eMedicine Specialties > Oncology > Carcinomas of Endocrine Organs

Neoplasms of the Endocrine Pancreas: Follow-up

Author: Eric J Hanly, MD, Resident, Department of Surgery, The Johns Hopkins University School of Medicine; Fellow, Department of Surgery, The Johns Hopkins University School of Medicine
Coauthor(s): Ozanan R Meireles, MD, Research Fellow in Minimally Invasive Surgery, Department of Surgery, Johns Hopkins University School of Medicine; Michael R Marohn, DO, Associate Professor, Department of Surgery and Surgical Sciences, John Hopkins University School of Medicine; Charles J Yeo, MD, Samuel D Gross Professor and Chair of Surgery at Jefferson Medical College of Thomas Jefferson University and at Thomas Jefferson University Hospital; Keith D Lillemoe, MD, Professor and Chairman, Department of Surgery, Indiana University School of Medicine; Lisa H McGrail, MD, Associate Chief, Department of Hematology/Oncology, Malcolm Grow Medical Center
Contributor Information and Disclosures

Updated: Jun 29, 2006

Follow-up

Further Inpatient Care

  • Patients who have complications from chemotherapy (eg, myelosuppression, renal failure, hepatic failure, severe gastrointestinal toxicity) may require inpatient treatment.
  • Patients with tumor recurrences that lead to the recurrence of symptomatic hormone-excess states may again require inpatient care to correct fluid, electrolyte, and nutritional disturbances.

Further Outpatient Care

  • Patients who have undergone surgical resection of neoplasms of the endocrine pancreas require close follow-up with an endocrinologist or medical oncologist, and surveillance serum hormone levels should be tested regularly to ensure that recurrence is diagnosed early and treated appropriately.
  • Proper outpatient treatment of patients with unresectable pancreatic endocrine tumors requires regular follow-up with endocrinologists and medical oncologists.

Inpatient & Outpatient Medications

  • Although octreotide is administered parenterally, usually via a subcutaneous injection, patients who have received the proper instruction can self-administer the medication.
  • Interferon and cytotoxic chemotherapeutic agents are generally administered in a well-controlled outpatient or inpatient setting.
  • Patients can also consider enrollment in some clinical trials conducted by the National Cancer Institute, if they meet criteria.
    • The NCT00003514 Phase II Study of Antineoplastons A10 and AS2-1 in Patients with Metastatic or Incurable Neuroendocrine Tumors (Verified by National Cancer Institute [NCI] October 2003)
    • The NCT00075439 Phase II Study of Gefitinib in Patients with Progressive Metastatic Neuroendocrine Tumors (Verified by National Cancer Institute [NCI] December 2004)

Transfer

  • Neoplasms of the endocrine pancreas are rare, their diagnosis and localization often require advanced technologies, and their successful treatment requires a careful multidisciplinary approach.
  • For these reasons, the patient with a possible or confirmed pancreatic endocrine tumor is best treated at a large referral center where endocrinologists, gastroenterologists, pancreatic surgeons, and medical oncologists are available.

Complications

  • Late in the course of pancreatic endocrine tumors, patients can have morbidity related to the mass effects of the disease.
    • Patients with tumors in the pancreatic head occasionally have biliary obstruction, pancreatic obstruction, or both.
    • Chronic abdominal pain can occur because of the compressive effects of a large intra-abdominal mass or obstructive pancreatitis.
  • Morbidity resulting from the effects of excess hormonal production by functional neoplasms of the endocrine pancreas occurs sooner than the mass effects in the course of the disease.
    • If untreated, patients with insulinoma can have hypoglycemic seizures and even frank coma (Welbourne, 1981).
    • Prior to the development of effective antisecretory medications (eg, H2 blockers, proton pump inhibitors), patients with gastrinoma often had life-threatening gastrointestinal bleeding from peptic ulcers.
    • Untreated patients with VIPoma can become severely dehydrated from diarrhea, and fatal cardiac arrhythmias can develop secondary to associated hypokalemia.
    • The morbidity associated with the hormonal effects of glucagonoma can be striking; patients can have diabetes, wasting, stomatitis, and other features similar to severe nutritional deficiency.
  • A study of 125 patients who underwent surgical resection of pancreatic neuroendocrine tumors between 1949 and 1996 had complications that included pancreatic fistula (16%), wound infection (15%), delayed gastric emptying (8%), and death (2.8%) (Phan, 1998):
  • Permanent diabetes mellitus can mandate extended pancreatic tumor resection.
  • Complications of medical therapies are discussed in Medication.

