgical Congress, Southern Medical Association, Surgical Infection Society

Disclosure: Nothing to disclose.

Additional Contributors

Robert C Shepard, MD, FACP Associate Professor of Medicine in Hematology and Oncology at University of North Carolina at Chapel Hill; Vice President of Scientific Affairs, Therapeutic Expertise, Oncology, at PRA International

Robert C Shepard, MD, FACP is a member of the following medical societies: American Association for Cancer Research, American Association for Physician Leadership, European Society for Medical Oncology, Association of Clinical Research Professionals, American Federation for Clinical Research, Eastern Cooperative Oncology Group, Society for Immunotherapy of Cancer, American Medical Informatics Association, American College of Physicians, American Federation for Medical Research, American Medical Association, American Society of Hematology, Massachusetts Medical Society

Disclosure: Nothing to disclose.

References
  1. Kang HJ, Koh KH, Yang E, You KT, Kim HJ, Paik YK. Differentially expressed proteins in gastrointestinal stromal tumors with KIT and PDGFRA mutations. Proteomics. 2006 Feb. 6(4):1151-7. [Medline].

  2. Medeiros F, Corless CL, Duensing A, Hornick JL, Oliveira AM, Heinrich MC. KIT-negative gastrointestinal stromal tumors: proof of concept and therapeutic implications. Am J Surg Pathol. 2004 Jul. 28(7):889-94. [Medline].

  3. 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 Oct. 13(10):1134-42. [Medline].

  4. Van Oosterom AT, Judson I, Verweij J, et al. STI 571, an active drug in metastatic gastrointestinal tumors (GIST), AN EORTC phase I study. Plenary Presentation. The American Society of Clinical Oncology, 37th Annual Meeting. 2001.

  5. Bauer S, Lang H, Schütte J, Hartmann JT. Complete remission with imatinib in metastastic gastrointestinal stromal tumors. J Clin Oncol. 2005 Sep 20. 23(27):6800-1; author reply 6801-2. [Medline].

  6. Benjamin RS, Blanke CD, Blay JY, Bonvalot S, Eisenberg B. Management of gastrointestinal stromal tumors in the imatinib era: selected case studies. Oncologist. 2006 Jan. 11(1):9-20. [Medline].

  7. Koh JS, Trent J, Chen L, et al. Gastrointestinal stromal tumors: overview of pathologic features, molecular biology, and therapy with imatinib mesylate. Histol Histopathol. 2004 Apr. 19(2):565-74. [Medline].

  8. Maki RG. Gastrointestinal Stromal Tumors Respond to Tyrosine Kinase-targeted Therapy. Curr Treat Options Gastroenterol. 2004 Feb. 7(1):13-17. [Medline].

  9. Melichar B, Voboril Z, Nozicka J, Ryska A, Urminská H, Vanecek T. Pathological complete response in advanced gastrointestinal stromal tumor after imatinib therapy. Intern Med. 2005 Nov. 44(11):1163-8. [Medline].

  10. DeMatteo RP, Ballman KV, Antonescu CR, et al. Adjuvant imatinib mesylate after resection of primary gastrointestinal stromal tumour: a randomized, double-blind, placebo-controlled trial. Lancet. March 28, 2009. 373:1097-1104. [Medline].

  11. Durham MM, Gow KW, Shehata BM, Katzenstein HM, Lorenzo RL, Ricketts RR. Gastrointestinal stromal tumors arising from the stomach: a report of three children. J Pediatr Surg. 2004 Oct. 39(10):1495-9. [Medline].

  12. Rege TA, Wagner AJ, Corless CL, Heinrich MC, Hornick JL. "Pediatric-type" gastrointestinal stromal tumors in adults: distinctive histology predicts genotype and clinical behavior. Am J Surg Pathol. 2011 Apr. 35(4):495-504. [Medline].

  13. Blanke C, Eisenberg BL, Heinrich M. Epidemiology of GIST. Am J Gastroenterol. 2005 Oct. 100(10):2366. [Medline].

  14. Graadt van Roggen JF, van Velthuysen ML, Hogendoorn PC. The histopathological differential diagnosis of gastrointestinal stromal tumours. J Clin Pathol. 2001 Feb. 54(2):96-102. [Medline].

