eMedicine Specialties > Oncology > Carcinomas of the Gastrointestinal Tract
Gastric Gastrointestinal Stromal Tumors: Follow-up
Updated: Jun 4, 2009
Follow-up
Further Outpatient Care
Follow-up care after curative operations is important because certain patients with recurrent disease may benefit from second surgical intervention and from systemic therapy with imatinib mesylate or sunitinib malate for unresectable and/or metastatic disease. Follow-up includes physical examination and periodical gastroscopies as well as CT scanning. Ideal time intervals for performing these studies have not been well established.
Prognosis
- In general, long-term survival for malignant GIST after a curative-intent surgery is strongly related to tumor size, mitotic rate, and tumor location.44,45,46 Gastric GISTs tend to act less aggressively than small bowel GISTs of comparable size and mitotic rate.
- Because no standardized staging system exists for stromal tumors of the GI tract and most series are small and heterogenous, comparison of the different published survival rates is difficult. However, various reports of 5-year survival rates after R0 resection for gastrointestinal stromal tumors range from 32-93%. In large series, this rate is about 50-60%. The median survival after palliative resection is about 10 months, with a 5-year survival rate as high as 10%. These rates improve with the addition of imatinib.
- The NCCN criteria for risk stratification of primary GIST have not been incorporated into the AJCC staging but may be more helpful in determining individual risk for progressive disease, after margin-negative resection.16 The stratification is by mitotic index (5 or less or more than 5 per 50 HPF) and then further divided by tumor size (2 cm or less or more than 2 cm; 5 cm or less or more than 5 cm; 10 cm or less or more than 10 cm) and tumor location (gastric, duodenum, jejunum-ileum, and rectum). Gastric GISTs greater than 10 cm but less than or equal to 5 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.
- Ng et al in 1992 have reported the long-term survival of 139 patients with gastrointestinal malignant stromal tumors from different sites—40% gastric tumors. The overall 5-year survival rates by stage for GI stromal tumors is as follows:47
- Stage I - 75%
- Stage II - 52%
- Stage III - 28%
- Stage IVa - 12%
- Stage IVb - 7%
- In another large series of patients after resection of malignant GISTs published by Koga et al in 1995, survival was studied according to a classification combining tumor size and mitotic index. A very high survival rate was found in patients with tumors smaller than 6 cm and low mitotic index.45 Table 2. Five-Year Survival According to Size and Number of Mitoses
Open table in new window
[ CLOSE WINDOW ]Table
Size,
cmMitoses per 20 HPF 5-Year Survival Rate <6 <4 97.5% >6 <4 91.5% <6 >4 80.0% >6 >4 17.7% Size,
cmMitoses per 20 HPF 5-Year Survival Rate <6 <4 97.5% >6 <4 91.5% <6 >4 80.0% >6 >4 17.7% - Histologic grade alone is a strong prognostic factor. In 1982, Shiu et al reported a 5-year survival rate of 80% in patients after resection of low-grade tumors (low mitotic index, no necrosis). The 5-year survival rate dropped to 32% in patients with high-grade tumors (high mitotic index, regions of necrosis).48
- Other factors found to have a negative impact on prognosis are tumor rupture during operation, involvement of histologic margins, and lymph node involvement.
- The liver and the peritoneal cavity represent the predominant sites of recurrence after attempted curative surgery. Extra-abdominal sites (eg, lungs) are less common. Evaluate patients with recurrent disease for possible second resection if feasible. Survival prolongation is reported for resected local recurrences and even for resected isolated hepatic or peritoneal recurrent lesions.
Miscellaneous
Medicolegal Pitfalls
- No consensus has been reached regarding a uniform staging system, and none of the currently used classifications is fully satisfactory.
- No standard regimen for adjuvant therapy presently exists for malignant gastric stromal tumors. While imatinib has been approved as adjuvant therapy for resected GIST, the optimal duration of therapy remains unknown. It is also unclear as to which population of patients should receive adjuvant therapy, as the initial phase III randomized controlled trial stratified outcomes only by size and not mitotic index or tumor location.
- Direct every effort at avoiding tumor rupture during surgical therapy. Tumor rupture is associated with a worse prognosis because of peritoneal seeding.
- Because malignant potential is difficult to determine preoperatively, a wide resection with clear margins is routinely indicated.
- Malignant behavior in low mitotic tumors smaller than 2 cm is rare but is reported.
