Diagnosis and staging of small bowel neoplasms
- James C Cusack, Jr, MD
James C Cusack, Jr, MD
- Associate Professor of Surgery
- Harvard Medical School
- Michael J Overman, MD
Michael J Overman, MD
- Associate Professor
- Department of Gastrointestinal Medical Oncology
- MD Anderson Cancer Center
A variety of tumors, both malignant and benign, may arise within the small intestine. Malignant tumors include adenocarcinomas, neuroendocrine tumors (carcinoids), stromal tumors, and lymphomas. Benign lesions that may arise in the small bowel include adenomas, leiomyomas, fibromas, and lipomas.
The diagnosis and staging of small bowel tumors will be reviewed here. The epidemiology, clinical manifestations, and treatment of the specific types of tumors are discussed separately. (See "Epidemiology, clinical features, and types of small bowel neoplasms" and "Treatment of small bowel neoplasms".)
The clinical features of small bowel neoplasms may include one or more of the following: pain, obstruction, bleeding, anorexia, weight loss, perforation, or in the case of a duodenal primary, jaundice. Malignant tumors are more likely to be symptomatic as compared with benign lesions. (See "Epidemiology, clinical features, and types of small bowel neoplasms", section on 'Clinical presentation'.)
The variable nature of the presenting symptoms, combined with the lack of physical findings, can contribute to a delay in diagnosis in many cases . In one study, failure to obtain a proper diagnostic test or misinterpretation of test results accounted for delays of 8 and 12 months, respectively; by comparison, the estimated diagnostic delay due to the patient's failure to report symptoms was less than two months . As prognosis is closely linked to disease extent for all of the major malignant small bowel tumors, early detection and treatment can contribute to a favorable outcome [2-4]. Because of the vagueness and nonspecificity of the presenting symptoms, a high index of suspicion is essential for early diagnosis and treatment [5,6].
Patients whose symptoms are such that a small bowel tumor is part of the differential diagnosis should undergo a complete history, physical examination, and screening for fecal occult blood. A minimum laboratory work-up should include a complete blood count, measurement of serum electrolytes, and liver function tests.
- Ciresi DL, Scholten DJ. The continuing clinical dilemma of primary tumors of the small intestine. Am Surg 1995; 61:698.
- Maglinte DD, O'Connor K, Bessette J, et al. The role of the physician in the late diagnosis of primary malignant tumors of the small intestine. Am J Gastroenterol 1991; 86:304.
- Cunningham JD, Aleali R, Aleali M, et al. Malignant small bowel neoplasms: histopathologic determinants of recurrence and survival. Ann Surg 1997; 225:300.
- North JH, Pack MS. Malignant tumors of the small intestine: a review of 144 cases. Am Surg 2000; 66:46.
- Van Weyenberg SJ, Meijerink MR, Jacobs MA, et al. MR enteroclysis in the diagnosis of small-bowel neoplasms. Radiology 2010; 254:765.
- Boudiaf M, Jaff A, Soyer P, et al. Small-bowel diseases: prospective evaluation of multi-detector row helical CT enteroclysis in 107 consecutive patients. Radiology 2004; 233:338.
- Bessette JR, Maglinte DD, Kelvin FM, Chernish SM. Primary malignant tumors in the small bowel: a comparison of the small-bowel enema and conventional follow-through examination. AJR Am J Roentgenol 1989; 153:741.
- Bruneton JN, Drouillard J, Bourry J, et al. [Adenocarcinoma of the small intestine. Current state of diagnosis and treatment. A study of 27 cases and a review of the literature]. J Radiol 1983; 64:117.
- Vuori JV. Primary malignant tumours of the small intestine. Analysis of cases diagnosed in Finland, 1953-1962. Acta Chir Scand 1971; 137:555.
- Minardi AJ Jr, Zibari GB, Aultman DF, et al. Small-bowel tumors. J Am Coll Surg 1998; 186:664.
- Laurent F, Raynaud M, Biset JM, et al. Diagnosis and categorization of small bowel neoplasms: role of computed tomography. Gastrointest Radiol 1991; 16:115.
- Horton KM, Fishman EK. Multidetector-row computed tomography and 3-dimensional computed tomography imaging of small bowel neoplasms: current concept in diagnosis. J Comput Assist Tomogr 2004; 28:106.
- Levy AD, Remotti HE, Thompson WM, et al. Gastrointestinal stromal tumors: radiologic features with pathologic correlation. Radiographics 2003; 23:283.