  15. Chak A, Canto MI, Rosch T, et al. Endosonographic differentiation of benign and malignant stromal cell tumors. Gastrointest Endosc. 1997 Jun. 45(6):468-73. [Medline].

  16. Palazzo L, Landi B, Cellier C, et al. Endosonographic features predictive of benign and malignant gastrointestinal stromal cell tumours. Gut. 2000 Jan. 46(1):88-92. [Medline].

  17. [Guideline] NCCN Clinical Practice Guidelines in Oncology. Soft Tissue Sarcoma: Version 2.2014. National Comprehensive Cancer Network. Available at http://www.nccn.org/professionals/physician_gls/pdf/sarcoma.pdf. Accessed: February 4, 2015.

  18. Fields S, Libson E. CT-guided aspiration core needle biopsy of gastrointestinal wall lesions. J Comput Assist Tomogr. 2000 Mar-Apr. 24(2):224-8. [Medline].

  19. Kikuchi H, Yamashita K, Kawabata T, Yamamoto M, Hiramatsu Y, Kondo K. Immunohistochemical and genetic features of gastric and metastatic liver gastrointestinal stromal tumors: sequential analyses. Cancer Sci. 2006 Feb. 97(2):127-32. [Medline].

  20. Montgomery E, Abraham SC, Fisher C, et al. CD44 loss in gastric stromal tumors as a prognostic marker. Am J Surg Pathol. 2004 Feb. 28(2):168-77. [Medline].

  21. Franquemont DW. Differentiation and risk assessment of gastrointestinal stromal tumors. Am J Clin Pathol. 1995 Jan. 103(1):41-7. [Medline].

  22. Tzen CY, Mau BL. Analysis of CD117-negative gastrointestinal stromal tumors. World J Gastroenterol. 2005 Feb 21. 11(7):1052-5. [Medline].

  23. Rutkowski P, Wozniak A, Debiec-Rychter M, et al. Clinical utility of the new American Joint Committee on Cancer staging system for gastrointestinal stromal tumors: current overall survival after primary tumor resection. Cancer. 2011 Nov 1. 117(21):4916-24. [Medline].

  24. Rutkowski P, Wozniak A, Debiec-Rychter M, et al. Clinical utility of the new american joint committee on cancer staging system for gastrointestinal stromal tumors: Current Overall Survival After Primary Tumor Resection. Cancer. 2011 Mar 31. [Medline].

  25. 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 Apr 5. 344(14):1052-6. [Medline].

  26. Tuveson DA, Willis NA, Jacks T, et al. STI571 inactivation of the gastrointestinal stromal tumor c-KIT oncoprotein: biological and clinical implications. Oncogene. 2001 Aug 16. 20(36):5054-8. [Medline].

  27. Joensuu H, Eriksson M, Hatrmann I, et al. Twelve versus 36 months of adjuvant imatinib (IM) as treatment of operable GIST with a high risk of recurrence: Final results of a randomized trial (SSGXVIII/AIO). 2011 American Society of Clinical Oncology (ASCO) Annual Meeting. Abstract LBA1. Presented June 5, 2011.

  28. Verweij J, Casali PG, Zalcberg J, LeCesne A, Reichardt P, Blay JY. Progression-free survival in gastrointestinal stromal tumours with high-dose imatinib: randomised trial. Lancet. 2004 Sep 25-Oct 1. 364(9440):1127-34. [Medline].

  29. Demetri GD, Reichardt P, Kang YK, Blay JY, Rutkowski P, Gelderblom H, et al. 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 Jan 26. 381(9863):295-302. [Medline].

  30. Boni L, Benevento A, Dionigi G, Rovera F, Dionigi R. Surgical resection for gastrointestinal stromal tumors (GIST): experience on 25 patients. World J Surg Oncol. 2005. 3:78. [Medline].

  31. Bucher P, Egger JF, Gervaz P, Ris F, Weintraub D, Villiger P. An audit of surgical management of gastrointestinal stromal tumours (GIST). Eur J Surg Oncol. 2006 Apr. 32(3):310-4. [Medline].

  32. Knoop M, St Friedrichs K, Dierschke J. Surgical management of gastrointestinal stromal tumors of the stomach. Langenbecks Arch Surg. 2000 Apr. 385(3):194-8. [Medline].

