Status of Surveillance and Nonsurgical Therapy for Small Nonfunctioning Pancreatic Neuroendocrine Tumors. 2023

Dirk-Jan van Beek, and Anna Vera D Verschuur, and Lodewijk A A Brosens, and Gerlof D Valk, and Carolina R C Pieterman, and Menno R Vriens
Department of Endocrine Surgical Oncology, University Medical Center Utrecht, Internal Mail Number G.04.228, PO Box 85500, Utrecht 3508 GA, the Netherlands.

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.

UI MeSH Term Description Entries
D010190 Pancreatic Neoplasms Tumors or cancer of the PANCREAS. Depending on the types of ISLET CELLS present in the tumors, various hormones can be secreted: GLUCAGON from PANCREATIC ALPHA CELLS; INSULIN from PANCREATIC BETA CELLS; and SOMATOSTATIN from the SOMATOSTATIN-SECRETING CELLS. Most are malignant except the insulin-producing tumors (INSULINOMA). Cancer of Pancreas,Pancreatic Cancer,Cancer of the Pancreas,Neoplasms, Pancreatic,Pancreas Cancer,Pancreas Neoplasms,Pancreatic Acinar Carcinoma,Pancreatic Carcinoma,Acinar Carcinoma, Pancreatic,Acinar Carcinomas, Pancreatic,Cancer, Pancreas,Cancer, Pancreatic,Cancers, Pancreas,Cancers, Pancreatic,Carcinoma, Pancreatic,Carcinoma, Pancreatic Acinar,Carcinomas, Pancreatic,Carcinomas, Pancreatic Acinar,Neoplasm, Pancreas,Neoplasm, Pancreatic,Neoplasms, Pancreas,Pancreas Cancers,Pancreas Neoplasm,Pancreatic Acinar Carcinomas,Pancreatic Cancers,Pancreatic Carcinomas,Pancreatic Neoplasm
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D018242 Neuroectodermal Tumors, Primitive A group of malignant tumors of the nervous system that feature primitive cells with elements of neuronal and/or glial differentiation. Use of this term is limited by some authors to central nervous system tumors and others include neoplasms of similar origin which arise extracranially (i.e., NEUROECTODERMAL TUMORS, PRIMITIVE, PERIPHERAL). This term is also occasionally used as a synonym for MEDULLOBLASTOMA. In general, these tumors arise in the first decade of life and tend to be highly malignant. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, p2059) Ependymoblastoma,Medulloepithelioma,Neuroepithelial Tumors, Primitive,PNET,Spongioblastoma,Cerebral Primitive Neuroectodermal Tumor,Neoplasms, Primitive Neuroepithelial,Neuroectodermal Tumor, Primitive,Neuroepithelial Neoplasms, Primitive,Primitive Neuroepithelial Neoplasms,Ependymoblastomas,Medulloepitheliomas,Neoplasm, Primitive Neuroepithelial,Neuroepithelial Neoplasm, Primitive,Neuroepithelial Tumor, Primitive,PNETs,Primitive Neuroectodermal Tumor,Primitive Neuroectodermal Tumors,Primitive Neuroepithelial Neoplasm,Primitive Neuroepithelial Tumor,Primitive Neuroepithelial Tumors,Spongioblastomas,Tumor, Primitive Neuroectodermal,Tumor, Primitive Neuroepithelial,Tumors, Primitive Neuroectodermal,Tumors, Primitive Neuroepithelial
D018358 Neuroendocrine Tumors Tumors whose cells possess secretory granules and originate from the neuroectoderm, i.e., the cells of the ectoblast or epiblast that program the neuroendocrine system. Common properties across most neuroendocrine tumors include ectopic hormone production (often via APUD CELLS), the presence of tumor-associated antigens, and isozyme composition. Neuroendocrine Tumor,Tumor, Neuroendocrine,Tumors, Neuroendocrine
D018761 Multiple Endocrine Neoplasia Type 1 A form of multiple endocrine neoplasia that is characterized by the combined occurrence of tumors in the PARATHYROID GLANDS, the PITUITARY GLAND, and the PANCREATIC ISLETS. The resulting clinical signs include HYPERPARATHYROIDISM; HYPERCALCEMIA; HYPERPROLACTINEMIA; CUSHING DISEASE; GASTRINOMA; and ZOLLINGER-ELLISON SYNDROME. This disease is due to loss-of-function of the MEN1 gene, a tumor suppressor gene (GENES, TUMOR SUPPRESSOR) on CHROMOSOME 11 (Locus: 11q13). Neoplasia, Multiple Endocrine Type 1,Neoplasms, Multiple Endocrine Type 1,Wermer Syndrome,Multiple Endocrine Neoplasia Type I,Multiple Endocrine Neoplasms Type 1,Neoplasms, Multiple Endocrine Type I

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