- HUDSON HL, MARGULIS AR. THE ROENTGEN FINDINGS OF CARCINOID TUMORS OF THE GASTROINTESTINAL TRACT: A REPORT OF 12 RECENT CASES. Am J Roentgenol Radium Ther Nucl Med 1964; 91:833.
- Dudiak KM, Johnson CD, Stephens DH. Primary tumors of the small intestine: CT evaluation. AJR Am J Roentgenol 1989; 152:995.
- Cockey BM, Fishman EK, Jones B, Siegelman SS. Computed tomography of abdominal carcinoid tumor. J Comput Assist Tomogr 1985; 9:38.
- Chambers AJ, Pasieka JL, Dixon E, Rorstad O. Role of imaging in the preoperative staging of small bowel neuroendocrine tumors. J Am Coll Surg 2010; 211:620.
- Seemann MD, Meisetschlaeger G, Gaa J, Rummeny EJ. Assessment of the extent of metastases of gastrointestinal carcinoid tumors using whole-body PET, CT, MRI, PET/CT and PET/MRI. Eur J Med Res 2006; 11:58.
- Bender GN, Maglinte DD, Klöppel VR, Timmons JH. CT enteroclysis: a superfluous diagnostic procedure or valuable when investigating small-bowel disease? AJR Am J Roentgenol 1999; 172:373.
- Orjollet-Lecoanet C, Ménard Y, Martins A, et al. [CT enteroclysis for detection of small bowel tumors]. J Radiol 2000; 81:618.
- Bender GN, Timmons JH, Williard WC, Carter J. Computed tomographic enteroclysis: one methodology. Invest Radiol 1996; 31:43.
- Maglinte DD, Bender GN, Heitkamp DE, et al. Multidetector-row helical CT enteroclysis. Radiol Clin North Am 2003; 41:249.
- Pilleul F, Penigaud M, Milot L, et al. Possible small-bowel neoplasms: contrast-enhanced and water-enhanced multidetector CT enteroclysis. Radiology 2006; 241:796.
- Schmidt S, Lepori D, Meuwly JY, et al. Prospective comparison of MR enteroclysis with multidetector spiral-CT enteroclysis: interobserver agreement and sensitivity by means of "sign-by-sign" correlation. Eur Radiol 2003; 13:1303.
- Paulsen SR, Huprich JE, Fletcher JG, et al. CT enterography as a diagnostic tool in evaluating small bowel disorders: review of clinical experience with over 700 cases. Radiographics 2006; 26:641.
- Masselli G, Polettini E, Casciani E, et al. Small-bowel neoplasms: prospective evaluation of MR enteroclysis. Radiology 2009; 251:743.
- Pappalardo G, Gualdi G, Nunziale A, et al. Impact of magnetic resonance in the preoperative staging and the surgical planning for treating small bowel neoplasms. Surg Today 2013; 43:613.
- Cronin CG, Scott J, Kambadakone A, et al. Utility of positron emission tomography/CT in the evaluation of small bowel pathology. Br J Radiol 2012; 85:1211.
- Watanabe N, Hayashi S, Kato H, et al. FDG-PET imaging in duodenal cancer. Ann Nucl Med 2004; 18:351.
- Sperti C, Pasquali C, Fiore V, et al. Clinical usefulness of 18-fluorodeoxyglucose positron emission tomography in the management of patients with nonpancreatic periampullary neoplasms. Am J Surg 2006; 191:743.
- Juweid ME, Stroobants S, Hoekstra OS, et al. Use of positron emission tomography for response assessment of lymphoma: consensus of the Imaging Subcommittee of International Harmonization Project in Lymphoma. J Clin Oncol 2007; 25:571.
- Pennazio M, Spada C, Eliakim R, et al. Small-bowel capsule endoscopy and device-assisted enteroscopy for diagnosis and treatment of small-bowel disorders: European Society of Gastrointestinal Endoscopy (ESGE) Clinical Guideline. Endoscopy 2015; 47:352.
- Cobrin GM, Pittman RH, Lewis BS. Increased diagnostic yield of small bowel tumors with capsule endoscopy. Cancer 2006; 107:22.
- Lewis BS, Eisen GM, Friedman S. A pooled analysis to evaluate results of capsule endoscopy trials. Endoscopy 2005; 37:960.
- Fry LC, Bellutti M, Neumann H, et al. Incidence of bleeding lesions within reach of conventional upper and lower endoscopes in patients undergoing double-balloon enteroscopy for obscure gastrointestinal bleeding. Aliment Pharmacol Ther 2009; 29:342.
- 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.
- Zhu L, Kim K, Domenico DR, et al. Adenocarcinoma of duodenum and ampulla of Vater: clinicopathology study and expression of p53, c-neu, TGF-alpha, CEA, and EMA. J Surg Oncol 1996; 61:100.