  33. Kosmadakis N, Visvardis EE, Kartsaklis P, Tsimara M, Chatziantoniou A, Panopoulos I. The role of surgery in the management of gastrointestinal stromal tumors (GISTs) in the era of imatinib mesylate effectiveness. Surg Oncol. 2005 Aug. 14(2):75-84. [Medline].

  34. Lehnert T, Sinn HP, Waldherr R, Herfarth C. Surgical treatment of soft tissue tumors of the stomach. Eur J Surg Oncol. 1990 Aug. 16(4):352-9. [Medline].

  35. Peiper M, Schroder S, Zornig C. Stromal sarcoma of the stomach--a report of 20 surgically treated patients. Langenbecks Arch Surg. 1998 Dec. 383(6):442-6. [Medline].

  36. Katai H, Sasako M, Sano T. Wedge resection of the stomach for gastric leiomyosarcoma. Br J Surg. 1997 Apr. 84(4):560-1. [Medline].

  37. Eisenberg BL, Judson I. Surgery and imatinib in the management of GIST: emerging approaches to adjuvant and neoadjuvant therapy. Ann Surg Oncol. 2004 May. 11(5):465-75. [Medline].

  38. Rutkowski P, Nowecki Z, Nyckowski P, Dziewirski W, Grzesiakowska U, Nasierowska-Guttmejer A. Surgical treatment of patients with initially inoperable and/or metastatic gastrointestinal stromal tumors (GIST) during therapy with imatinib mesylate. J Surg Oncol. 2006 Mar 15. 93(4):304-11. [Medline].

  39. Chen H, Pruitt A, Nicol TL, et al. Complete hepatic resection of metastases from leiomyosarcoma prolongs survival. J Gastrointest Surg. 1998 Mar-Apr. 2(2):151-5. [Medline].

  40. Aogi K, Hirai T, Mukaida H, et al. Laparoscopic resection of submucosal gastric tumors. Surg Today. 1999. 29(2):102-6. [Medline].

  41. Bedard EL, Mamazza J, Schlachta CM, Poulin EC. Laparoscopic resection of gastrointestinal stromal tumors: not all tumors are created equal. Surg Endosc. 2006 Mar. 20(3):500-3. [Medline].

  42. Hindmarsh A, Koo B, Lewis MP, Rhodes M. Laparoscopic resection of gastric gastrointestinal stromal tumors. Surg Endosc. 2005 Aug. 19(8):1109-12. [Medline].

  43. Kitano S, Shiraishi N. Minimally invasive surgery for gastric tumors. Surg Clin North Am. 2005 Feb. 85(1):151-64, xi. [Medline].

  44. Nguyen SQ, Divino CM, Wang JL, Dikman SH. Laparoscopic management of gastrointestinal stromal tumors. Surg Endosc. 2006 May. 20(5):713-6. [Medline].

  45. Otani Y, Kitajima M. Laparoscopic surgery for GIST: too soon to decide. Gastric Cancer. 2005. 8(3):135-6. [Medline].

  46. Rosen MJ, Heniford BT. Endoluminal gastric surgery: the modern era of minimally invasive surgery. Surg Clin North Am. 2005 Oct. 85(5):989-1007, vii. [Medline].

  47. Aparicio T, Boige V, Sabourin JC, Crenn P, Ducreux M, Le Cesne A. Prognostic factors after surgery of primary resectable gastrointestinal stromal tumours. Eur J Surg Oncol. 2004 Dec. 30(10):1098-103. [Medline].

  48. Koga H, Ochiai A, Nakanishi Y, et al. Reevaluation of prognostic factors in gastric leiomyosarcoma. Am J Gastroenterol. 1995 Aug. 90(8):1307-12. [Medline].

  49. Ng EH, Pollock RE, Romsdahl MM. Prognostic implications of patterns of failure for gastrointestinal leiomyosarcomas. Cancer. 1992 Mar 15. 69(6):1334-41. [Medline].

  50. Ng EH, Pollock RE, Munsell MF, et al. Prognostic factors influencing survival in gastrointestinal leiomyosarcomas. Implications for surgical management and staging. Ann Surg. 1992 Jan. 215(1):68-77. [Medline].

  51. Shiu MH, Farr GH, Papachristou DN, Hajdu SI. Myosarcomas of the stomach: natural history, prognostic factors and management. Cancer. 1982 Jan 1. 49(1):177-87. [Medline].

