Periampullary pancreatic somatostatinoma

Ann Surg Oncol. 2002 Nov;9(9):869-74. doi: 10.1007/BF02557523.


Background: Somatostatinomas involving the gastrointestinal tract are extremely rare neoplasms that typically present with indolent, nonspecific symptoms in the absence of systemic neuroendocrine manifestations that characterize the somatostatinoma syndrome. Because of a relatively large size at the time of presentation (average diameter of 5 cm) and common location within the head of the pancreas, the Whipple procedure (pancreaticoduodenectomy) serves as the predominant modality for curative and palliative surgical approaches.

Methods: Two cases of somatostatinoma involving the minor duodenal papilla with concomitant pancreatic divisum were reviewed, with a general overview of this unique islet cell tumor.

Results: Unlike typical somatostatinomas, these two tumors were subcentimeter in size but were associated with synchronous regional metastasis.

Conclusions: Somatostatinomas are often associated with regional and/or portal metastases at the time of diagnosis, and only 60% to 70% of surgical cases result in complete tumor resection. Predictors of an unfavorable prognosis include size >3 cm, poor cytological differentiation, regional and/or portal metastasis, and incomplete surgical resection. Even in the presence of synchronous metastases, the 5-year overall survival for patients with somatostatinoma is approximately 40%. Currently, there are no clinical trials demonstrating significant improvement in survival with the use of adjuvant therapy.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Cholangiopancreatography, Endoscopic Retrograde
  • Female
  • Humans
  • Male
  • Middle Aged
  • Pancreatic Ducts* / pathology
  • Pancreatic Neoplasms / diagnostic imaging
  • Pancreatic Neoplasms / surgery*
  • Pancreaticoduodenectomy*
  • Somatostatinoma / diagnostic imaging
  • Somatostatinoma / surgery*
  • Tomography, X-Ray Computed