Primary Gallbladder Neuroendocrine Tumors: Insights into a Rare Histology Using a Large National Database
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Primary gallbladder neuroendocrine tumors (NETs) are rare, poorly understood cancers infrequently encountered at even the largest of tertiary referral centers. We therefore sought to identify a large cohort of patients with gallbladder NETs using a national database, with the aim of defining treatment modalities employed and survival associated with these uncommon malignancies.
Patients with primary gallbladder NETs were identified in the National Cancer Database, and clinicopathologic characteristics were recorded. A univariate log-rank survival analysis was completed for patients who underwent resection. Parameters found to be significant were entered into a multivariate accelerated failure time analysis. For context, survival comparisons were included for patients who underwent resections for NETs at any gastrointestinal site and for gallbladder adenocarcinoma.
Overall, 754 patients with gallbladder NETs were identified. Patients were predominantly female (n = 518, 69%), White (n = 503, 67%), presented with stage IV disease (n = 295, 39%) and had high-grade lesions (n = 312, 41%). The majority underwent resection (n = 480, 64%), primarily simple cholecystectomy (n = 431, 90%), whereas a minority received multimodal therapy (n = 145, 21%). Among patients who underwent resection, older age (p = 0.001), large cell histology (p = 0.012), and positive margins (p = 0.030) were independently associated with worse overall survival. Patients with gallbladder NETs had improved survival relative to those with gallbladder adenocarcinoma (p = 0.001), but significantly worse survival than patients with NETs from other gastrointestinal sites (p < 0.001).
Primary gallbladder NETs are aggressive lesions that carry a worse prognosis than NETs of other gastrointestinal sites. Older age, positive margins, and large cell histology are associated with abbreviated survival after resection.
This research did not receive any specific grants from funding agencies in the public, commercial, or not-for-profit sectors.
- 1.American Cancer Society. Cancer Facts & Figures 2017 Special Section: Rare Cancers in Adults. 2017. Available at: https://www.cancer.org/content/dam/cancer-org/research/cancer-facts-and-statistics/annual-cancer-facts-and-figures/2017/cancer-facts-and-figures-2017-special-section-rare-cancers-in-adults.pdf. Accessed 5 Nov 2018.
- 4.Eltawil KM, Gustafsson BI, Kidd M, Modlin IM. Neuroendocrine tumors of the gallbladder: an evaluation and reassessment of management strategy. J Clin Gastroenterol. 2010;44(10):687–95.Google Scholar
- 10.Amin MB, Edge SB, Greene FL, et al. AJCC cancer staging manual. 8th ed. Chicago: Springer; 2018.Google Scholar
- 11.NCCN practice guidelines in oncology: neuroendocrine and adrenal tumors. 2018. Available at: https://www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf. Accessed 31 Oct 2018.
- 18.Furrukh M, Qureshi A, Saparamadu A, Kumar S. Malignant neuroendocrine tumour of the gallbladder with elevated carcinoembryonic antigen: case report and literature review. BMJ Case Rep. 2013;2013.Google Scholar
- 23.Fong Y, Wagman L, Gonen M, et al. Evidence-based gallbladder cancer staging: changing cancer staging by analysis of data from the national cancer database. Ann Surg. 2006;243(6):767–71; discussion 771–764.Google Scholar
- 25.Mantripragada KC, Hamid F, Shafqat H, Olszewski AJ. Adjuvant therapy for resected gallbladder cancer: analysis of the national cancer data base. J Natl Cancer Inst. 2017;109(2).Google Scholar
- 28.Landry CS, Woodall C, Scoggins CR, McMasters KM, Martin RC, 2nd. Analysis of 900 appendiceal carcinoid tumors for a proposed predictive staging system. Arch Surg. 2008;143(7):664–70; discussion 670.Google Scholar
- 30.Kanakala V, Kasaraneni R, Smith DA, Goulbourne IA. Primary neuroendocrine neoplasm of the gallbladder. BMJ Case Rep. 2009;2009.Google Scholar
- 31.Lee JM, Hwang S, Lee SG, et al. Neuroendocrine tumors of the gallbladder: twelve cases in a single institution. Hepatogastroenterology. 2010;57(102–103):1064–8.Google Scholar