  52. Bandoh T, Isoyama T, Toyoshima H. Submucosal tumors of the stomach: a study of 100 operative cases. Surgery. 1993 May. 113(5):498-506. [Medline].

  53. Blay JY, Bonvalot S, Casali P, Choi H, Debiec-Richter M, Dei Tos AP. 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 Apr. 16(4):566-78. [Medline].

  54. Carson W, Karakousis C, Douglass H, et al. Results of aggressive treatment of gastric sarcoma. Ann Surg Oncol. 1994 May. 1(3):244-51. [Medline].

  55. Corless CL, Fletcher JA, Heinrich MC. Biology of gastrointestinal stromal tumors. J Clin Oncol. 2004 Sep 15. 22(18):3813-25. [Medline].

  56. Cypriano MS, Jenkins JJ, Pappo AS, Rao BN, Daw NC. Pediatric gastrointestinal stromal tumors and leiomyosarcoma. Cancer. 2004 Jul 1. 101(1):39-50. [Medline].

  57. Darnell A, Dalmau E, Pericay C, Musulén E, Martín J, Puig J. Gastrointestinal stromal tumors. Abdom Imaging. 2006 Jul-Aug. 31(4):387-99. [Medline].

  58. de Mestier P, des Guetz G. Treatment of gastrointestinal stromal tumors with imatinib mesylate: a major breakthrough in the understanding of tumor-specific molecular characteristics. World J Surg. 2005/03. 29(3):357-61; discussion 362.

  59. Efron DT, Lillemoe KD. The current management of gastrointestinal stromal tumors. Adv Surg. 2005. 39:193-221. [Medline].

  60. Grant CS, Kim CH, Farrugia G, et al. Gastric leiomyosarcoma. Prognostic factors and surgical management. Arch Surg. 1991 Aug. 126(8):985-9; discussion 989-90. [Medline].

  61. Haider N, Kader M, Mc Dermott M, et al. Gastric stromal tumors in children. Pediatr Blood Cancer. 2004/02. 42(2):186-9.

  62. Hepworth CC, Menzies D, Motson RW. Minimally invasive surgery for posterior gastric stromal tumors. Surg Endosc. 2000 Apr. 14(4):349-53. [Medline].

  63. Hirota S, Isozaki K. Pathology of gastrointestinal stromal tumors. Pathol Int. 2006 Jan. 56(1):1-9. [Medline].

  64. Iwahashi M, Takifuji K, Ojima T, et al. Surgical management of small gastrointestinal stromal tumors of the stomach. World J Surg. 2006 Jan. 30(1):28-35. [Medline].

  65. King DM. The radiology of gastrointestinal stromal tumours (GIST). Cancer Imaging. 2005. 5:150-6. [Medline].

  66. Ludwig DJ, Traverso LW. Gut stromal tumors and their clinical behavior. Am J Surg. 1997 May. 173(5):390-4. [Medline].

  67. Matsui M, Goto H, Niwa Y, et al. Preliminary results of fine needle aspiration biopsy histology in upper gastrointestinal submucosal tumors. Endoscopy. 1998 Nov. 30(9):750-5. [Medline].

  68. Miettinen M, Lasota J. Gastrointestinal stromal tumors--definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis. Virchows Arch. 2001 Jan. 438(1):1-12. [Medline].

  69. Pidhorecky I, Cheney RT, Kraybill WG, Gibbs JF. Gastrointestinal stromal tumors: current diagnosis, biologic behavior, and management. Ann Surg Oncol. 2000 Oct. 7(9):705-12. [Medline].

  70. Rubin BP. Gastrointestinal stromal tumours: an update. Histopathology. 2006 Jan. 48(1):83-96. [Medline].

  71. Ruiz AR Jr, Nassar AJ, Fromm H. Multiple malignant gastric stromal tumors presenting with GI bleeding: a case report and a review of the literature. Gastrointest Endosc. 2000 Feb. 51(2):225-8. [Medline].

  72. Shah JN, Sun W, Seethala RR, Livolsi VA, Fry RD, Ginsberg GG. Neoadjuvant therapy with imatinib mesylate for locally advanced GI stromal tumor. Gastrointest Endosc. 2005 Apr. 61(4):625-7. [Medline].

