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Carcinoids, GISTs, and Lymphomas of Colon and Rectum

  • David J. MaronEmail author
Chapter

Abstract

Less common tumors of the colon and rectum include carcinoids, gastrointestinal stromal tumors, and gastrointestinal lymphomas. Carcinoids are of neuroectodermal origin and frequently secrete bioactive compounds such as serotonin and histamine. Carcinoids that have metastasized to the liver may cause systemic symptoms referred to the carcinoid syndrome. Treatment of carcinoid tumors is surgical resection; however, the type of resection is dependent on the size and location of the tumor. Adjuvant treatment of carcinoid tumors involves the use of somatostatin analogues. Gastrointestinal stromal tumors occur less frequently in the colon and rectum than in other organs of the intestinal tract, but may be detected during endoscopic evaluation of the colon. Surgical treatment of GISTs also depends on the size and location of the tumor. Adjuvant treatment with imatinib (a tyrosine kinase inhibitor that blocks KIT activation) has been shown to reduce the risk of recurrence. The gastrointestinal tract is the most common site of extranodal lymphoma. Treatment typically involves a combination of surgical resection and systemic chemotherapy.

Keywords

Carcinoid Gastrointestinal stromal tumors (GIST) Lymphoma Colorectum Treatment TMN staging 

Supplementary material

Video 38.1

Transanal resection of a rectal GIST (Courtesy of Rodrigo Perez, M.D.) (MP4 287,260 kb)

