Background
The incidence, clinical characteristics, and long-term outcomes of patients with gastroenteropancreatic
neuroendrocrine tumors and carcinoid syndrome undergoing operative resection have
not been well characterized.
Methods
Patients undergoing resection of primary or metastatic gastroenteropancreatic neuroendrocrine
tumors between 2000 and 2016 were identified from an 8-institution collaborative database.
Clinicopathologic and postoperative characteristics as well as overall survival and
disease-free survival were compared among patients with and without carcinoid syndrome.
Results
Among 2,182 patients who underwent resection, 139 (6.4%) had preoperative carcinoid
syndrome. Patients with carcinoid syndrome were more likely to have midgut primary
tumors (44.6% vs 21.4%, P < .001), lymph node metastasis (63.4% vs 44.3%, P < .001), and metastatic disease (62.8% vs 26.7%, P < .001). There was no difference in tumor differentiation, grade, or Ki67 status.
Perioperative carcinoid crisis was rare (1.6% vs 0%, P < .01), and the presence of preoperative carcinoid syndrome was not associated with
postoperative morbidity (38.8% vs 45.5%, P = .129). Substantial symptom improvement was reported in 59.5% of patients who underwent
curative-intent resection, but occurred in only 22.7% who underwent debulking. Despite
an association on univariate analysis (P = .04), carcinoid syndrome was not independently associated with disease-free survival
after controlling for confounding factors (hazard ratio 0.97, 95% confidence interval
0.64–1.45). Preoperative carcinoid syndrome was not associated with overall survival on
univariate or multivariate analysis.
Conclusion
Among patients undergoing operative resection of gastroenteropancreatic neuroendrocrine
tumors, the prevalence of preoperative carcinoid syndrome was low. Although operative intervention with resection or especially debulking in patients
with carcinoid syndrome was disappointing and often failed to improve symptoms, after
controlling for markers of tumor burden, carcinoid syndrome was not independently
associated with worse disease-free survival or overall survival.
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Article info
Publication history
Published online: October 27, 2018
Accepted:
September 15,
2018
Received in revised form:
September 10,
2018
Received:
June 15,
2018
Identification
Copyright
© 2018 Elsevier Inc. All rights reserved.
