UpToDate
Official reprint from UpToDate®
www.uptodate.com ©2016 UpToDate®

Treatment of small bowel neoplasms

Authors
James C Cusack, Jr, MD
Michael J Overman, MD
Section Editor
Richard M Goldberg, MD
Deputy Editor
Diane MF Savarese, MD

INTRODUCTION

A variety of tumors, both malignant and benign, arise within the small intestine. Malignant tumors include adenocarcinomas, carcinoids, sarcomas, and lymphomas, while benign lesions include adenomas, leiomyomas, lipomas, and hamartomas.

The treatment of the various types of neoplasms that arise in the small bowel will be reviewed here. The epidemiology, clinical manifestations, diagnosis, and staging of small bowel tumors are discussed separately. (See "Epidemiology, clinical features, and types of small bowel neoplasms" and "Diagnosis and staging of small bowel neoplasms".)

ADENOCARCINOMA

Locoregional disease

Surgery — Localized adenocarcinomas of the small bowel are best managed with wide segmental surgical resection. Resection of the primary and investing mesentery achieves surgical clearance of both the primary and the regional nodes at risk for metastases, and provides important staging information that impacts decisions regarding the need for adjuvant therapy (see below). However, resection of adequate mesentery may be limited by the proximity of the nodes or tumor to the superior mesenteric artery.

Pancreaticoduodenectomy is required for tumors involving the first and second portions of the duodenum. Some surgeons promote pancreaticoduodenectomy rather than wide local excision as a superior operation for all duodenal adenocarcinomas because of its more radical clearance of the tumor bed and regional lymph nodes [1,2]. However, this is not necessary for the following reasons:

Unlike pancreatic cancers, which diffusely infiltrate into the surrounding soft tissues, the extension of duodenal adenocarcinomas into adjacent tissues is usually a more localized process, and tumor-free resection margins may be obtained without resection of adjacent organs and soft tissues. Because a negative margin is critical to a curative procedure, the margin status of the resected specimen must be confirmed on frozen-section and subsequent permanent histologic sections [1,3,4].

                                     