References

  1. 1.
    Oberndorfer S. Karzinoide tumoren des dunndarms. Frankf Z Pathol. 1907;1:7.Google Scholar
  2. 2.
    Kulke MH, Mayer RJ. Carcinoid tumors. N Engl J Med. 1999;340(11):858–68.CrossRefGoogle Scholar
  3. 3.
    Ganim RB, Norton JA. Recent advances in carcinoid pathogenesis, diagnosis and management. Surg Oncol. 2000;9(4):173–9.CrossRefGoogle Scholar
  4. 4.
    Eriksson B, Oberg K, Stridsberg M. Tumor markers in neuroendocrine tumors. Digestion. 2000;62 Suppl 1:33–8.CrossRefGoogle Scholar
  5. 5.
    Lips CJ, Lentjes EG, Hoppener JW. The spectrum of carcinoid tumours and carcinoid syndromes. Ann Clin Biochem. 2003;40(Pt 6):612–27.CrossRefGoogle Scholar
  6. 6.
    Godwin 2nd JD. Carcinoid tumors. An analysis of 2,837 cases. Cancer. 1975;36(2):560–9.CrossRefGoogle Scholar
  7. 7.
    Modlin IM, Sandor A. An analysis of 8305 cases of carcinoid tumors. Cancer. 1997;79(4):813–29.CrossRefGoogle Scholar
  8. 8.
    Taghavi S, Jayarajan SN, Powers BD, Davey A, Willis AI. Examining rectal carcinoids in the era of screening colonoscopy: a surveillance, epidemiology, and end results analysis. Dis Colon Rectum. 2013;56(8):952–9.CrossRefGoogle Scholar
  9. 9.
    Habal N, Sims C, Bilchik AJ. Gastrointestinal carcinoid tumors and second primary malignancies. J Surg Oncol. 2000;75(4):310–6.CrossRefGoogle Scholar
  10. 10.
    Tichansky DS, Cagir B, Borrazzo E, Topham A, Palazzo J, Weaver EJ, et al. Risk of second cancers in patients with colorectal carcinoids. Dis Colon Rectum. 2002;45(1):91–7.CrossRefGoogle Scholar
  11. 11.
    Rosenberg JM, Welch JP. Carcinoid tumors of the colon. A study of 72 patients. Am J Surg. 1985;149(6):775–9.CrossRefGoogle Scholar
  12. 12.
    Orloff MJ. Carcinoid tumors of the rectum. Cancer. 1971;28(1):175–80.CrossRefGoogle Scholar
  13. 13.
    Ballantyne GH, Savoca PE, Flannery JT, Ahlman MH, Modlin IM. Incidence and mortality of carcinoids of the colon. Data from the Connecticut Tumor Registry. Cancer. 1992;69(10):2400–5.CrossRefGoogle Scholar
  14. 14.
    Murray SE, Lloyd RV, Sippel RS, Chen H. Clinicopathologic characteristics of colonic carcinoid tumors. J Surg Res. 2013;184(1):183–8.CrossRefGoogle Scholar
  15. 15.
    Matsui K, Iwase T, Kitagawa M. Small, polypoid-appearing carcinoid tumors of the rectum: clinicopathologic study of 16 cases and effectiveness of endoscopic treatment. Am J Gastroenterol. 1993;88(11):1949–53.PubMedGoogle Scholar
  16. 16.
    Pronay G, Nagy G, Ujszaszy L, Minik K. Carcinoid tumours of the rectum. Dtsch Z Verdau Stoffwechselkr. 1983;43(2):78–81.PubMedGoogle Scholar
  17. 17.
    Lucas KJ, Feldman JM. Flushing in the carcinoid syndrome and plasma kallikrein. Cancer. 1986;58(10):2290–3.CrossRefGoogle Scholar
  18. 18.
    Schreurs AJ, Westermann CJ, van den Bosch JM, Vanderschueren RG, Brutel de la Riviere A, Knaepen PJ. A twenty-five-year follow-up of ninety-three resected typical carcinoid tumors of the lung. J Thorac Cardiovasc Surg. 1992;104(5):1470–5.PubMedGoogle Scholar
  19. 19.
    Barclay TH, Schapira DV. Malignant tumors of the small intestine. Cancer. 1983;51(5):878–81.CrossRefGoogle Scholar
  20. 20.
    Yoshikane H, Tsukamoto Y, Niwa Y, Goto H, Hase S, Mizutani K, et al. Carcinoid tumors of the gastrointestinal tract: evaluation with endoscopic ultrasonography. Gastrointest Endosc. 1993;39(3):375–83.CrossRefGoogle Scholar
  21. 21.
    Kobayashi K, Katsumata T, Yoshizawa S, Sada M, Igarashi M, Saigenji K, et al. Indications of endoscopic polypectomy for rectal carcinoid tumors and clinical usefulness of endoscopic ultrasonography. Dis Colon Rectum. 2005;48(2):285–91.CrossRefGoogle Scholar
  22. 22.
    Feldman JM. Carcinoid tumors and syndrome. Semin Oncol. 1987;14(3):237–46.PubMedGoogle Scholar
  23. 23.
    Reubi JC, Laissue J, Waser B, Horisberger U, Schaer JC. Expression of somatostatin receptors in normal, inflamed, and neoplastic human gastrointestinal tissues. Ann N Y Acad Sci. 1994;733:122–37.CrossRefGoogle Scholar
  24. 24.
    Hoegerle S, Altehoefer C, Ghanem N, Koehler G, Waller CF, Scheruebl H, et al. Whole-body 18F dopa PET for detection of gastrointestinal carcinoid tumors. Radiology. 2001;220(2):373–80.CrossRefGoogle Scholar
