Carcinoid and Neuroendocrine Tumors of the Colon and Rectum

ABSTRACT

Carcinoid and neuroendocrine tumors of the colon and rectum arise from the amine precursor uptake and decarboxylation (APUD) cells of the intestine. Carcinoid tumors are most commonly found in the gastrointestinal tract and are located in decreasing order of frequency in appendix, ileum, rectum, stomach, and colon. The vast majority of lesions are asymptomatic and are found incidentally during endoscopy. The management of these lesions depends upon the size of the lesion, involvement of the muscularis, location, and presence of metastatic disease. Small lesions (1 cm) can often be treated locally, either endoscopically or transanally. However, larger lesions (> 2 cm) require a formal oncologic resection. Adjuvant therapy is indicated only for metastatic disease, and admirable advances have been made in the realm of chemotherapy for reduction of disease and palliation of the symptoms of carcinoid syndrome. In this article, we discuss the nature of these interesting and uncommon tumors, clinical presentation, treatment options, and prognosis.

KEYWORDS

Carcinoid - neuroendocrine tumor - colon - rectum

REFERENCES

• 1 Caplin M E, Buscombe J R, Hilson A J, Jones A L, Watkinson A F, Burroughs A K. Carcinoid tumour.  Lancet. 1998;  352 799-805
• 2 Kloppel G, Perren A, Heitz P U. The gastroenteropancreatic neuroendocrine cell system and its tumors: the WHO classification.  Ann NY Acad Sci. 2004;  1014 13-27
• 3 Zhao J, de Krijger R R, Meier D et al.. Genomic alterations in well-differentiated gastrointestinal and bronchial neuroendocrine tumors (carcinoids): marked differences indicating diversity in molecular pathogenesis.  Am J Pathol. 2000;  157 1431-1438
• 4 Pearse A G. The APUD concept and hormone production.  Clin Endocrinol Metab. 1980;  9 211-222
• 5 Williams E D, Sandler M. The classification of carcinoid tumours.  Lancet. 1963;  1 238-239
• 6 Kytola S, Hoog A, Nord B et al.. Comparative genomic hybridization identifies loss of 18q22-qter as an early and specific event in tumorigenesis of midgut carcinoids.  Am J Pathol. 2001;  158 1803-1808
• 7 Duh Q Y, Hybarger C P, Geist R et al.. Carcinoids associated with multiple endocrine neoplasia syndromes.  Am J Surg. 1987;  154 142-148
• 8 Jakobovitz O, Nass D, DeMarco L et al.. Carcinoid tumors frequently display genetic abnormalities involving chromosome 11.  J Clin Endocrinol Metab. 1996;  81 3164-3167
• 9 Modlin I M, Sandor A. An analysis of 8305 cases of carcinoid tumors.  Cancer. 1997;  79 813-829
• 10 Modlin I M, Lye K D, Kidd M. A 5-decade analysis of 13,715 carcinoid tumors.  Cancer. 2003;  97 934-959
• 11 Hay D J, Curt J R. Carcinoid tumour as a complication of ulcerative colitis.  Postgrad Med J. 1979;  55 430-432
• 12 Dodd S M. Chronic ulcerative colitis complicated by atypical carcinoid tumour.  J Clin Pathol. 1986;  39 913-916
• 13 Owen D A, Hwang W S, Thorlakson R H, Walli E. Malignant carcinoid tumor complicating chronic ulcerative colitis.  Am J Clin Pathol. 1981;  76 333-338
• 14 Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 28-2000. A 34-year-old man with ulcerative colitis and a large perirectal mass.  N Engl J Med. 2000;  343 794-800
• 15 Berge T, Linell F. Carcinoid tumours. Frequency in a defined population during a 12-year period.  Acta Pathol Microbiol Scand [A]. 1976;  84 322-330