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Nov 2016. | This topic last updated: Fri Mar 25 00:00:00 GMT+00:00 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2016 UpToDate, Inc.
References
Top
  1. Sohn TA, Lillemoe KD, Cameron JL, et al. Adenocarcinoma of the duodenum: factors influencing long-term survival. J Gastrointest Surg 1998; 2:79.
  2. Lai EC, Doty JE, Irving C, Tompkins RK. Primary adenocarcinoma of the duodenum: analysis of survival. World J Surg 1988; 12:695.
  3. Abrahams NA, Halverson A, Fazio VW, et al. Adenocarcinoma of the small bowel: a study of 37 cases with emphasis on histologic prognostic factors. Dis Colon Rectum 2002; 45:1496.
  4. Brücher BL, Stein HJ, Roder JD, et al. New aspects of prognostic factors in adenocarcinomas of the small bowel. Hepatogastroenterology 2001; 48:727.
  5. Joesting DR, Beart RW Jr, van Heerden JA, Weiland LH. Improving survival in adenocarcinoma of the duodenum. Am J Surg 1981; 141:228.
  6. Lowell JA, Rossi RL, Munson JL, Braasch JW. Primary adenocarcinoma of third and fourth portions of duodenum. Favorable prognosis after resection. Arch Surg 1992; 127:557.
  7. van Ooijen B, Kalsbeek HL. Carcinoma of the duodenum. Surg Gynecol Obstet 1988; 166:343.
  8. Kaklamanos IG, Bathe OF, Franceschi D, et al. Extent of resection in the management of duodenal adenocarcinoma. Am J Surg 2000; 179:37.
  9. Barnes G Jr, Romero L, Hess KR, Curley SA. Primary adenocarcinoma of the duodenum: management and survival in 67 patients. Ann Surg Oncol 1994; 1:73.
  10. Bakaeen FG, Murr MM, Sarr MG, et al. What prognostic factors are important in duodenal adenocarcinoma? Arch Surg 2000; 135:635.
  11. Dabaja BS, Suki D, Pro B, et al. Adenocarcinoma of the small bowel: presentation, prognostic factors, and outcome of 217 patients. Cancer 2004; 101:518.
  12. Bilimoria KY, Bentrem DJ, Wayne JD, et al. Small bowel cancer in the United States: changes in epidemiology, treatment, and survival over the last 20 years. Ann Surg 2009; 249:63.
  13. Howe JR, Karnell LH, Menck HR, Scott-Conner C. The American College of Surgeons Commission on Cancer and the American Cancer Society. Adenocarcinoma of the small bowel: review of the National Cancer Data Base, 1985-1995. Cancer 1999; 86:2693.
  14. Contant CM, Damhuis RA, van Geel AN, et al. Prognostic value of the TNM-classification for small bowel cancer. Hepatogastroenterology 1997; 44:430.
  15. Overman MJ, Hu CY, Kopetz S, et al. A population-based comparison of adenocarcinoma of the large and small intestine: insights into a rare disease. Ann Surg Oncol 2012; 19:1439.
  16. Young JI, Mongoue-Tchokote S, Wieghard N, et al. Treatment and Survival of Small-bowel Adenocarcinoma in the United States: A Comparison With Colon Cancer. Dis Colon Rectum 2016; 59:306.
  17. Ouriel K, Adams JT. Adenocarcinoma of the small intestine. Am J Surg 1984; 147:66.
  18. Adler SN, Lyon DT, Sullivan PD. Adenocarcinoma of the small bowel. Clinical features, similarity to regional enteritis, and analysis of 338 documented cases. Am J Gastroenterol 1982; 77:326.
  19. Struck A, Howard T, Chiorean EG, et al. Non-ampullary duodenal adenocarcinoma: factors important for relapse and survival. J Surg Oncol 2009; 100:144.
  20. Overman MJ, Hu CY, Wolff RA, Chang GJ. Prognostic value of lymph node evaluation in small bowel adenocarcinoma: analysis of the surveillance, epidemiology, and end results database. Cancer 2010; 116:5374.
  21. American Joint Committee on Cancer Staging Manual, 7th, Edge SB, Byrd DR, Compton CC, et al (Eds), Springer, New York 2010. p.127.
  22. Nicholl MB, Ahuja V, Conway WC, et al. Small bowel adenocarcinoma: understaged and undertreated? Ann Surg Oncol 2010; 17:2728.
  23. Wilhelm A, Müller SA, Steffen T, et al. Patients with Adenocarcinoma of the Small Intestine with 9 or More Regional Lymph Nodes Retrieved Have a Higher Rate of Positive Lymph Nodes and Improved Survival. J Gastrointest Surg 2016; 20:401.
