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Need, timing of surgery must be weighed against potential benefits in MEN1
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In adults with multiple endocrine neoplasia type 1, or MEN1, the timing and extent of surgical intervention for pancreaticoduodenal neuroendocrine tumors must be weighed against the risk for meaningful morbidity, including exocrine and endocrine pancreatic insufficiency and worsening quality of life, according to a retrospective analysis of patient data.
“Although the ideal treatment strategy for [pancreaticoduodenal neuroendocrine tumors] is uncertain, it is clear that the overall goal is to control symptoms and disease burden without any detrimental effect on the quality of life,” Diane Donegan, MD, of the division of endocrinology, diabetes, metabolism and nutrition at the Mayo Clinic in Rochester, Minnesota, and colleagues wrote. “However, there is paucity of data to help clinicians identify the exact time in which the benefits of treatment (surgical or medical) outweigh the possible risks. In fact, researchers have been unable to find a genotype/phenotype correlation, and markers of aggressive disease (rapid growth and or malignant potential) remain elusive.”
In a retrospective study, Donegan and colleagues analyzed electronic medical records from 287 patients with a diagnosis of MEN1 who attended the Mayo Clinic between 1997 and 2014 (167 women; mean age at diagnosis, 37 years), assessing biochemical testing, radiologic imaging, surgical procedures, associated complications and pathology results. Primary hyperparathyroidism was the most common clinical manifestation (n = 255), followed by 199 patients with 217 pancreaticoduodenal neuroendocrine tumors (PD-NETS).
Among patients with PD-NETS, 128 underwent surgery (90 procedures performed at Mayo Clinic); patients were followed postoperatively for a mean of 8 years. During follow-up, 13 patients who underwent surgery died (mean age at death, 51 years); probability of survival after surgery for those with metastases at surgery vs. patients without metastasis at surgery was not statistically different (HR = 2.2; 95% CI, 0.7-6.6).
“There was no association between sex or size of the largest tumor at surgery and the presence of metastasis,” the researchers wrote. “For each year older a patient was at the time of surgery, the odds of metastasis increased by 6%.”
Among patients with PD-NETs who underwent active surveillance, median tumor growth (calculated in 21 patients) was 0.02 cm per year; four patients died (mean age, 77 years).
“Among those with PD-NETs who are surgical candidates, considering surgery early may be beneficial as we showed that for every year older, the presence of metastasis increased by 6%,” the researchers wrote. “On the other hand, limitations of the available evidence (small sample size, selection bias, short duration of follow-up, heterogeneity in populations
and treatment) and concerns regarding the morbidity, economic and emotional burden of early and aggressive treatment strategies add complexity to our treatment decisions.” – by Regina Schaffer
Disclosure: The researchers report no relevant financial disclosures.
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