More on Gastric Gastrointestinal Stromal Tumors |
| Overview: Gastric Gastrointestinal Stromal Tumors |
| Differential Diagnoses & Workup: Gastric Gastrointestinal Stromal Tumors |
| Treatment & Medication: Gastric Gastrointestinal Stromal Tumors |
Follow-up: Gastric Gastrointestinal Stromal Tumors |
| Multimedia: Gastric Gastrointestinal Stromal Tumors |
| References |
| « Previous Page | Next Page » |
References
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. Feb 2006;6(4):1151-7. [Medline].
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. Jul 2004;28(7):889-94. [Medline].
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. Oct 2000;13(10):1134-42. [Medline].
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.
Bauer S, Lang H, Schütte J, Hartmann JT. Complete remission with imatinib in metastastic gastrointestinal stromal tumors. J Clin Oncol. Sep 20 2005;23(27):6800-1; author reply 6801-2. [Medline].
Benjamin RS, Blanke CD, Blay JY, Bonvalot S, Eisenberg B. Management of gastrointestinal stromal tumors in the imatinib era: selected case studies. Oncologist. Jan 2006;11(1):9-20. [Medline].
Koh JS, Trent J, Chen L, et al. Gastrointestinal stromal tumors: overview of pathologic features, molecular biology, and therapy with imatinib mesylate. Histol Histopathol. Apr 2004;19(2):565-74. [Medline].
Maki RG. Gastrointestinal Stromal Tumors Respond to Tyrosine Kinase-targeted Therapy. Curr Treat Options Gastroenterol. Feb 2004;7(1):13-17. [Medline].
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. Nov 2005;44(11):1163-8. [Medline].
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].
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. Oct 2004;39(10):1495-9. [Medline].
Blanke C, Eisenberg BL, Heinrich M. Epidemiology of GIST. Am J Gastroenterol. Oct 2005;100(10):2366. [Medline].
Graadt van Roggen JF, van Velthuysen ML, Hogendoorn PC. The histopathological differential diagnosis of gastrointestinal stromal tumours. J Clin Pathol. Feb 2001;54(2):96-102. [Medline].
Chak A, Canto MI, Rosch T, et al. Endosonographic differentiation of benign and malignant stromal cell tumors. Gastrointest Endosc. Jun 1997;45(6):468-73. [Medline].
Palazzo L, Landi B, Cellier C, et al. Endosonographic features predictive of benign and malignant gastrointestinal stromal cell tumours. Gut. Jan 2000;46(1):88-92. [Medline].
[Guideline] Demetri GD, Benjamin RS, Blanke CD, 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 (2):S1-S29. [Medline].
Fields S, Libson E. CT-guided aspiration core needle biopsy of gastrointestinal wall lesions. J Comput Assist Tomogr. Mar-Apr 2000;24(2):224-8. [Medline].
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. Feb 2006;97(2):127-32. [Medline].
Montgomery E, Abraham SC, Fisher C, et al. CD44 loss in gastric stromal tumors as a prognostic marker. Am J Surg Pathol. Feb 2004;28(2):168-77. [Medline].
Franquemont DW. Differentiation and risk assessment of gastrointestinal stromal tumors. Am J Clin Pathol. Jan 1995;103(1):41-7. [Medline].
Tzen CY, Mau BL. Analysis of CD117-negative gastrointestinal stromal tumors. World J Gastroenterol. Feb 21 2005;11(7):1052-5. [Medline].
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. Apr 5 2001;344(14):1052-6. [Medline].
Tuveson DA, Willis NA, Jacks T, et al. STI571 inactivation of the gastrointestinal stromal tumor c-KIT oncoprotein: biological and clinical implications. Oncogene. Aug 16 2001;20(36):5054-8. [Medline].
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. Sep 25-Oct 1 2004;364(9440):1127-34. [Medline].
Negri T, Pavan GM, Virdis E, Greco A, Fermeglia M, Sandri M, et al. T670X KIT mutations in gastrointestinal stromal tumors: making sense of missense. J Natl Cancer Inst. Feb 4 2009;101(3):194-204. [Medline].
Schittenhelm MM, Shiraga S, Schroeder A, Corbin AS, Griffith D, Lee FY. Dasatinib (BMS-354825), a dual SRC/ABL kinase inhibitor, inhibits the kinase activity of wild-type, juxtamembrane, and activation loop mutant KIT isoforms associated with human malignancies. Cancer Res. Jan 1 2006;66(1):473-81. [Medline].