• 16 Warner T F, O'Reilly G, Lee G A. Mesenteric occlusive lesion and ileal carcinoids.  Cancer. 1979;  44 758-762
• 17 Marshall J B, Bodnarchuk G. Carcinoid tumors of the gut. Our experience over three decades and review of the literature.  J Clin Gastroenterol. 1993;  16 123-129
• 18 Tormey W P, FitzGerald R J. The clinical and laboratory correlates of an increased urinary 5-hydroxyindoleacetic acid.  Postgrad Med J. 1995;  71 542-545
• 19 Eriksson B, Oberg K, Stridsberg M. Tumor markers in neuroendocrine tumors.  Digestion. 2000;  62(Suppl 1) 33-38
• 20 Janson E T, Holmberg L, Stridsberg M et al.. Carcinoid tumors: analysis of prognostic factors and survival in 301 patients from a referral center.  Ann Oncol. 1997;  8 685-690
• 21 Pantongrag-Brown L, Buetow P C, Carr N J, Lichtenstein J E, Buck J L. Calcification and fibrosis in mesenteric carcinoid tumor: CT findings and pathologic correlation.  AJR Am J Roentgenol. 1995;  164 387-391
• 22 Modlin I M, Tang L H. Approaches to the diagnosis of gut neuroendocrine tumors: the last word (today).  Gastroenterology. 1997;  112 583-590
• 23 Modlin I M, Kidd M, Latich I, Zikusoka M N, Shapiro M D. Current status of gastrointestinal carcinoids.  Gastroenterology. 2005;  128 1717-1751
• 24 Ballantyne G H, Savoca P E, Flannery J T, Ahlman M H, Modlin I M. Incidence and mortality of carcinoids of the colon. Data from the Connecticut Tumor Registry.  Cancer. 1992;  69 2400-2405
• 25 Crocetti E, Paci E. Malignant carcinoids in the USA, SEER 1992-1999. An epidemiological study with 6830 cases.  Eur J Cancer Prev. 2003;  12 191-194
• 26 Kulke M H, Mayer R J. Carcinoid tumors.  N Engl J Med. 1999;  340 858-868
• 27 Hellman P, Lundstrom T, Ohrvall U et al.. Effect of surgery on the outcome of midgut carcinoid disease with lymph node and liver metastases.  World J Surg. 2002;  26 991-997
• 28 Rosenberg J M, Welch J P. Carcinoid tumors of the colon. A study of 72 patients.  Am J Surg. 1985;  149 775-779
• 29 Spread C, Berkel H, Jewell L, Jenkins H, Yakimets W. Colon carcinoid tumors. A population-based study.  Dis Colon Rectum. 1994;  37 482-491
• 30 Jetmore A B, Ray J E, Gathright Jr J B, McMullen K M, Hicks T C, Timmcke A E. Rectal carcinoids: the most frequent carcinoid tumor.  Dis Colon Rectum. 1992;  35 717-725
• 31 Soga J. Carcinoids of the colon and ileocecal region: a statistical evaluation of 363 cases collected from the literature.  J Exp Clin Cancer Res. 1998;  17 139-148
• 32 Mani S, Modlin I M, Ballantyne G, Ahlman H, West B. Carcinoids of the rectum.  J Am Coll Surg. 1994;  179 231-248
• 33 Shirouzu K, Isomoto H, Kakegawa T, Morimatsu M. Treatment of rectal carcinoid tumors.  Am J Surg. 1990;  160 262-265
• 34 Koura A N, Giacco G G, Curley S A, Skibber J M, Feig B W, Ellis L M. Carcinoid tumors of the rectum: effect of size, histopathology, and surgical treatment on metastasis free survival.  Cancer. 1997;  79 1294-1298
• 35 Sauven P, Ridge J A, Quan S H, Sigurdson E R. Anorectal carcinoid tumors. Is aggressive surgery warranted?.  Ann Surg. 1990;  211 67-71
• 36 Eriksson B, Arnberg H, Oberg K et al.. A polyclonal antiserum against chromogranin A and B-a new sensitive marker for neuroendocrine tumours.  Acta Endocrinol (Copenh). 1990;  122 145-155
• 37 Que F G, Nagorney D M, Batts K P, Linz L J, Kvols L K. Hepatic resection for metastatic neuroendocrine carcinomas.  Am J Surg. 1995;  169 36-42 discussion 42-33