Periampullary pancreatic somatostatinoma

Michael G. House, Charles J. Yeo, Richard D. Schulick

Research output: Contribution to journalArticle

46 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)869-874
Number of pages6
JournalAnnals of Surgical Oncology
Volume9
Issue number9
DOIs
StatePublished - Nov 2002
Externally publishedYes

Keywords

  • Islet cell
  • Neuroendocrine
  • Pancreas
  • Pancreaticoduodenectomy
  • Somatostatinoma
  • Whipple procedure

ASJC Scopus subject areas

  • Surgery
  • Oncology

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