Neuroendocrine- and mixed adenoneuroendocrine carcinomas (NEC, MANEC) of the
esophagus and stomach are very rare. Optimal treatment strategies remain unknown. Based
upon mostly outdated and partly Asian literature, survival is poor. This study aimed to
describe survival, choices of treatment and accuracy of biopsy diagnosis.
Patients with MANEC or NEC of the esophagus or stomach, who underwent surgical
resection between 2006-2016, were included from the nationwide network and registry of
histo- and cytopathology of the Netherlands (PALGA). Kaplan Meier survival analysis and
log-rank tests were used for calculating and comparing survival outcome.
A total of 47 patients (9 esophageal MANEC, 12 esophageal NEC, 8 gastric MANEC and 18
gastric NEC),were included. Neither for esophageal nor for gastric cancer did survival
significantly differ between NEC and MANEC patients (p=0.902 and p=0.237 respectively).
For esophageal cancer (NEC and MANEC pooled together), the median survival was 45
months. The 1-, 3- and 5-year overall survival (OS) were 74%, 52% and 38%, respectively.
For gastric cancer, the median survival was 41 months and 1-, 3- and 5-year OS were 73%,
58% and 45%, respectively.
Twenty out of 24 (NEC) and 3 out of 3 (MANEC) biopsy diagnosis were concordant with
diagnosis by resection . However, biopsy diagnosis of 17 adenocarcinoma and 3 other tumors,
were in fact 11 MANEC and 9 NEC on resection. Biopsy of recurrence was taken in 8
patients with MANEC, showing 5 NEC component, 3 MANEC and 0 adenocarcinoma
NEC and MANEC of esophagus and stomach showed a relatively good survival after
resection, comparable with those of adenocarcinoma and squamous cell carcinoma from
literature. Curative treatment should therefore be attempted when feasible. NEC and MANEC
had similar survival. Recurrence of MANEC never contained an isolated adenocarcinoma
component, suggesting MANEC should be treated as NEC.
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