Prognosis

  • Endocrine tumors of the pancreas typically have indolent growth; nevertheless, the rate of tumor growth is still a major determinant of survival in patients with these lesions (Metz, 1995).
  • Because these tumors usually grow slowly and have a relatively low metastatic potential and because no specific criteria have been defined to predict their behavior, the distinction between benign and malignant neoplasms is based on the presence of metastatic disease; therefore, long-term clinical follow-up is often required (Norton, 1993; Metz, 1994).
  • Neoplasms of the endocrine pancreas have patterns of initial tumor spread that are similar to those of other gastrointestinal carcinomas.
    • The neoplasms spread first to regional lymph nodes and then to the liver.
    • Bone metastases may occur late in the course of the disease and indicate a poor prognosis (Kloppel, 1988).
    • In rare cases, pancreatic endocrine tumors metastasize to the lungs or brain.
  • Currently, the most important prognostic factor that determines the survival of patients with neoplasms of the endocrine pancreas is the presence or absence of metastatic disease.
    • Because even metastatic pancreatic endocrine neoplasms typically grow slowly, the prognosis associated with these tumors is relatively good compared with nonendocrine pancreatic cancers.
    • More than 90% of patients with insulinomas have benign neoplasms without evidence of metastases, and as many as 97% of these patients can be cured with surgical resection (Rothmund, 1990).
    • Patients with gastrinomas have a poorer prognosis; 60% of these tumors are malignant (McGuigan, 1998). However, survival rates are strikingly different between patients in whom gastrinoma metastasis is limited to the lymph nodes and those with hepatic metastases.
      • Patients in whom gastrinoma metastasis involves only the lymph nodes can live a quarter decade or longer, and their life expectancy is indistinguishable from that of patients with gastrinoma in whom no tumor is identified at surgery (Stabile, 1985; Fraker, 1994; Weber, 1995).
      • Patients with hepatic gastrinoma metastases have a 5-year survival rate of 20-30%, compared with approximately 90% in patients without liver metastasis (Jensen, 1994).
    • Among pancreatic endocrine tumors, glucagonomas tend to be relatively large (5-10 cm) at diagnosis. Also, as many as 80% are invasive or metastatic; this frequency rate is higher than that of the other tumors (Leichter, 1980; Stacpoole, 1981; Holst, 1985).
    • Because glucagonoma syndrome is recognized relatively late in most patients, surgical cure is likely in fewer than 20% of all patients (Jensen, 1998).
    • Approximately half of all VIPomas are metastatic at the time of diagnosis or surgery (Jensen, 1998), and approximately one third of patients are cured with surgery (Verner, 1974; Long, 1981).
    • Most somatostatinomas (84%) are metastatic at the time of presentation, but a number of patients survive 5 years after combination surgery and chemotherapy (Boden, 1985; Vinik, 1987).
    • The malignancy rate of nonfunctioning pancreatic endocrine tumors varies from 64-92% in different series (Jensen, 1998).
    • One series of 25 patients with nonfunctioning neoplasms of the endocrine pancreas had 3- and 5-year survival rates of 60% and 44%, respectively, although most patients (92%) had metastatic disease (Kent, 1981).
    • Finally, radical surgery continues to have a central role in the therapeutic approach to endocrine tumors of the pancreas. Tumor resection, the absence of liver and lymph node metastases, and the presence of MEN 1 syndrome are related to a better survival rate (Tomassetti, 2005).

Miscellaneous

Medicolegal Pitfalls

  • Because neoplasms of the pancreas are rare and because their heralding signs and symptoms are often nonspecific, delays in diagnosis of as long as 4 years are reported (Welbourne, 1981; Norton, 1993; Metz, 1994). Because these tumors can be cured if identified early, such errors in clinical judgment can be profoundly devastating for patients.
  • As discussed in the Introduction, some patients, especially those with multiple lesions, have neoplasms of the endocrine pancreas as part of MEN 1 syndrome. If unrecognized, the hypercalcemia that results from hyperparathyroidism in these patients can lead to disastrous surgical complications; therefore, all patients who undergo surgical exploration for pancreatic endocrine tumors should be screened preoperatively for MEN 1.
  • Unnecessary surgical exploration in patients who surreptitiously use insulin can be avoided in those patients with possible insulinoma by first measuring serum levels of proinsulin, C peptide, and anti-insulin antibodies.
 


