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Pancreatic neuroendocrine tumors (PNETs) occur in < 1/100,000 patients and most are nonfunctioning (NF). Approximately 5% occur as part of multiple endocrine neoplasia type 1. Anatomic and molecular imaging have a pivotal role in the diagnosis, staging and active surveillance. Surgery is generally recommended for nonfunctional pancreatic neuroendocrine tumors (NF-PNETs) >2 cm to prevent metastases. For tumors ≤2 cm, active surveillance is a viable alternative. Tumor size and grade are important factors to guide management. Assessment of death domain-associated protein 6/alpha-thalassemia/mental retardation X-linked and alternative lengthening of telomeres are promising novel prognostic markers. This review summarizes the status of surveillance and nonsurgical management for small NF-PNETs, including factors that can guide management.
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http://dx.doi.org/10.1016/j.soc.2022.10.010 | DOI Listing |
Endocr Relat Cancer
September 2025
Endocrinology, Diabetology and Medical Andrology Unit, IRCCS, Humanitas Research Hospital, Rozzano, Milan, Italy.
Bone metastases (BMs) are rare and late event in patients with neuroendocrine tumors (NETs). The aim of our study was to investigate clinical presentation and outcome of BMs in a large cohort of patients with NETs. A retrospective study was performed at two referral centers of Northern Italy (IRCCS Humanitas Research Hospital in Milan and S.
View Article and Find Full Text PDFCureus
August 2025
Surgery and Anatomy, Hospital das Clinicas da Faculdade de Medicina de Ribeirão Preto - Universidade de São Paulo, Ribeirão Preto, BRA.
The Von Hippel-Lindau disease (VHL) is an autosomal dominant condition characterized by multiple cystic tumors in several organs, including the pancreas. The symptoms are variable, and suspicion must be raised with typical lesions, such as a hemangioblastoma of the central nervous system (CNS) or retina, associated with a renal cell carcinoma, a pheochromocytoma or multiple pancreatic cysts, besides neuroendocrine tumors (NET). The diagnosis in a patient without a family history should be suspected in case of a hemangioblastoma of the CNS and/or retina, which could also be associated with other lesions, such as pancreatic cysts and NETs.
View Article and Find Full Text PDFCan J Ophthalmol
September 2025
University of British Columbia, Department of Ophthalmology and Visual Sciences, Vancouver, BC, Canada.
Objective: To assess the effect of belzutifan, a first-in-class oral hypoxia-inducible factor 2α inhibitor, on retinal hemangioblastoma (RH) outcomes.
Subjects/methods: This is a single-centre retrospective cohort study of patients with confirmed von Hippel-Lindau syndrome (VHLS) and RH. Subjects were taking oral belzutifan for renal cell carcinoma, central nervous system hemangioblastoma, or pancreatic neuroendocrine tumours.
Chromogranin A (CgA), a neuroendocrine pro-hormone, undergoes proteolytic cleavage to yield bioactive peptides, notably catestatin (CST) and pancreastatin (PST), which exert opposing effects on metabolic and inflammatory processes. Using CgA and CST knockout (KO) mice, this study investigated their roles in pancreatic endocrine function, morphology, neurotransmitter dynamics, and systemic glucose homeostasis. CST deficiency induced insulin resistance, altered islet architecture, and heightened catecholamine levels, whereas CgA-KO mice lacking both CST and PST exhibited improved insulin sensitivity due to absence of PST.
View Article and Find Full Text PDFFront Med (Lausanne)
August 2025
Department of Pathology, Molecular and Cell-Based Medicine, Icahn School of Medicine at Mount Sinai, New York, NY, United States.
"Primary neuroendocrine breast carcinoma (NEBC) is an underdiagnosed subtype of breast cancer, which includes small cell (SCNEC) and large cell neuroendocrine carcinomas (LCNEC). Accurate diagnosis remains challenging given their low incidence; misclassification as invasive breast carcinoma of no special type (IBC-NST), invasive ductal carcinoma (IDC), or a metastatic neuroendocrine carcinoma may occur. Cases with any component of adenocarcinoma and well-differentiated neuroendocrine tumors were excluded.
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