  24. SEER Survival Monograph: Cancer Survivor Among Adults: US Seer Program, 1988-2001, Patient and Tumor Characteristics. Cancers of the esophagus, stomach, and small intestine. NIH Pub No 07-6215, SEER Program; National Cancer Institute, Bethesda, MD 2007.
  25. Wu TJ, Yeh CN, Chao TC, et al. Prognostic factors of primary small bowel adenocarcinoma: univariate and multivariate analysis. World J Surg 2006; 30:391.
  26. Zar N, Holmberg L, Wilander E, Rastad J. Survival in small intestinal adenocarcinoma. Eur J Cancer 1996; 32A:2114.
  27. Halfdanarson TR, McWilliams RR, Donohue JH, Quevedo JF. A single-institution experience with 491 cases of small bowel adenocarcinoma. Am J Surg 2010; 199:797.
  28. Kelsey CR, Nelson JW, Willett CG, et al. Duodenal adenocarcinoma: patterns of failure after resection and the role of chemoradiotherapy. Int J Radiat Oncol Biol Phys 2007; 69:1436.
  29. Rose DM, Hochwald SN, Klimstra DS, Brennan MF. Primary duodenal adenocarcinoma: a ten-year experience with 79 patients. J Am Coll Surg 1996; 183:89.
  30. Talamonti MS, Goetz LH, Rao S, Joehl RJ. Primary cancers of the small bowel: analysis of prognostic factors and results of surgical management. Arch Surg 2002; 137:564.
  31. Agrawal S, McCarron EC, Gibbs JF, et al. Surgical management and outcome in primary adenocarcinoma of the small bowel. Ann Surg Oncol 2007; 14:2263.
  32. Bauer RL, Palmer ML, Bauer AM, et al. Adenocarcinoma of the small intestine: 21-year review of diagnosis, treatment, and prognosis. Ann Surg Oncol 1994; 1:183.
  33. Overman MJ, Varadhachary GR, Kopetz S, et al. Phase II study of capecitabine and oxaliplatin for advanced adenocarcinoma of the small bowel and ampulla of Vater. J Clin Oncol 2009; 27:2598.
  34. Poultsides GA, Huang LC, Cameron JL, et al. Duodenal adenocarcinoma: clinicopathologic analysis and implications for treatment. Ann Surg Oncol 2012; 19:1928.
  35. Singhal N, Singhal D. Adjuvant chemotherapy for small intestine adenocarcinoma. Cochrane Database Syst Rev 2007; :CD005202.
  36. Moon YW, Rha SY, Shin SJ, et al. Adenocarcinoma of the small bowel at a single Korean institute: management and prognosticators. J Cancer Res Clin Oncol 2010; 136:387.
  37. Swartz MJ, Hughes MA, Frassica DA, et al. Adjuvant concurrent chemoradiation for node-positive adenocarcinoma of the duodenum. Arch Surg 2007; 142:285.
  38. Ecker BL, McMillan MT, Datta J, et al. Efficacy of adjuvant chemotherapy for small bowel adenocarcinoma: A propensity score-matched analysis. Cancer 2016; 122:693.
  39. Lepage C, Bouvier AM, Manfredi S, et al. Incidence and management of primary malignant small bowel cancers: a well-defined French population study. Am J Gastroenterol 2006; 101:2826.
  40. Coia L, Hoffman J, Scher R, et al. Preoperative chemoradiation for adenocarcinoma of the pancreas and duodenum. Int J Radiat Oncol Biol Phys 1994; 30:161.
  41. Yeung RS, Weese JL, Hoffman JP, et al. Neoadjuvant chemoradiation in pancreatic and duodenal carcinoma. A Phase II Study. Cancer 1993; 72:2124.
  42. Fishman PN, Pond GR, Moore MJ, et al. Natural history and chemotherapy effectiveness for advanced adenocarcinoma of the small bowel: a retrospective review of 113 cases. Am J Clin Oncol 2006; 29:225.
  43. Czaykowski P, Hui D. Chemotherapy in small bowel adenocarcinoma: 10-year experience of the British Columbia Cancer Agency. Clin Oncol (R Coll Radiol) 2007; 19:143.
  44. Gibson MK, Holcroft CA, Kvols LK, Haller D. Phase II study of 5-fluorouracil, doxorubicin, and mitomycin C for metastatic small bowel adenocarcinoma. Oncologist 2005; 10:132.
  45. Nakayama N, Horimatsu T, Takagi S, et al. A phase II study of 5-FU/l-LV/oxaliplatin (mFOLFOX6) in patients with metastatic or unresectable small bowel adenocarcinoma (abstract). J Clin Oncol 32: 5s, 2014 (suppl; abstr 3646). Abstract available online at http://meetinglibrary.asco.org/content/130721-144 (Accessed on June 17, 2014).
