Conserved neuronal-like and secretory programs define the spatial architecture of gastroenteropancreatic neuroendocrine tumors

Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are clinically heterogeneous malignancies whose biology and microenvironmental organization remain poorly understood. Here, we integrated single-nucleus multiomic (snRNA-seq and snATAC-seq) and spatial transcriptomic profiling across 38 well-differentiated pancreatic (PanNET) and small-intestinal (siNET) tumors to define conserved malignant programs, their regulatory circuits, and spatial niches. We observed two conserved malignant cell programs spanning a continuous transcriptional spectrum: a neuronal-like program, and a secretory neuroendocrine program. Matched chromatin accessibility profiles uncovered distinct, tissue-specific regulatory networks, including MAX::MYC and MITF transcription factor binding motifs in siNETs versus ISL1 and TFAP4 in PanNETs, indicating organ-specific epigenetic control. Spatial transcriptomic analyses revealed that neuronal-like-high regions localized to densely cellular tumor areas with relative depletion of stromal infiltration, whereas secretory neuroendocrine-high regions occupied fibrovascular and stromal niches enriched for endothelial, fibroblast, and myeloid populations, and associated with TGFB1-ITGB1, VEGFA-FLT1, and LAMA2-ITGA1 signaling. Across both tumor types, the cNMF2 program was enriched in metastatic lesions and was enriched for pro-fibrotic and pro-angiogenic gene signatures. Thus, GEP-NETs are organized along a conserved neuronal-to-secretory axis defined by distinct epigenetic programs and spatially coupled to specific microenvironmental niches. This framework unifies NET heterogeneity across organ sites and identifies pathway-specific, microenvironment-linked vulnerabilities for therapeutic targeting.

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Publication Details

Journal
Journal of Clinical Investigation
Published
2026-09-01
DOI
https://doi.org/10.1172/jci204119
Primary Topic
Neuroendocrine Tumor Research Advances
Type
article
Field-Weighted Citation Impact
0.00

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article

Conserved neuronal-like and secretory programs define the spatial architecture of gastroenteropancreatic neuroendocrine tumors

Erica Pimenta, Dan Y. Gui, Lauren K. Brais, Eliezer M. Van Allen et al.
Journal of Clinical Investigation
Neuroendocrine Tumor Research Advances
article

Conserved neuronal-like and secretory programs define the spatial architecture of gastroenteropancreatic neuroendocrine tumors

Erica Pimenta, Dan Y. Gui, Lauren K. Brais, Eliezer M. Van Allen, Hannah I. Hoffman, Samantha E. Hoffman, Kevin Bi, Erin Shannon, Θεοδώρα Παππά, Jason L. Hornick, Julie Karam, Laura Valderrábano, Riaz Gillani, Jihye Park, Yutaro Tanaka, Amanda Garza, Jihye Park, Julie Karam, Erica Pimenta, Riaz Gillani, Breanna M. Titchen, Jennifer Chan
article en

Abstract

Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are clinically heterogeneous malignancies whose biology and microenvironmental organization remain poorly understood. Here, we integrated single-nucleus multiomic (snRNA-seq and snATAC-seq) and spatial transcriptomic profiling across 38 well-differentiated pancreatic (PanNET) and small-intestinal (siNET) tumors to define conserved malignant programs, their regulatory circuits, and spatial niches. We observed two conserved malignant cell programs spanning a continuous transcriptional spectrum: a neuronal-like program, and a secretory neuroendocrine program. Matched chromatin accessibility profiles uncovered distinct, tissue-specific regulatory networks, including MAX::MYC and MITF transcription factor binding motifs in siNETs versus ISL1 and TFAP4 in PanNETs, indicating organ-specific epigenetic control. Spatial transcriptomic analyses revealed that neuronal-like-high regions localized to densely cellular tumor areas with relative depletion of stromal infiltration, whereas secretory neuroendocrine-high regions occupied fibrovascular and stromal niches enriched for endothelial, fibroblast, and myeloid populations, and associated with TGFB1-ITGB1, VEGFA-FLT1, and LAMA2-ITGA1 signaling. Across both tumor types, the cNMF2 program was enriched in metastatic lesions and was enriched for pro-fibrotic and pro-angiogenic gene signatures. Thus, GEP-NETs are organized along a conserved neuronal-to-secretory axis defined by distinct epigenetic programs and spatially coupled to specific microenvironmental niches. This framework unifies NET heterogeneity across organ sites and identifies pathway-specific, microenvironment-linked vulnerabilities for therapeutic targeting.

Journal of Clinical Investigation
Broad Institute (US), Brigham and Women's Hospital (US), Boston Children's Hospital (US), Joslin Diabetes Center (US), Harvard University (US), Harvard University Press (US), Harvard–MIT Division of Health Sciences and Technology (US), Dana-Farber Cancer Institute (US)
Brigham and Women's Hospital, Broad Institute, Robert O. and Annamae Orr Family Foundation, National Institutes of Health, National Institute of General Medical Sciences
Openalex Percentile: Top 99%
Neuroendocrine Tumor Research Advances
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