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Periampullary Pancreatic Somatostatinoma

From the Department of Surgery, The Johns Hopkins Hospital, Baltimore, Maryland.

Correspondence: Address correspondence and reprint requests to: Richard D. Schulick, MD, The Johns Hopkins Hospital, 600 N. Wolfe St., Blalock 657, Baltimore, MD 21287; Fax: 410-614-9882; E-mail: rschulick@ jhmi.edu.

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.

Key Words: Somatostatinoma • Pancreas • Islet cell • Neuroendocrine • Whipple procedure • Pancreaticoduodenectomy

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