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].
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. Apr 2006;32(3):310-4. [Medline].
Knoop M, St Friedrichs K, Dierschke J. Surgical management of gastrointestinal stromal tumors of the stomach. Langenbecks Arch Surg. Apr 2000;385(3):194-8. [Medline].
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. Aug 2005;14(2):75-84. [Medline].
Lehnert T, Sinn HP, Waldherr R, Herfarth C. Surgical treatment of soft tissue tumors of the stomach. Eur J Surg Oncol. Aug 1990;16(4):352-9. [Medline].
Peiper M, Schroder S, Zornig C. Stromal sarcoma of the stomach--a report of 20 surgically treated patients. Langenbecks Arch Surg. Dec 1998;383(6):442-6. [Medline].
Katai H, Sasako M, Sano T. Wedge resection of the stomach for gastric leiomyosarcoma. Br J Surg. Apr 1997;84(4):560-1. [Medline].
Eisenberg BL, Judson I. Surgery and imatinib in the management of GIST: emerging approaches to adjuvant and neoadjuvant therapy. Ann Surg Oncol. May 2004;11(5):465-75. [Medline].
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. Mar 15 2006;93(4):304-11. [Medline].
Chen H, Pruitt A, Nicol TL, et al. Complete hepatic resection of metastases from leiomyosarcoma prolongs survival. J Gastrointest Surg. Mar-Apr 1998;2(2):151-5. [Medline].
Aogi K, Hirai T, Mukaida H, et al. Laparoscopic resection of submucosal gastric tumors. Surg Today. 1999;29(2):102-6. [Medline].
Bedard EL, Mamazza J, Schlachta CM, Poulin EC. Laparoscopic resection of gastrointestinal stromal tumors: not all tumors are created equal. Surg Endosc. Mar 2006;20(3):500-3. [Medline].
Hindmarsh A, Koo B, Lewis MP, Rhodes M. Laparoscopic resection of gastric gastrointestinal stromal tumors. Surg Endosc. Aug 2005;19(8):1109-12. [Medline].
Kitano S, Shiraishi N. Minimally invasive surgery for gastric tumors. Surg Clin North Am. Feb 2005;85(1):151-64, xi. [Medline].
Nguyen SQ, Divino CM, Wang JL, Dikman SH. Laparoscopic management of gastrointestinal stromal tumors. Surg Endosc. May 2006;20(5):713-6. [Medline].
Otani Y, Kitajima M. Laparoscopic surgery for GIST: too soon to decide. Gastric Cancer. 2005;8(3):135-6. [Medline].
Rosen MJ, Heniford BT. Endoluminal gastric surgery: the modern era of minimally invasive surgery. Surg Clin North Am. Oct 2005;85(5):989-1007, vii. [Medline].
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. Dec 2004;30(10):1098-103. [Medline].
Koga H, Ochiai A, Nakanishi Y, et al. Reevaluation of prognostic factors in gastric leiomyosarcoma. Am J Gastroenterol. Aug 1995;90(8):1307-12. [Medline].
Ng EH, Pollock RE, Romsdahl MM. Prognostic implications of patterns of failure for gastrointestinal leiomyosarcomas. Cancer. Mar 15 1992;69(6):1334-41. [Medline].
Ng EH, Pollock RE, Munsell MF, et al. Prognostic factors influencing survival in gastrointestinal leiomyosarcomas. Implications for surgical management and staging. Ann Surg. Jan 1992;215(1):68-77. [Medline].
Shiu MH, Farr GH, Papachristou DN, Hajdu SI. Myosarcomas of the stomach: natural history, prognostic factors and management. Cancer. Jan 1 1982;49(1):177-87. [Medline].
Bandoh T, Isoyama T, Toyoshima H. Submucosal tumors of the stomach: a study of 100 operative cases. Surgery. May 1993;113(5):498-506. [Medline].
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. Apr 2005;16(4):566-78. [Medline].
Carson W, Karakousis C, Douglass H, et al. Results of aggressive treatment of gastric sarcoma. Ann Surg Oncol. May 1994;1(3):244-51. [Medline].