  73. Shinomura Y, Kinoshita K, Tsutsui S, Hirota S. Pathophysiology, diagnosis, and treatment of gastrointestinal stromal tumors. J Gastroenterol. 2005 Aug. 40(8):775-80. [Medline].

  74. Stewart AE, Heslin MH, Arch J, Jhala N, Ragland B, Gomez F. Cyclooxygenase-2 expression and clinical outcome in gastrointestinal stromal tumors. J Gastrointest Surg. 2006 Feb. 10(2):315-9. [Medline].

  75. Sugár I, Forgács B, István G, Bognár G, Sápy Z, Ondrejka P. Gastrointestinal stromal tumors (GIST). Hepatogastroenterology. 2005 Mar-Apr. 52(62):409-13. [Medline].

  76. van der Zwan SM, DeMatteo RP. Gastrointestinal stromal tumor: 5 years later. Cancer. 2005 Nov 1. 104(9):1781-8. [Medline].

  77. Wang L, Vargas H, French SW. Cellular origin of gastrointestinal stromal tumors: a study of 27 cases. Arch Pathol Lab Med. 2000 Oct. 124(10):1471-5. [Medline].

  78. Warakaulle DR, Gleeson F. MDCT appearance of gastrointestinal stromal tumors after therapy with imatinib mesylate. AJR Am J Roentgenol. 2006 Feb. 186(2):510-5. [Medline].

  79. Wong NA, Young R, Malcomson RD, et al. Prognostic indicators for gastrointestinal stromal tumours: a clinicopathological and immunohistochemical study of 108 resected cases of the stomach. Histopathology. 2003 Aug. 43(2):118-26. [Medline].

  80. Wu L, Zhang Z, Yao H, Liu K, Wen Y, Xiong L. Clinical efficacy of second-generation tyrosine kinase inhibitors in imatinib-resistant gastrointestinal stromal tumors: a meta-analysis of recent clinical trials. Drug Des Devel Ther. 2014. 8:2061-7. [Medline]. [Full Text].

  81. Sineshaw HM, Jemal A, Lin CC, McGinnis LS, Ward EM. Contemporary Patterns and Survival Outcome of Adjuvant Systemic Therapy for Localized Gastrointestinal Stromal Tumors. Am J Clin Oncol. 2015 Jan 16. [Medline].

 
Previous
Next
 
Shown here is a gastric gastrointestinal stromal tumor (GIST). This is a gross specimen following partial gastrectomy. Note the submucosal tumor mass with the classic features of central umbilication and ulceration.
CT scan of the abdomen with oral contrast in a 60-year-old woman with a gastric gastrointestinal stromal tumor (GIST). A huge mass with central necrosis is observed originating from the gastric wall and narrowing its lumen. An ulcer crater can be identified within the mass (arrow).
Photomicrograph of gastrointestinal stromal tumor (GIST) stained with hematoxylin and eosin (H&E) and magnified 40X. Note the solid sheet of spindle cells.
Photomicrograph of gastric gastrointestinal stromal tumor (GIST) stained with hematoxylin and eosin (H&E) and magnified 400X. This stromal tumor demonstrates spindle cells with epithelioid features.
Photomicrograph of gastrointestinal stromal tumor (GIST) with immunohistochemical staining for CD117. Note the strong positive staining of tumor cells with negative staining of the adjacent vessel. Positive stain for CD117 is diagnostic of GIST.
Table 1. Staging System for Malignant Gastrointestinal Stromal Tumors
Group Tumor Size Regional Lymph Node Metastasis Mitosis
Stage IA T1 or T2 N0 M0 Low
Stage IB T3 N0 M0 High
  T1 N0 N0 High
Stage II T2 N0 M0 High
  T4 N0 M0 Low
Stage IIIA T3 N0 M0 High
Stage IIIB T4 N0 M0 High
  Any T N1 M0 Any rate
Stage IV Any T Any N M1 Any rate
Table 2. Five-Year Survival According to Size and Number of Mitoses
Size,



cm



Mitoses per 20 HPF 5-Year Survival Rate
< 6 < 4 97.5%
>6 < 4 91.5%
< 6 >4 80.0%
>6 >4 17.7%
Previous
Next
 
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.