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.
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.
Authors: Radoje B Colović; Slavko V Matić; Marjan T Micev; Nikica M Grubor; Henry Dushan Atkinson; Stojan M Latincić Journal: World J Gastroenterol Date: 2009-12-14 Impact factor: 5.742
Authors: Jung A Kim; Won-Ho Choi; Chul Nam Kim; Young Soo Moon; Sun Hee Chang; Hye Ran Lee Journal: Korean J Intern Med Date: 2011-03-02 Impact factor: 2.884