Somatostatinomas Treatment & Management

  • Author: Eric J Hanly, MD; Chief Editor: Julian Katz, MD   more...
 
Updated: Jan 11, 2010
 

Medical Care

  • Chemotherapeutic treatment of metastatic somatostatinoma usually consists of combination treatment with intravenous 5-fluorouracil (5-FU) and streptozotocin. Somatostatinomas are very rare; therefore, assessing the efficacy of chemotherapy is difficult, although up to 50% of patients have had an objective humoral and clinical response to this treatment. Doxorubicin and 5-FU have also been used and may be a more effective combination. Palliative hepatic embolization or chemoembolization is another treatment option for patients with unresectable hepatic metastatic disease.
  • Patients with metastatic and unresectable somatostatinomas also require specific symptomatic treatments. Associated diabetes can usually be controlled with oral agents and rarely requires the use of insulin. Pancreatic enzyme supplementation is useful to reduce diarrhea and steatorrhea.
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Surgical Care

  • Surgical resection is the only option that offers the hope of cure. The goals of surgical therapy are the same as for any pancreatic endocrine neoplasm, as follows:
    • To control symptoms of hormone excess
    • To safely resect maximal tumor mass
    • To preserve maximal pancreatic parenchyma
  • Though most somatostatinomas are localized preoperatively, surgical exploration begins with a complete evaluation of the pancreas and peripancreatic regions. Dividing the gastrocolic ligament exposes the body and tail of the pancreas. This portion of the pancreas can be partially elevated out of the retroperitoneum by dividing the inferior retroperitoneal attachments to the gland. After the second portion of the duodenum has been elevated out of the retroperitoneum using the Kocher maneuver, the pancreatic head and uncinate process are palpated bimanually. The liver is carefully assessed for evidence of metastatic disease. Though somatostatinomas are less likely to originate from extrapancreatic locations than gastrinomas, evaluate potential extrapancreatic tumor sites. Evaluation includes inspection, palpation, and ultrasonographic examination of the duodenum, splenic hilum, small intestine (and its mesentery), peripancreatic lymph nodes, and, in women, the reproductive tract.
  • Small (< 2 cm) benign lesions that are remote from the main pancreatic duct may be enucleated. Regional pancreatectomy is usually necessary for tumors that are deep in the substance of the pancreatic gland (and therefore near the main duct), have ill-defined capsules, or are large (>2 cm). Tumors in the body or tail of the pancreas can be managed with distal pancreatectomy, whereas lesions in the head or uncinate process of the gland can be resected via pancreaticoduodenectomy. Tumors in the neck of the pancreas can be managed with middle segment pancreatectomy (oversewing the proximal pancreatic stump and draining the distal pancreatic duct via pancreaticogastrostomy or pancreaticojejunostomy).
  • Debulking cytoreductive surgical resection of liver metastases can be performed in patients with limited hepatic spread. Unique among surgical treatment of pancreatic endocrine tumors, perform cholecystectomies during the first exploration of patients with somatostatinomas, so that if recurrence develops, cholelithiasis will not add to patient morbidity.
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Consultations

  • Endocrinologists
  • Gastroenterologists
  • Pancreatic or oncologic surgeons
  • Medical oncologists
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Diet

Fluid, electrolyte, and nutritional deficits are common complications of somatostatinoma syndrome. Patients in hormone-excess states may require parenteral supplementation.

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Activity

Encourage patients to engage in activity as tolerated. No specific activity requirements or restrictions exist.

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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)

Michael Cooperman, MD  Clinical Associate Professor of Endocrinology, Temple University; Chair, Department of Internal Medicine, Division of Endocrinology, Jeanes Hospital

Michael Cooperman, MD is a member of the following medical societies: Alpha Omega Alpha, American Association of Clinical Endocrinologists, and Endocrine Society

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.

Leon W Kundrotas, MD, Col, USAF, MC  Chief, Medical Staff 48th Medical Group, Malcolm Grow Medical Center; Assistant Professor, Department of Medicine, USAF Hospital

Leon W Kundrotas, MD, Col, USAF, MC is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, and American Society for Gastrointestinal Endoscopy

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.

Specialty Editor Board

Mounzer Al Al Samman, MD  Department of Internal Medicine, Division of Gastroenterology, Assistant Professor, Texas Tech University School of Medicine

Mounzer Al Al Samman, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, and American Gastroenterological Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Noel Williams, MD  Professor Emeritus, Department of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada; Professor, Department of Internal Medicine, Division of Gastroenterology, University of Alberta, Edmonton, Alberta, Canada

Noel Williams, MD is a member of the following medical societies: Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Alex J Mechaber, MD, FACP  Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD  Clinical Professor of Medicine, Drexel University College of Medicine; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania

Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law, Medicine & Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility

Disclosure: Nothing to disclose.

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