  46. Xiang XJ, Liu YW, Zhang L, et al. A phase II study of modified FOLFOX as first-line chemotherapy in advanced small bowel adenocarcinoma. Anticancer Drugs 2012; 23:561.
  47. McWilliams RR, Mahoney MR, Machello BT, et al. Pharmacogenetic dosing by UGT1A1 genotype as first-line therapy for advanced small-bowel adenocarcinoma: A North Central Cancer Treatment Group (NCCTG) trial (abstract 314). J Clin Oncol 30, 2012 (suppl 4; abstr 314). Abstract available online at http://meetinglibrary.asco.org/content/88820-115 (Accessed on March 16, 2015).
  48. McWilliams RR, Mahoney MR, Machello BT, et al. Pharmacogenetic dosing by UGT1A1 genotype as first-line therapy for advanced small-bowel adenocarcinoma: A North Central Cancer Treatment Group (NCCTG) trial. J Clin Oncol 2012; 30.
  49. Enzinger PC, Zhu A, Blaszkowsky L, et al. Phae I dose fiinding and pharmacologic study of cisplatin (P), irinotecan (C), and either capecitabine (X) or infusional 5-FU (F) in patinets with advanced gastrointestinal malignancies (abstract). Data presented at the 2005 ASCO Gastrointestinal Cancers Symposium, Miami, FL, January 27-29, 2005.
  50. Overman MJ, Kopetz S, Wen S, et al. Chemotherapy with 5-fluorouracil and a platinum compound improves outcomes in metastatic small bowel adenocarcinoma. Cancer 2008; 113:2038.
  51. Locher C, Malka D, Boige V, et al. Combination chemotherapy in advanced small bowel adenocarcinoma. Oncology 2005; 69:290.
  52. Polyzos A, Kouraklis G, Giannopoulos A, et al. Irinotecan as salvage chemotherapy for advanced small bowel adenocarcinoma: a series of three patients. J Chemother 2003; 15:503.
  53. Crawley C, Ross P, Norman A, et al. The Royal Marsden experience of a small bowel adenocarcinoma treated with protracted venous infusion 5-fluorouracil. Br J Cancer 1998; 78:508.
  54. Jigyasu D, Bedikian AY, Stroehlein JR. Chemotherapy for primary adenocarcinoma of the small bowel. Cancer 1984; 53:23.
  55. Ono M, Shirao K, Takashima A, et al. Combination chemotherapy with cisplatin and irinotecan in patients with adenocarcinoma of the small intestine. Gastric Cancer 2008; 11:201.
  56. Suenaga M, Mizunuma N, Chin K, et al. Chemotherapy for small-bowel Adenocarcinoma at a single institution. Surg Today 2009; 39:27.
  57. Zaanan A, Costes L, Gauthier M, et al. Chemotherapy of advanced small-bowel adenocarcinoma: a multicenter AGEO study. Ann Oncol 2010; 21:1786.
  58. Tsushima T, Taguri M, Honma Y, et al. Multicenter retrospective study of 132 patients with unresectable small bowel adenocarcinoma treated with chemotherapy. Oncologist 2012; 17:1163.
  59. Zaanan A, Gauthier M, Malka D, et al. Second-line chemotherapy with fluorouracil, leucovorin, and irinotecan (FOLFIRI regimen) in patients with advanced small bowel adenocarcinoma after failure of first-line platinum-based chemotherapy: a multicenter AGEO study. Cancer 2011; 117:1422.
  60. Tsang H, Yau T, Khong PL, Epstein RJ. Bevacizumab-based therapy for advanced small bowel adenocarcinoma. Gut 2008; 57:1631.
  61. Santini D, Fratto ME, Spoto C, et al. Cetuximab in small bowel adenocarcinoma: a new friend? Br J Cancer 2010; 103:1305; author reply 1306.
  62. Overman MJ, Wolff RA, Wang H. Reply: Cetuximab in small bowel adenocarcinoma: a new friend? [letter to the editor]. Br J Cancer 2010; 103:1306.
  63. Hemming AW, Sielaff TD, Gallinger S, et al. Hepatic resection of noncolorectal nonneuroendocrine metastases. Liver Transpl 2000; 6:97.
  64. Ercolani G, Grazi GL, Ravaioli M, et al. The role of liver resections for noncolorectal, nonneuroendocrine metastases: experience with 142 observed cases. Ann Surg Oncol 2005; 12:459.
  65. Adam R, Chiche L, Aloia T, et al. Hepatic resection for noncolorectal nonendocrine liver metastases: analysis of 1,452 patients and development of a prognostic model. Ann Surg 2006; 244:524.