  25. 25.
    Krausz Y, Freedman N, Rubinstein R, Lavie E, Orevi M, Tshori S, et al. 68Ga-DOTA-NOC PET/CT imaging of neuroendocrine tumors: comparison with (1)(1)(1)In-DTPA-octreotide (OctreoScan(R)). Mol Imaging Biol. 2011;13(3):583–93.CrossRefGoogle Scholar
  26. 26.
    Stinner B, Kisker O, Zielke A, Rothmund M. Surgical management for carcinoid tumors of small bowel, appendix, colon, and rectum. World J Surg. 1996;20(2):183–8.CrossRefGoogle Scholar
  27. 27.
    Memon MA, Nelson H. Gastrointestinal carcinoid tumors: current management strategies. Dis Colon Rectum. 1997;40(9):1101–18.CrossRefGoogle Scholar
  28. 28.
    Welch JP, Donaldson GA. Recent experience in the management of cancer of the colon and rectum. Am J Surg. 1974;127(3):258–66.CrossRefGoogle Scholar
  29. 29.
    Berardi RS. Carcinoid tumors of the colon (exclusive of the rectum): review of the literature. Dis Colon Rectum. 1972;15(5):383–91.CrossRefGoogle Scholar
  30. 30.
    Sanders RJ, Axtell HK. Carcinoids of the gastrointestinal tract. Surg Gynecol Obstet. 1964;119:369–80.PubMedGoogle Scholar
  31. 31.
    Spread C, Berkel H, Jewell L, Jenkins H, Yakimets W. Colon carcinoid tumors. A population-based study. Dis Colon Rectum. 1994;37(5):482–91.CrossRefGoogle Scholar
  32. 32.
    Al Natour RH, Saund MS, Sanchez VM, Whang EE, Sharma AM, Huang Q, et al. Tumor size and depth predict rate of lymph node metastasis in colon carcinoids and can be used to select patients for endoscopic resection. J Gastrointest Surg. 2012;16(3):595–602.CrossRefGoogle Scholar
  33. 33.
    Naunheim KS, Zeitels J, Kaplan EL, Sugimoto J, Shen KL, Lee CH, et al. Rectal carcinoid tumors—treatment and prognosis. Surgery. 1983;94(4):670–6.PubMedGoogle Scholar
  34. 34.
    Wang M, Peng J, Yang W, Chen W, Mo S, Cai S. Prognostic analysis for carcinoid tumours of the rectum: a single institutional analysis of 106 patients. Colorectal Dis. 2011;13(2):150–3.CrossRefGoogle Scholar
  35. 35.
    Wang YZ, Diebold A, Boudreaux P, Raines D, Campeau R, Anthony L, et al. Surgical treatment options for rectal carcinoid cancer: local versus low radical excision. Am Surg. 2014;80(1):31–5.PubMedGoogle Scholar
  36. 36.
    Shields CJ, Tiret E, Winter DC, International Rectal Carcinoid Study Group. Carcinoid tumors of the rectum: a multi-institutional international collaboration. Ann Surg. 2010;252(5):750–5.CrossRefGoogle Scholar
  37. 37.
    Yoon SN, Yu CS, Shin US, Kim CW, Lim SB, Kim JC. Clinicopathological characteristics of rectal carcinoids. Int J Colorectal Dis. 2010;25(9):1087–92.CrossRefGoogle Scholar
  38. 38.
    Sarmiento JM, Heywood G, Rubin J, Ilstrup DM, Nagorney DM, Que FG. Surgical treatment of neuroendocrine metastases to the liver: a plea for resection to increase survival. J Am Coll Surg. 2003;197(1):29–37.CrossRefGoogle Scholar
  39. 39.
    Christante D, Pommier S, Givi B, Pommier R. Hepatic artery chemoinfusion with chemoembolization for neuroendocrine cancer with progressive hepatic metastases despite octreotide therapy. Surgery. 2008;144(6):885–93. discussion 93-4.CrossRefGoogle Scholar
  40. 40.
    Gedaly R, Daily MF, Davenport D, McHugh PP, Koch A, Angulo P, et al. Liver transplantation for the treatment of liver metastases from neuroendocrine tumors: an analysis of the UNOS database. Arch Surg. 2011;146(8):953–8.CrossRefGoogle Scholar
  41. 41.
    Sun W, Lipsitz S, Catalano P, Mailliard JA, Haller DG, Eastern Cooperative Oncology Group. Phase II/III study of doxorubicin with fluorouracil compared with streptozocin with fluorouracil or dacarbazine in the treatment of advanced carcinoid tumors: Eastern Cooperative Oncology Group Study E1281. J Clin Oncol. 2005;23(22):4897–904.CrossRefGoogle Scholar
  42. 42.
    Moertel CG, Kvols LK, O’Connell MJ, Rubin J. Treatment of neuroendocrine carcinomas with combined etoposide and cisplatin. Evidence of major therapeutic activity in the anaplastic variants of these neoplasms. Cancer. 1991;68(2):227–32.CrossRefGoogle Scholar
  43. 43.
