eMedicine Specialties > Oncology > Carcinomas of the Gastrointestinal Tract
Ampullary Carcinoma: Differential Diagnoses & Workup
Updated: Jul 18, 2006
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Differential Diagnoses
| Bile Duct Strictures | Lymphoma, Non-Hodgkin |
| Bile Duct Tumors | Pancreatic Cancer |
| Carcinoma of the Ampulla of Vater | |
| Cholangiocarcinoma | |
| Gallbladder Cancer |
Other Problems to Be Considered
Biliary cirrhosis
Workup
Laboratory Studies
- Routine laboratory studies include a complete blood cell count, electrolyte panel, liver function studies (prothrombin time, bilirubin [direct and indirect], transaminases, alkaline phosphatase), CEA, and CA 19-9.
- CA 19-9 is a recently discovered tumor marker that is detectable in serum. It often is elevated in pancreatic malignancies and might have a role in assessing response to therapy, predicting tumor recurrence, or both.
- CEA is another nonspecific tumor marker that sometimes is elevated in pancreatic malignancies. It might have a role in assessing response to treatment or predicting tumor recurrence. Because CEA also is elevated in patients with other gastrointestinal malignancies (eg, colon and rectal in particular), exclude the possibility of a second primary tumor in these patients.
Imaging Studies
- Ultrasound of the abdomen
- Obtain an ultrasound image of the abdomen to evaluate the common bile duct and the pancreatic ducts.
- Dilatation of these ducts essentially is diagnostic for extrahepatic obstruction.
- CT scan of the abdomen and/or pelvis: Obtain a CT scan image to evaluate the local region of interest and evaluate for possible metastases.
- Endoscopic retrograde cholangiopancreatography
- Obtain ERCP findings to evaluate the ductal architecture further.
- Narrowing or irregularities might suggest malignancy.
- Chest radiograph: Obtain a chest x-ray film to complete the workup (ie, for staging purposes).
- Positron emission tomography (PET) or PET-CT scans: These scans have been widely adopted in the author's clinic as a means of imaging the metabolic activity of a particular tumor. When metastases are smaller than they can be reliably detected on a CT scan, PET or PET-CT scans can detect them.
Staging
Over the years, multiple systems for staging this tumor have been proposed.
- Martin proposed a 4-stage system, as follows:
- Stage I - Vegetating tumor limited to the epithelium with no involvement of the sphincter of Oddi
- Stage II - Tumor localized in the duodenal submucosa without involvement of the duodenal muscularis propria but possible involvement of the sphincter of Oddi
- Stage III - Tumor of the duodenal muscularis propria
- Stage IV - Tumor of the periduodenal area or pancreas, with proximal or distal lymph node involvement
- The classification system of Yamaguchi and Enjoji is similar to the Martin classification.
- Talbot et al devised a system that scored tumors according to the degree of infiltration (from 1-4 according to increasing infiltration) and according to tumor differentiation (from 1-3 for well, moderately, and poorly differentiated tumors), the sum of which separated the patients into 2 groups (scores 2-4 and scores 5-7).
- The currently accepted American Joint Committee on Cancer staging system for ampullary carcinoma emphasizes the importance of pancreatic invasion and lymph node metastases (see below and see Table 1, below). Size has little impact on tumor stage. The definition of primary tumor (T), regional lymph node (N), and remote metastases (M) for classification and staging of thyroid node metastasis and staging for cancer of the ampulla of Vater is as follows:
- Primary tumor
- TX – Primary tumor cannot be assessed
- T0 – No evidence of primary tumor
- Tis – Carcinoma in situ
- T1 – Tumor limited to ampulla of Vater
- T2 – Tumor invades duodenal wall
- T3 – Tumor invades less than 2 cm into pancreas
- T4 – Tumor invades more than 2 cm into pancreas or other organs
- Regional lymph nodes
- NX – Regional lymph nodes cannot be assessed
- N0 – No regional lymph node metastases
- N1 – Lymph node metastases
- Distant metastases
- MX – Presence of distant metastases cannot be assessed
- M0 – No distant metastases
- M1 – Distant metastases
- Primary tumor
- Table 1. Staging of Ampullary Cancers by the TNM System
Open table in new window
[ CLOSE WINDOW ]Table
Stage T N M Stage 0 Tis N0 M0 Stage I T1 N0 M0 Stage II T2-3 N0 M0 Stage III T1-3 N1 M0 Stage IV T4 N0-1 M0 … T1-4 N0-1 M1 Stage T N M Stage 0 Tis N0 M0 Stage I T1 N0 M0 Stage II T2-3 N0 M0 Stage III T1-3 N1 M0 Stage IV T4 N0-1 M0 … T1-4 N0-1 M1
More on Ampullary Carcinoma |
| Overview: Ampullary Carcinoma |
Differential Diagnoses & Workup: Ampullary Carcinoma |
| Treatment & Medication: Ampullary Carcinoma |
| Follow-up: Ampullary Carcinoma |
| References |
| « Previous Page | Next Page » |
References
Akwari OE, van Heerden JA, Adson MA, Baggenstoss AH. Radical pancreatoduodenectomy for cancer of the papilla of Vater. Arch Surg. Apr 1977;112(4):451-6. [Medline].