  66. Gleisner AL, Assumpcao L, Cameron JL, et al. Is resection of periampullary or pancreatic adenocarcinoma with synchronous hepatic metastasis justified? Cancer 2007; 110:2484.
  67. Jacks SP, Hundley JC, Shen P, et al. Cytoreductive surgery and intraperitoneal hyperthermic chemotherapy for peritoneal carcinomatosis from small bowel adenocarcinoma. J Surg Oncol 2005; 91:112.
  68. Marchettini P, Sugarbaker PH. Mucinous adenocarcinoma of the small bowel with peritoneal seeding. Eur J Surg Oncol 2002; 28:19.
  69. Rorstad O. Prognostic indicators for carcinoid neuroendocrine tumors of the gastrointestinal tract. J Surg Oncol 2005; 89:151.
  70. Jann H, Roll S, Couvelard A, et al. Neuroendocrine tumors of midgut and hindgut origin: tumor-node-metastasis classification determines clinical outcome. Cancer 2011; 117:3332.
  71. AJCC (American Joint Committee on Cancer) Cancer Staging Manual, 7th ed, Edge, SB, Byrd, DR, Compton, CC, et al (Eds), Springer, New York 2010. p.181.
  72. Kim MK, Warner RR, Roayaie S, et al. Revised staging classification improves outcome prediction for small intestinal neuroendocrine tumors. J Clin Oncol 2013; 31:3776.
  73. Welch JP, Malt RA. Management of carcinoid tumors of the gastrointestinal tract. Surg Gynecol Obstet 1977; 145:223.
  74. Strodel WE, Talpos G, Eckhauser F, Thompson N. Surgical therapy for small-bowel carcinoid tumors. Arch Surg 1983; 118:391.
  75. Rothmund M, Kisker O. Surgical treatment of carcinoid tumors of the small bowel, appendix, colon and rectum. Digestion 1994; 55 Suppl 3:86.
  76. Nikou GC, Lygidakis NJ, Toubanakis C, et al. Current diagnosis and treatment of gastrointestinal carcinoids in a series of 101 patients: the significance of serum chromogranin-A, somatostatin receptor scintigraphy and somatostatin analogues. Hepatogastroenterology 2005; 52:731.
  77. Ito H, Perez A, Brooks DC, et al. Surgical treatment of small bowel cancer: a 20-year single institution experience. J Gastrointest Surg 2003; 7:925.
  78. Loftus JP, van Heerden JA. Surgical management of gastrointestinal carcinoid tumors. Adv Surg 1995; 28:317.
  79. Akerström G, Makridis C, Johansson H. Abdominal surgery in patients with midgut carcinoid tumors. Acta Oncol 1991; 30:547.
  80. Landerholm K, Zar N, Andersson RE, et al. Survival and prognostic factors in patients with small bowel carcinoid tumour. Br J Surg 2011; 98:1617.
  81. 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.
  82. Howe JR, Karnell LH, Scott-Conner C. Small bowel sarcoma: analysis of survival from the National Cancer Data Base. Ann Surg Oncol 2001; 8:496.
  83. Kadakia SC, Parker A, Canales L. Metastatic tumors to the upper gastrointestinal tract: endoscopic experience. Am J Gastroenterol 1992; 87:1418.
  84. Richie RE, Reynolds VH, Sawyers JL. Tumor metastases to the small bowel from extra-abdominal sites. South Med J 1973; 66:1383.
  85. FARMER RG, HAWK WA. METASTATIC TUMORS OF THE SMALL BOWEL. Gastroenterology 1964; 47:496.
  86. Zollinger RM Jr. Primary neoplasms of the small intestine. Am J Surg 1986; 151:654.
  87. Desa LA, Bridger J, Grace PA, et al. Primary jejunoileal tumors: a review of 45 cases. World J Surg 1991; 15:81.
  88. Ashley SW, Wells SA Jr. Tumors of the small intestine. Semin Oncol 1988; 15:116.
  89. Bjork KJ, Davis CJ, Nagorney DM, Mucha P Jr. Duodenal villous tumors. Arch Surg 1990; 125:961.
  90. Saurin JC, Gutknecht C, Napoleon B, et al. Surveillance of duodenal adenomas in familial adenomatous polyposis reveals high cumulative risk of advanced disease. J Clin Oncol 2004; 22:493.
  91. Morgan BK, Compton C, Talbert M, et al. Benign smooth muscle tumors of the gastrointestinal tract. A 24-year experience. Ann Surg 1990; 211:63.
  92. Sarr MG, Murr MM. Small Intestinal Neoplasms. In: Surgery: Scientific Principles and Practice, Greenfield LJ (Ed), Lippincott-Raven, 1997. p.844.