Corless CL, Fletcher JA, Heinrich MC. Biology of gastrointestinal stromal tumors. J Clin Oncol. Sep 15 2004;22(18):3813-25. [Medline].
Cypriano MS, Jenkins JJ, Pappo AS, Rao BN, Daw NC. Pediatric gastrointestinal stromal tumors and leiomyosarcoma. Cancer. Jul 1 2004;101(1):39-50. [Medline].
Darnell A, Dalmau E, Pericay C, Musulén E, Martín J, Puig J. Gastrointestinal stromal tumors. Abdom Imaging. Jul-Aug 2006;31(4):387-99. [Medline].
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.
Efron DT, Lillemoe KD. The current management of gastrointestinal stromal tumors. Adv Surg. 2005;39:193-221. [Medline].
Grant CS, Kim CH, Farrugia G, et al. Gastric leiomyosarcoma. Prognostic factors and surgical management. Arch Surg. Aug 1991;126(8):985-9; discussion 989-90. [Medline].
Haider N, Kader M, Mc Dermott M, et al. Gastric stromal tumors in children. Pediatr Blood Cancer. 2004/02;42(2):186-9.
Hepworth CC, Menzies D, Motson RW. Minimally invasive surgery for posterior gastric stromal tumors. Surg Endosc. Apr 2000;14(4):349-53. [Medline].
Hirota S, Isozaki K. Pathology of gastrointestinal stromal tumors. Pathol Int. Jan 2006;56(1):1-9. [Medline].
Iwahashi M, Takifuji K, Ojima T, et al. Surgical management of small gastrointestinal stromal tumors of the stomach. World J Surg. Jan 2006;30(1):28-35. [Medline].
King DM. The radiology of gastrointestinal stromal tumours (GIST). Cancer Imaging. 2005;5:150-6. [Medline].
Ludwig DJ, Traverso LW. Gut stromal tumors and their clinical behavior. Am J Surg. May 1997;173(5):390-4. [Medline].
Matsui M, Goto H, Niwa Y, et al. Preliminary results of fine needle aspiration biopsy histology in upper gastrointestinal submucosal tumors. Endoscopy. Nov 1998;30(9):750-5. [Medline].
Miettinen M, Lasota J. Gastrointestinal stromal tumors--definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis. Virchows Arch. Jan 2001;438(1):1-12. [Medline].
Pidhorecky I, Cheney RT, Kraybill WG, Gibbs JF. Gastrointestinal stromal tumors: current diagnosis, biologic behavior, and management. Ann Surg Oncol. Oct 2000;7(9):705-12. [Medline].
Rubin BP. Gastrointestinal stromal tumours: an update. Histopathology. Jan 2006;48(1):83-96. [Medline].
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. Feb 2000;51(2):225-8. [Medline].
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. Apr 2005;61(4):625-7. [Medline].
Shinomura Y, Kinoshita K, Tsutsui S, Hirota S. Pathophysiology, diagnosis, and treatment of gastrointestinal stromal tumors. J Gastroenterol. Aug 2005;40(8):775-80. [Medline].
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. Feb 2006;10(2):315-9. [Medline].
Sugár I, Forgács B, István G, Bognár G, Sápy Z, Ondrejka P. Gastrointestinal stromal tumors (GIST). Hepatogastroenterology. Mar-Apr 2005;52(62):409-13. [Medline].
van der Zwan SM, DeMatteo RP. Gastrointestinal stromal tumor: 5 years later. Cancer. Nov 1 2005;104(9):1781-8. [Medline].
Wang L, Vargas H, French SW. Cellular origin of gastrointestinal stromal tumors: a study of 27 cases. Arch Pathol Lab Med. Oct 2000;124(10):1471-5. [Medline].
Warakaulle DR, Gleeson F. MDCT appearance of gastrointestinal stromal tumors after therapy with imatinib mesylate. AJR Am J Roentgenol. Feb 2006;186(2):510-5. [Medline].
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. Aug 2003;43(2):118-26. [Medline].
Further Reading
Keywords
gastric gastrointestinal stromal tumors, gastric GISTs, malignant gastric stromal tumors, gastric leiomyosarcomas, gastrointestinal stromal tumors, GISTs, gastric GIST, fibrosarcoma, angiosarcoma, hemangiopericytoma, gastric smooth muscle tumors, intestinal smooth muscle tumors
Follow-up: Gastric Gastrointestinal Stromal Tumors