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Value of surgery in patients with negative imaging and sporadic Zollinger-Ellison syndrome.
Ann Surg. 2012 Sep; 256(3):509-17.AnnS

Abstract

OBJECTIVES

To address the value of surgery in patients with sporadic Zollinger-Ellison syndrome (ZES) with negative imaging studies.

BACKGROUND

Medical control of acid hypersecretion in patients with sporadic ZES is highly effective. This has led to these patients frequently not being sent to surgery, especially if preoperative imaging studies are negative, due, in large part, to existence of almost no data on the success of surgery in this group.

METHODS

Fifty-eight prospectively studied patients with sporadic ZES (17% of total studied) had negative imaging studies, and their surgical outcome was compared with 117 patients with positive imaging results.

RESULTS

Thirty-five patients had negative imaging studies in the pre-somatostatin receptor scintigraphy (SRS) era, and 23 patients in the post-SRS era. Patients with negative imaging studies had long disease histories before surgery [mean ± SEM (from onset) = 7.9 ± 1 [range, -0.25 to 35 years]) and 25% were followed for 2 or more years from diagnosis. At surgery, gastrinoma was found in 57 of 58 patients (98%). Tumors were small (mean = 0.8 cm, 60% <1 cm). The most common primary sites were duodenal 64%, pancreatic 17%, and lymph node (10%). Fifty percent had a primary-only, 41% primary + lymph node, and 7% had liver metastases. Thirty-five of 58 patients (60%) were cured immediately postoperatively, and at last follow-up [mean = -9.4 years; range, 0.2-22 years], 27 patients (46%) remained cured. During follow-up, 3 patients died, each had liver metastases at surgery. In comparison to positive imaging patients, those with negative imaging studies had lower preoperative fasting gastrin levels; had a longer delay before surgery; more frequently had a small duodenal tumor; less frequently had a pancreatic tumor, multiple tumors, or developed a new lesion postoperatively; and had a longer survival.

CONCLUSIONS

Sporadic ZES patients with negative imaging studies are not rare even in the post-SRS period. An experienced surgeon can find gastrinoma in almost every patient (98%) and nearly one half (46%) are cured, a rate similar to patients with positive imaging findings. Because liver metastases were found in 7%, which may have been caused by a long delay in surgery and all the disease-related deaths occurred in this group, surgery should be routinely undertaken early in ZES patients despite negative imaging studies.

Authors+Show Affiliations

Stanford University Medical Center, Stanford, CA 94305, USA. janorton@stanford.eduNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Clinical Trial
Comparative Study
Journal Article

Language

eng

PubMed ID

22868363

Citation

Norton, Jeffrey A., et al. "Value of Surgery in Patients With Negative Imaging and Sporadic Zollinger-Ellison Syndrome." Annals of Surgery, vol. 256, no. 3, 2012, pp. 509-17.
Norton JA, Fraker DL, Alexander HR, et al. Value of surgery in patients with negative imaging and sporadic Zollinger-Ellison syndrome. Ann Surg. 2012;256(3):509-17.
Norton, J. A., Fraker, D. L., Alexander, H. R., & Jensen, R. T. (2012). Value of surgery in patients with negative imaging and sporadic Zollinger-Ellison syndrome. Annals of Surgery, 256(3), 509-17. https://doi.org/10.1097/SLA.0b013e318265f08d
Norton JA, et al. Value of Surgery in Patients With Negative Imaging and Sporadic Zollinger-Ellison Syndrome. Ann Surg. 2012;256(3):509-17. PubMed PMID: 22868363.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Value of surgery in patients with negative imaging and sporadic Zollinger-Ellison syndrome. AU - Norton,Jeffrey A, AU - Fraker,Douglas L, AU - Alexander,H Richard, AU - Jensen,Robert T, PY - 2012/8/8/entrez PY - 2012/8/8/pubmed PY - 2012/10/23/medline SP - 509 EP - 17 JF - Annals of surgery JO - Ann. Surg. VL - 256 IS - 3 N2 - OBJECTIVES: To address the value of surgery in patients with sporadic Zollinger-Ellison syndrome (ZES) with negative imaging studies. BACKGROUND: Medical control of acid hypersecretion in patients with sporadic ZES is highly effective. This has led to these patients frequently not being sent to surgery, especially if preoperative imaging studies are negative, due, in large part, to existence of almost no data on the success of surgery in this group. METHODS: Fifty-eight prospectively studied patients with sporadic ZES (17% of total studied) had negative imaging studies, and their surgical outcome was compared with 117 patients with positive imaging results. RESULTS: Thirty-five patients had negative imaging studies in the pre-somatostatin receptor scintigraphy (SRS) era, and 23 patients in the post-SRS era. Patients with negative imaging studies had long disease histories before surgery [mean ± SEM (from onset) = 7.9 ± 1 [range, -0.25 to 35 years]) and 25% were followed for 2 or more years from diagnosis. At surgery, gastrinoma was found in 57 of 58 patients (98%). Tumors were small (mean = 0.8 cm, 60% <1 cm). The most common primary sites were duodenal 64%, pancreatic 17%, and lymph node (10%). Fifty percent had a primary-only, 41% primary + lymph node, and 7% had liver metastases. Thirty-five of 58 patients (60%) were cured immediately postoperatively, and at last follow-up [mean = -9.4 years; range, 0.2-22 years], 27 patients (46%) remained cured. During follow-up, 3 patients died, each had liver metastases at surgery. In comparison to positive imaging patients, those with negative imaging studies had lower preoperative fasting gastrin levels; had a longer delay before surgery; more frequently had a small duodenal tumor; less frequently had a pancreatic tumor, multiple tumors, or developed a new lesion postoperatively; and had a longer survival. CONCLUSIONS: Sporadic ZES patients with negative imaging studies are not rare even in the post-SRS period. An experienced surgeon can find gastrinoma in almost every patient (98%) and nearly one half (46%) are cured, a rate similar to patients with positive imaging findings. Because liver metastases were found in 7%, which may have been caused by a long delay in surgery and all the disease-related deaths occurred in this group, surgery should be routinely undertaken early in ZES patients despite negative imaging studies. SN - 1528-1140 UR - https://www.unboundmedicine.com/medline/citation/22868363/Value_of_surgery_in_patients_with_negative_imaging_and_sporadic_Zollinger_Ellison_syndrome_ L2 - http://Insights.ovid.com/pubmed?pmid=22868363 DB - PRIME DP - Unbound Medicine ER -