Allema JH, Reinders ME, van Gulik TM, et al. Results of pancreaticoduodenectomy for ampullary carcinoma and analysis of prognostic factors for survival. Surgery. Mar 1995;117(3):247-53. [Medline].
Barton RM, Copeland EM 3d. Carcinoma of the ampulla of Vater. Surg Gynecol Obstet. Mar 1983;156(3):297-301. [Medline].
Brown KM, Tompkins AJ, Yong S, et al. Pancreaticoduodenectomy is curative in the majority of patients with node-negative ampullary cancer. Arch Surg. Jun 2005;140(6):529-32; discussion 532-3.
Chan C, Herrera MF, de la Garza L, et al. Clinical behavior and prognostic factors of periampullary adenocarcinoma. Ann Surg. Nov 1995;222(5):632-7. [Medline].
Dawson PJ, Connolly MM. Influence of site of origin and mucin production on survival in ampullary carcinoma. Ann Surg. Aug 1989;210(2):173-9. [Medline].
Di Giorgio A, Alfieri S, Rotondi F, et al. Pancreatoduodenectomy for tumors of Vater''s ampulla: report on 94 consecutive patients. World J Surg. Apr 2005;29(4):513-8.
Dorandeu A, Raoul JL, Siriser F, et al. Carcinoma of the ampulla of Vater: prognostic factors after curative surgery: a series of 45 cases. Gut. Mar 1997;40(3):350-5. [Medline].
Duffy JP, Hines OJ, Liu JH, et al. Improved survival for adenocarcinoma of the ampulla of Vater: fifty-five consecutive resections. Arch Surg. Sep 2003;138(9):941-8; discussion 948-50. [Medline].
Gastrointestinal Tumor Study Group. A multi-institutional comparative trial of radiation therapy alone and in combination with 5-fluorouracil for locally unresectable pancreatic carcinoma. The Gastrointestinal Tumor Study Group. Ann Surg. Feb 1979;189(2):205-8. [Medline].
Gastrointestinal Tumor Study Group. Treatment of locally unresectable carcinoma of the pancreas: comparison of combined-modality therapy (chemotherapy plus radiotherapy) to chemotherapy alone. Gastrointestinal Tumor Study Group. J Natl Cancer Inst. Jul 20 1988;80(10):751-5. [Medline].
Howe JR, Klimstra DS, Moccia RD, et al. Factors predictive of survival in ampullary carcinoma. Ann Surg. Jul 1998;228(1):87-94. [Medline].
Kamisawa T, Fukayama M, Koike M, et al. Carcinoma of the ampulla of Vater: expression of cancer-associated antigens inversely correlated with prognosis. Am J Gastroenterol. Oct 1988;83(10):1118-23. [Medline].
Kayahara M, Nagakawa T, Ohta T, et al. Surgical strategy for carcinoma of the papilla of Vater on the basis of lymphatic spread and mode of recurrence. Surgery. Jun 1997;121(6):611-7. [Medline].
Klempnauer J, Ridder GJ, Pichlmayr R. Prognostic factors after resection of ampullary carcinoma: multivariate survival analysis in comparison with ductal cancer of the pancreatic head. Br J Surg. Dec 1995;82(12):1686-91. [Medline].
