eMedicine Specialties > Oncology > Carcinomas of the Gastrointestinal Tract

Ampullary Carcinoma: Differential Diagnoses & Workup

Author: Vivek K Mehta, MD, Radiation Oncologist, Director, Center for Advanced Targeted Radiotherapies, Department of Radiation Oncology, Swedish Cancer Institute, Seattle, Washington
Coauthor(s): George Fisher, MD, PhD, Associate Professor, Department of Internal Medicine, Division of Medical Oncology, Stanford University School of Medicine
Contributor Information and Disclosures

Updated: Jul 18, 2006

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
  • Table 1. Staging of Ampullary Cancers by the TNM System

    Open table in new window

    Table
    StageTNM
    Stage 0TisN0M0
    Stage IT1N0M0
    Stage IIT2-3N0M0
    Stage IIIT1-3N1M0
    Stage IVT4N0-1M0
    T1-4N0-1M1
    StageTNM
    Stage 0TisN0M0
    Stage IT1N0M0
    Stage IIT2-3N0M0
    Stage IIIT1-3N1M0
    Stage IVT4N0-1M0
    T1-4N0-1M1

More on Ampullary Carcinoma

Overview: Ampullary Carcinoma
Differential Diagnoses & Workup: Ampullary Carcinoma
Treatment & Medication: Ampullary Carcinoma
Follow-up: Ampullary Carcinoma
References

References

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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

Contributor Information and Disclosures

Author

Vivek K Mehta, MD, Radiation Oncologist, Director, Center for Advanced Targeted Radiotherapies, Department of Radiation Oncology, Swedish Cancer Institute, Seattle, Washington
Vivek K Mehta, MD is a member of the following medical societies: American Society for Therapeutic Radiology and Oncology, Phi Beta Kappa, and Sigma Xi
Disclosure: Nothing to disclose.

Coauthor(s)

George Fisher, MD, PhD, Associate Professor, Department of Internal Medicine, Division of Medical Oncology, Stanford University School of Medicine
George Fisher, MD, PhD is a member of the following medical societies: American Cancer Society, American Medical Association, and American Society of Clinical Oncology
Disclosure: Nothing to disclose.

Medical Editor

Clarence Sarkodee-Adoo, MD, Consulting Staff, Department of Bone Marrow Transplantation, City of Hope Samaritan BMT Program
Clarence Sarkodee-Adoo, MD is a member of the following medical societies: American Society of Clinical Oncology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Benjamin Movsas, MD, Vice-Chairman, Department of Radiation Oncology, Fox Chase Cancer Center
Benjamin Movsas, MD is a member of the following medical societies: American College of Radiology, American Radium Society, American Society for Therapeutic Radiology and Oncology, and American Society of Clinical Oncology
Disclosure: Nothing to disclose.

CME Editor

Rajalaxmi McKenna, MD, FACP, Consulting Staff, Department of Medicine, Southwest Medical Consultants, SC, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

John S Macdonald, MD, Professor of Medicine, New York Medical College; Chief, Division of Medical Oncology, St Vincent's Hospital and Medical Center; Medical Director, Saint Vincent's Comprehensive Cancer Center
Disclosure: Nothing to disclose.

 
 
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