BACKGROUND: Primary duodenal gastrinomas are now recognized as a common etiology for patients with sporadic Zollinger Ellison Syndrome (ZES); however, the clinical and pathologic features of this condition and long-term outcome after operation are not well characterized. METHODS: Between November 1982 and September 2000, 63 patients diagnosed with sporadic ZES underwent resection of a primary duodenal gastrinoma and regional nodal metastases with curative intent. Data from a prospectively maintained database were reviewed for clinical and pathologic parameters relating to primary tumor size, location, frequency of lymph node metastases, and disease-specific and disease-free survival. RESULTS: There were 41 males and 22 females (mean age, 48.6 years). The majority of duodenal gastrinomas were in the first or second portions of the duodenum (83%). Tumor size ranged from 0.2 to 2.0 cm with 62% measuring less than 1.0 cm. Sixty percent of individuals had regional lymph node metastases identified primarily in proximity to the primary tumor. At a median 10-year follow-up, the overall disease-specific and disease-free survivals were 100% and 60%, respectively. Actuarial 10-year disease-free survival was significantly higher for patients without lymph node metastases versus those with lymph node metastases (78% versus 48%, P = 0.0137). CONCLUSIONS: Duodenal gastrinomas in patients with sporadic ZES are frequently small, most commonly located in the proximal duodenum, and associated with regional lymph node metastases in 60%. Disease-free survival is lower for patients with regional lymph node metastases suggesting that a more systematic lymphadenectomy to extirpate occult disease may be indicated in this group.
BACKGROUND: Primary duodenal gastrinomas are now recognized as a common etiology for patients with sporadic Zollinger Ellison Syndrome (ZES); however, the clinical and pathologic features of this condition and long-term outcome after operation are not well characterized. METHODS: Between November 1982 and September 2000, 63 patients diagnosed with sporadic ZES underwent resection of a primary duodenal gastrinoma and regional nodal metastases with curative intent. Data from a prospectively maintained database were reviewed for clinical and pathologic parameters relating to primary tumor size, location, frequency of lymph node metastases, and disease-specific and disease-free survival. RESULTS: There were 41 males and 22 females (mean age, 48.6 years). The majority of duodenal gastrinomas were in the first or second portions of the duodenum (83%). Tumor size ranged from 0.2 to 2.0 cm with 62% measuring less than 1.0 cm. Sixty percent of individuals had regional lymph node metastases identified primarily in proximity to the primary tumor. At a median 10-year follow-up, the overall disease-specific and disease-free survivals were 100% and 60%, respectively. Actuarial 10-year disease-free survival was significantly higher for patients without lymph node metastases versus those with lymph node metastases (78% versus 48%, P = 0.0137). CONCLUSIONS: Duodenal gastrinomas in patients with sporadic ZES are frequently small, most commonly located in the proximal duodenum, and associated with regional lymph node metastases in 60%. Disease-free survival is lower for patients with regional lymph node metastases suggesting that a more systematic lymphadenectomy to extirpate occult disease may be indicated in this group.
Authors: J A Norton; D T Cromack; T H Shawker; J L Doppman; R Comi; P Gorden; P N Maton; J D Gardner; R T Jensen Journal: Ann Surg Date: 1988-02 Impact factor: 12.969
Authors: J A Norton; D L Fraker; H R Alexander; D J Venzon; J L Doppman; J Serrano; S U Goebel; P L Peghini; P K Roy; F Gibril; R T Jensen Journal: N Engl J Med Date: 1999-08-26 Impact factor: 91.245
Authors: S A Wank; J L Doppman; D L Miller; M J Collen; P N Maton; R Vinayek; J I Slaff; J A Norton; J D Gardner; R T Jensen Journal: Gastroenterology Date: 1987-04 Impact factor: 22.682
Authors: Jeffrey A Norton; H Richard Alexander; Douglas L Fraker; David J Venzon; Fathia Gibril; Robert T Jensen Journal: Ann Surg Date: 2004-05 Impact factor: 12.969
Authors: M Johannes Rosentraeger; Nele Garbrecht; Martin Anlauf; Andreas Raffel; Wolfram T Knoefel; Bertram Wiedenmann; Günter Klöppel Journal: Virchows Arch Date: 2015-12-09 Impact factor: 4.064