More on Neoplasms of the Endocrine Pancreas

Overview: Neoplasms of the Endocrine Pancreas
Differential Diagnoses & Workup: Neoplasms of the Endocrine Pancreas
Treatment & Medication: Neoplasms of the Endocrine Pancreas
Follow-up: Neoplasms of the Endocrine Pancreas
Multimedia: Neoplasms of the Endocrine Pancreas
References
[ CLOSE WINDOW ]

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Further Reading

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Keywords

pancreatic cancer, pancreas cancer, pancreatic neoplasm, pancreas neoplasm, pancreatic islet cell tumor, pancreatic islet cell adenoma, pancreatic endocrine tumor, pancreatic neuroendocrine tumor, insulinoma syndrome, insulinoma, Zollinger-Ellison syndrome, ZES, gastrinoma syndrome, gastrinoma, Verner-Morrison syndrome, WDHA syndrome, watery diarrhea, hypokalemia, achlorhydria, pancreatic cholera, vasoactive intestinal peptide-releasing tumor, VIPoma, glucagonoma syndrome, somatostatinoma syndrome, calcitoninoma, parathyrinoma, growth hormone releasing facto-secreting tumor, GRFoma, adrenocorticotropin hormone-secreting tumor, ACTHoma, neurotensinoma, pancreatic endocrine neoplasm, pancreatic polypeptidomas, PPomas, islet cell tumor, pancreatic cholera, MEN 1 syndrome, MEN-I syndrome, MEN-1 syndrome, multiple endocrine neoplasia type 1, multiple endocrine neoplasia type I, MEN syndrome, APUDomas

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Contributor Information and Disclosures

Author

Eric J Hanly, MD, Resident, Department of Surgery, The Johns Hopkins University School of Medicine; Fellow, Department of Surgery, The Johns Hopkins University School of Medicine
Eric J Hanly, MD is a member of the following medical societies: American Medical Association, Association of Military Surgeons of the US, MedChi, Phi Beta Kappa, and Society of USAF Flight Surgeons
Disclosure: Nothing to disclose.

Coauthor(s)

Ozanan R Meireles, MD, Research Fellow in Minimally Invasive Surgery, Department of Surgery, Johns Hopkins University School of Medicine
Ozanan R Meireles, MD is a member of the following medical societies: American College of Surgeons and Society of American Gastrointestinal and Endoscopic Surgeons
Disclosure: Nothing to disclose.

Michael R Marohn, DO, Associate Professor, Department of Surgery and Surgical Sciences, John Hopkins University School of Medicine
Disclosure: Nothing to disclose.

Charles J Yeo, MD, Samuel D Gross Professor and Chair of Surgery at Jefferson Medical College of Thomas Jefferson University and at Thomas Jefferson University Hospital
Charles J Yeo, MD is a member of the following medical societies: Alpha Omega Alpha, American Association of Endocrine Surgeons, American College of Surgeons, American Gastroenterological Association, American Hepato-Pancreato-Biliary Association, American Surgical Association, Association for Academic Surgery, Johns Hopkins Medical and Surgical Association, Pancreas Club, Phi Beta Kappa, Sigma Xi, Society for Surgery of the Alimentary Tract, and Society of University Surgeons
Disclosure: Nothing to disclose.

Keith D Lillemoe, MD, Professor and Chairman, Department of Surgery, Indiana University School of Medicine
Disclosure: Nothing to disclose.

Lisa H McGrail, MD, Associate Chief, Department of Hematology/Oncology, Malcolm Grow Medical Center
Disclosure: Nothing to disclose.

Medical Editor

Michael C Perry, MD, Professor, Department of Internal Medicine, Nellie B Smith Chair of Oncology, Director, Division of Hematology and Oncology, University of Missouri at Columbia/Ellis Fischel Cancer Center
Michael C Perry, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for Cancer Research, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Medical Association, American Society of Clinical Oncology, American Society of Hematology, Missouri State Medical Association, Southern Association for Oncology, and Southern Medical Association
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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