    Brizzi MP, Berruti A, Ferrero A, Milanesi E, Volante M, Castiglione F, et al. Continuous 5-fluorouracil infusion plus long acting octreotide in advanced well-differentiated neuroendocrine carcinomas. A phase II trial of the Piemonte oncology network. BMC Cancer. 2009;9:388.CrossRefGoogle Scholar
  44. 44.
    Modlin IM, Pavel M, Kidd M, Gustafsson BI. Review article: somatostatin analogues in the treatment of gastroenteropancreatic neuroendocrine (carcinoid) tumours. Aliment Pharmacol Ther. 2010;31(2):169–88.PubMedGoogle Scholar
  45. 45.
    Rinke A, Muller HH, Schade-Brittinger C, Klose KJ, Barth P, Wied M, et al. Placebo-controlled, double-blind, prospective, randomized study on the effect of octreotide LAR in the control of tumor growth in patients with metastatic neuroendocrine midgut tumors: a report from the PROMID Study Group. J Clin Oncol. 2009;27(28):4656–63.CrossRefGoogle Scholar
  46. 46.
    Di Bartolomeo M, Bajetta E, Zilembo N, de Braud F, Di Leo A, Verusio C, et al. Treatment of carcinoid syndrome with recombinant interferon alpha-2a. Acta Oncol. 1993;32(2):235–8.CrossRefGoogle Scholar
  47. 47.
    Kolby L, Persson G, Franzen S, Ahren B. Randomized clinical trial of the effect of interferon alpha on survival in patients with disseminated midgut carcinoid tumours. Br J Surg. 2003;90(6):687–93.CrossRefGoogle Scholar
  48. 48.
    Mazur MT, Clark HB. Gastric stromal tumors. Reappraisal of histogenesis. Am J Surg Pathol. 1983;7(6):507–19.CrossRefGoogle Scholar
  49. 49.
    Davila RE, Faigel DO. GI stromal tumors. Gastrointest Endosc. 2003;58(1):80–8.CrossRefGoogle Scholar
  50. 50.
    Steigen SE, Eide TJ. Gastrointestinal stromal tumors (GISTs): a review. APMIS. 2009;117(2):73–86.CrossRefGoogle Scholar
  51. 51.
    Kingham TP, DeMatteo RP. Multidisciplinary treatment of gastrointestinal stromal tumors. Surg Clin North Am. 2009;89(1):217–33. x.CrossRefGoogle Scholar
  52. 52.
    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.CrossRefGoogle Scholar
  53. 53.
    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(5):703–8.CrossRefGoogle Scholar
  54. 54.
    Goerres GW, Stupp R, Barghouth G, Hany TF, Pestalozzi B, Dizendorf E, et al. The value of PET, CT and in-line PET/CT in patients with gastrointestinal stromal tumours: long-term outcome of treatment with imatinib mesylate. Eur J Nucl Med Mol Imaging. 2005;32(2):153–62.CrossRefGoogle Scholar
  55. 55.
    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.CrossRefGoogle Scholar
  56. 56.
    Eisenberg BL, Judson I. Surgery and imatinib in the management of GIST: emerging approaches to adjuvant and neoadjuvant therapy. Ann Surg Oncol. 2004;11(5):465–75.CrossRefGoogle Scholar
  57. 57.
    Liu H, Yan Z, Liao G, Yin H. Treatment strategy of rectal gastrointestinal stromal tumor (GIST). J Surg Oncol. 2014;109(7):708–13.CrossRefGoogle Scholar
  58. 58.
    Changchien CR, Wu MC, Tasi WS, Tang R, Chiang JM, Chen JS, et al. Evaluation of prognosis for malignant rectal gastrointestinal stromal tumor by clinical parameters and immunohistochemical staining. Dis Colon Rectum. 2004;47(11):1922–9.CrossRefGoogle Scholar
  59. 59.
    DeMatteo RP, Ballman KV, Antonescu CR, Corless C, Kolesnikova V, von Mehren M, et al. Long-term results of adjuvant imatinib mesylate in localized, high-risk, primary gastrointestinal stromal tumor: ACOSOG Z9000 (Alliance) intergroup phase 2 trial. Ann Surg. 2013;258(3):422–9.CrossRefGoogle Scholar
  60. 60.
    Fernandes Gdos S, Blanke CD, Freitas D, Guedes R, Hoff PM. Perioperative treatment of gastrointestinal stromal tumors. Oncology. 2009;23(1):54–61.PubMedGoogle Scholar
  61. 61.
    Gold JS, Dematteo RP. Combined surgical and molecular therapy: the gastrointestinal stromal tumor model. Ann Surg. 2006;244(2):176–84.CrossRefGoogle Scholar
  62. 62.
    Wang JP, Wang T, Huang MJ, Wang L, Kang L, Wu XJ. The role of neoadjuvant imatinib mesylate therapy in sphincter-preserving procedures for anorectal gastrointestinal stromal tumor. Am J Clin Oncol. 2011;34(3):314–6.CrossRefGoogle Scholar
  63. 63.