Kopelson G, Galdabini J, Warshaw AL, Gunderson LL. Patterns of failure after curative surgery for extra-hepatic biliary tract carcinoma: implications for adjuvant therapy. Int J Radiat Oncol Biol Phys. Mar 1981;7(3):413-7. [Medline].
Moertel CG, Frytak S, Hahn RG, et al. Therapy of locally unresectable pancreatic carcinoma: a randomized comparison of high dose (6000 rads) radiation alone, moderate dose radiation (4000 rads + 5-fluorouracil), and high dose radiation + 5-fluorouracil: The Gastrointestinal Tumor Study Group. Cancer. Oct 15 1981;48(8):1705-10. [Medline].
Neoptolemos JP, Talbot IC, Carr-Locke DL, et al. Treatment and outcome in 52 consecutive cases of ampullary carcinoma. Br J Surg. Oct 1987;74(10):957-61. [Medline].
Padilla D, Cubo T, Pardo R, et al. Late development of cholangiocarcinoma after hepaticojejunostomy due to ampullary carcinoma. Gut. Mar 2004;53(3):472-3. [Medline].
Saurin JC, Chavaillon A, Napoleon B, et al. Long-term follow-up of patients with endoscopic treatment of sporadic adenomas of the papilla of vater. Endoscopy. May 2003;35(5):402-6. [Medline].
Shirai Y, Ohtani T, Tsukada K, Hatakeyama K. Patterns of lymphatic spread of carcinoma of the ampulla of Vater. Br J Surg. Jul 1997;84(7):1012-6. [Medline].
Shutze WP, Sack J, Aldrete JS. Long-term follow-up of 24 patients undergoing radical resection for ampullary carcinoma, 1953 to 1988. Cancer. Oct 15 1990;66(8):1717-20. [Medline].
Sikora SS, Balachandran P, Dimri K, et al. Adjuvant chemo-radiotherapy in ampullary cancers. Eur J Surg Oncol. Mar 2005;31(2):158-63.
Sperti C, Pasquali C, Piccoli A, et al. Radical resection for ampullary carcinoma: long-term results. Br J Surg. May 1994;81(5):668-71. [Medline].
Talamini MA, Moesinger RC, Pitt HA, et al. Adenocarcinoma of the ampulla of Vater. A 28-year experience. Ann Surg. May 1997;225(5):590-9; discussion 599-600. [Medline].
Talbot IC, Neoptolemos JP, Shaw DE, Carr-Locke D. The histopathology and staging of carcinoma of the ampulla of Vater. Histopathology. Feb 1988;12(2):155-65. [Medline].
Tarazi RY, Hermann RE, Vogt DP, et al. Results of surgical treatment of periampullary tumors: a thirty-five-year experience. Surgery. Oct 1986;100(4):716-23. [Medline].
Willett CG, Warshaw AL, Convery K, et al. Patterns of failure after pancreaticoduodenectomy for ampullary carcinoma. Surg Gynecol Obstet. Jan 1993;176(1):33-8. [Medline].
Yamaguchi K, Enjoji M. Carcinoma of the ampulla of vater. A clinicopathologic study and pathologic staging of 109 cases of carcinoma and 5 cases of adenoma. Cancer. Feb 1 1987;59(3):506-15. [Medline].
Yamaguchi K, Nishihara K. Long- and short-term survivors after pancreatoduodenectomy for ampullary carcinoma. J Surg Oncol. Jul 1992;50(3):195-200. [Medline].
Yeung RS, Weese JL, Hoffman JP. Neoadjuvant chemoradiation in pancreatic and duodenal carcinoma. A Phase II Study. Cancer. Oct 1 1993;72(7):2124-33. [Medline].
el-Ghazzawy AG, Wade TP, Virgo KS, Johnson FE. Recent experience with cancer of the ampulla of Vater in a national hospital group. Am Surg. Jul 1995;61(7):607-11. [Medline].
Further Reading
Keywords
cancer, carcinoma, bile duct cancer, common bile duct, duodenal mucosa, pancreatic duct, adenocarcinoma, gastrointestinal malignancy, gastrointestinal cancer, GI cancer, GI malignancy, ampulla of Vater, ampullary carcinoma, periampullary carcinoma, cancer of the ampulla of Vater, pancreaticoduodenal resection, Whipple procedure
Differential Diagnoses & Workup: Ampullary Carcinoma