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Life expectancy low in MEN1 duodenopancreatic neuroendocrine tumor liver metastases
Half of patients with multiple endocrine neoplasia type 1, or MEN1, who developed duodenopancreatic neuroendocrine tumor liver metastases died within 10 years, according to researchers in the Netherlands.
“In historical series, ulcerous disease due to gastrin excess was reported as the most important cause of MEN1-related death. However, since the introduction of H2 receptor antagonists and proton pump inhibitors, metastatic [duodenopancreatic neuroendocrine tumor] is the leading cause of death,” Elfi B. Conemans, MD, of the department of endocrine oncology at the University Medical Center Utrecht in the Netherlands, and colleagues wrote. “Reliable data on survival of MEN1 patients with liver metastases from [duodenopancreatic neuroendocrine tumors] are not available so far. Prognosis for advanced disease in MEN1 might be different from sporadic cases, due to factors such as younger age at diagnosis, earlier tumor detection due to screening programs, multiplicity of [duodenopancreatic neuroendocrine tumors] and comorbidity due to other (neuroendocrine) tumors.”
Conemans and colleagues performed a cohort study using data from the Dutch National MEN1 database, which includes more than 90% of the Dutch population with MEN1 from 1990 to 2014. The researchers assessed overall survival and prognostic factors.
Fifty-six percent (n = 220) of patients were diagnosed with duodenopancreatic neuroendocrine tumors, with 34 (15%) of those developing liver metastases. The patients’ median age at the diagnosis of metastases was 53 years. Overall survival at 2, 5 and 10 years was 91%, 65% and 50%, respectively, the researchers reported. Men had worse survival than women (5-year overall survival, 58% vs. 75%; P = .07), and patients with multiple liver metastases had worse survival compared with patients with a solitary metastasis (59% vs. 83%; P = .09).
“Although progression of liver metastases from [duodenopancreatic neuroendocrine tumors] in MEN1 can be fairly slow, life expectancy of this relatively young patient group is clearly reduced as the 10-year overall survival rate is only 50%,” the researchers wrote. “Our findings suggest an association between overall survival and both gender and tumor load in patients with MEN1 [duodenopancreatic neuroendocrine tumor] liver metastases. Prospective clinical studies are warranted to see whether gender and tumor load status are of prognostic value in MEN1 screening and surveillance practices.” – by Andy Polhamus
Disclosure: The researchers report no relevant financial disclosures.