    Tielen R, Verhoef C, van Coevorden F, Reyners AK, van der Graaf WT, Bonenkamp JJ, et al. Surgical management of rectal gastrointestinal stromal tumors. J Surg Oncol. 2013;107(4):320–3.CrossRefGoogle Scholar
  64. 64.
    Yeh CY, Chen HH, Tang R, Tasi WS, Lin PY, Wang JY. Surgical outcome after curative resection of rectal leiomyosarcoma. Dis Colon Rectum. 2000;43(11):1517–21.CrossRefGoogle Scholar
  65. 65.
    Verweij J, Casali PG, Zalcberg J, LeCesne A, Reichardt P, Blay JY, et al. Progression-free survival in gastrointestinal stromal tumours with high-dose imatinib: randomised trial. Lancet. 2004;364(9440):1127–34.CrossRefGoogle Scholar
  66. 66.
    Blanke CD, Rankin C, Demetri GD, Ryan CW, von Mehren M, Benjamin RS, et al. Phase III randomized, intergroup trial assessing imatinib mesylate at two dose levels in patients with unresectable or metastatic gastrointestinal stromal tumors expressing the kit receptor tyrosine kinase: S0033. J Clin Oncol. 2008;26(4):626–32.CrossRefGoogle Scholar
  67. 67.
    Koch P, del Valle F, Berdel WE, Willich NA, Reers B, Hiddemann W, et al. Primary gastrointestinal non-Hodgkin’s lymphoma: II. Combined surgical and conservative or conservative management only in localized gastric lymphoma—results of the prospective German Multicenter Study GIT NHL 01/92. J Clin Oncol. 2001;19(18):3874–83.CrossRefGoogle Scholar
  68. 68.
    Gustafsson BI, Siddique L, Chan A, Dong M, Drozdov I, Kidd M, et al. Uncommon cancers of the small intestine, appendix and colon: an analysis of SEER 1973–2004, and current diagnosis and therapy. Int J Oncol. 2008;33(6):1121–31.PubMedGoogle Scholar
  69. 69.
    Myung SJ, Joo KR, Yang SK, Jung HY, Chang HS, Lee HJ, et al. Clinicopathologic features of ileocolonic malignant lymphoma: analysis according to colonoscopic classification. Gastrointest Endosc. 2003;57(3):343–7.CrossRefGoogle Scholar
  70. 70.
    Howell JM, Auer-Grzesiak I, Zhang J, Andrews CN, Stewart D, Urbanski SJ. Increasing incidence rates, distribution and histological characteristics of primary gastrointestinal non-Hodgkin lymphoma in a North American population. Can J Gastroenterol. 2012;26(7):452–6.CrossRefGoogle Scholar
  71. 71.
    Shaheen S, Guddati AK. Secondary mucosa-associated lymphoid tissue (MALT) lymphoma of the colon. Med Oncol. 2013;30(2):502.CrossRefGoogle Scholar
  72. 72.
    Dawson IM, Cornes JS, Morson BC. Primary malignant lymphoid tumours of the intestinal tract. Report of 37 cases with a study of factors influencing prognosis. Br J Surg. 1961;49:80–9.CrossRefGoogle Scholar
  73. 73.
    Fan CW, Changchien CR, Wang JY, Chen JS, Hsu KC, Tang R, et al. Primary colorectal lymphoma. Dis Colon Rectum. 2000;43(9):1277–82.CrossRefGoogle Scholar
  74. 74.
    Dionigi G, Annoni M, Rovera F, Boni L, Villa F, Castano P, et al. Primary colorectal lymphomas: review of the literature. Surg Oncol. 2007;16 Suppl 1:S169–71.CrossRefGoogle Scholar
  75. 75.
    Henry CA, Berry RE. Primary lymphoma of the large intestine. Am Surg. 1988;54(5):262–6.PubMedGoogle Scholar
  76. 76.
    Devine RM, Beart Jr RW, Wolff BG. Malignant lymphoma of the rectum. Dis Colon Rectum. 1986;29(12):821–4.CrossRefGoogle Scholar
  77. 77.
    Aviles A, Neri N, Huerta-Guzman J. Large bowel lymphoma: an analysis of prognostic factors and therapy in 53 patients. J Surg Oncol. 2002;80(2):111–5.CrossRefGoogle Scholar
  78. 78.
    Jinnai D, Iwasa Z, Watanuki T. Malignant lymphoma of the large intestine—operative results in Japan. Jpn J Surg. 1983;13(4):331–6.CrossRefGoogle Scholar
  79. 79.
    Lai YL, Lin JK, Liang WY, Huang YC, Chang SC. Surgical resection combined with chemotherapy can help achieve better outcomes in patients with primary colonic lymphoma. J Surg Oncol. 2011;104(3):265–8.CrossRefGoogle Scholar
  80. 80.
    Kim YH, Lee JH, Yang SK, Kim TI, Kim JS, Kim HJ, et al. Primary colon lymphoma in Korea: a KASID (Korean Association for the Study of Intestinal Diseases) study. Dig Dis Sci. 2005;50(12):2243–7.CrossRefGoogle Scholar

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Authors and Affiliations

  1. 1.Department of Colorectal SurgeryCleveland Clinic FloridaWestonUSA

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