- Overman MJ, Morris J, Adinin R, et al. A phase II study of capecitabine and oxaliplatin (CAPOX) in metastatic adenocarcinoma of the small bowel or ampulla of Vater (abstract). Data presented at the ASCO Annual Gastrointestinal Cancers Symposium, Orlando, Florida, January 20, 2007.
- Feldman JM. Urinary serotonin in the diagnosis of carcinoid tumors. Clin Chem 1986; 32:840.
- Kema IP, de Vries EG, Slooff MJ, et al. Serotonin, catecholamines, histamine, and their metabolites in urine, platelets, and tumor tissue of patients with carcinoid tumors. Clin Chem 1994; 40:86.
- Modlin IM, Gustafsson BI, Moss SF, et al. Chromogranin A--biological function and clinical utility in neuro endocrine tumor disease. Ann Surg Oncol 2010; 17:2427.
- Campana D, Nori F, Piscitelli L, et al. Chromogranin A: is it a useful marker of neuroendocrine tumors? J Clin Oncol 2007; 25:1967.
- Raderer M, Traub T, Formanek M, et al. Somatostatin-receptor scintigraphy for staging and follow-up of patients with extraintestinal marginal zone B-cell lymphoma of the mucosa associated lymphoid tissue (MALT)-type. Br J Cancer 2001; 85:1462.
- Kvols LK, Brown ML, O'Connor MK, et al. Evaluation of a radiolabeled somatostatin analog (I-123 octreotide) in the detection and localization of carcinoid and islet cell tumors. Radiology 1993; 187:129.
- Lamberts SW, Bakker WH, Reubi JC, Krenning EP. Somatostatin-receptor imaging in the localization of endocrine tumors. N Engl J Med 1990; 323:1246.
- Hanson MW, Feldman JM, Blinder RA, et al. Carcinoid tumors: iodine-131 MIBG scintigraphy. Radiology 1989; 172:699.
- Bomanji J, Mather S, Moyes J, et al. A scintigraphic comparison of iodine-123-metaiodobenzylguanidine and an iodine-labeled somatostatin analog (Tyr-3-octreotide) in metastatic carcinoid tumors. J Nucl Med 1992; 33:1121.
- Strosberg J, Nasir A, Coppola D, et al. Correlation between grade and prognosis in metastatic gastroenteropancreatic neuroendocrine tumors. Hum Pathol 2009; 40:1262.
- http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm504524.htm (Accessed on June 07, 2016).
- Janson ET, Westlin JE, Eriksson B, et al. [111In-DTPA-D-Phe1]octreotide scintigraphy in patients with carcinoid tumours: the predictive value for somatostatin analogue treatment. Eur J Endocrinol 1994; 131:577.
- Kwekkeboom DJ, de Herder WW, Kam BL, et al. Treatment with the radiolabeled somatostatin analog [177 Lu-DOTA 0,Tyr3]octreotate: toxicity, efficacy, and survival. J Clin Oncol 2008; 26:2124.
- 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.
- 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.
- American Joint Committee on Cancer Staging Manual, 7th, Edge SB, Byrd DR, Compton CC, et al (Eds), Springer, New York 2010. p.285.
- American Joint Committee on Cancer Staging Manual, 7th, Edge SB, Byrd DR, Compton CC, et al (Eds), Springer, New York 2010. p.175.
- Lee MJ, Lee HS, Kim WH, et al. Expression of mucins and cytokeratins in primary carcinomas of the digestive system. Mod Pathol 2003; 16:403.
- Chen ZM, Wang HL. Alteration of cytokeratin 7 and cytokeratin 20 expression profile is uniquely associated with tumorigenesis of primary adenocarcinoma of the small intestine. Am J Surg Pathol 2004; 28:1352.
- DIAGNOSTIC EVALUATION
- Radiographic imaging
- - UGI/SBFT
- - CT scan
- - Angiography and radionuclide scanning
- - Enteroclysis and enterography
- CT enterography
- MR enterography
- - PET and integrated PET/CT
- Neuroendocrine tumors
- Endoscopic techniques
- - Upper endoscopy
- - Wireless video capsule endoscopy
- - Enteroscopy
- - Other methods
- Tumor markers
- Diagnostic testing for neuroendocrine tumors
- - Urinary excretion of 5-HIAA
- - Other biochemical tests
- - Tumor localization by somatostatin receptor-based imaging techniques
- Surgical exploration
- Sarcoma and neuroendocrine tumors
- HISTOLOGY AND DIFFERENTIAL DIAGNOSIS