Gastroenteropancreatic neuendocrine tumors (GEPNETs) share a common cell of origin, the neuroendocrine 12 cell, diffusely spread throughout the body. They represent a group with numerous subtypes whose primaries 13 arise in numerous locations and tissues, have widely differing presentations, diagnostic approaches and benign 14 to dire prognoses. Though sharing a common cell type, many subtypes of GEPNETs have defied "lumping" in 15 efforts to better understand their basic biology, clinical characterization, or to organize large clinical trials to 16 define diagnostic and therapeutic approaches. Of all GEPNETs, neuroendocrine carcinoma (GEPNEC) is the 17 most aggressive. GEPNEC is a rare and highly aggressive cancer with a poor prognosis and rapid disease 18 progression, comprising less than 1% of all digestive system malignancies. Data indicate that staging, primary 19 tumor site, and histology may be associated with the prognosis of GEPNEC. Their study provides strong evidence for a survival benefit for surgical cytoreduction over 37 chemotherapy across many presentations. A close reading of the included manuscripts, though acknowledging 38 the limitations of retrospective design and lack of granularity and incomplete details of large datasets, will 39 provide important advances in understanding these rare tumors, planning further studies, and approaching 40 clinical decision making for often challenging cases.
Vosburgh et al. (Mon,) studied this question.