Pancreatic endocrine tumors (PETs) are uncommon tumors with an annual incidence <1 per 100 000 person-years in the general population. The PETs that produce hormones resulting in symptoms are designated as functional. The majority of PETs are non-functional. Of the functional tumors, insulinomas are the most common, followed by gastrinomas. The clinical course of patients with PETs is variable and depends on the extent of the disease and the treatment rendered. Patients with completely resected tumors generally have a good prognosis, and aggressive surgical therapy in patients with advanced disease may also prolong survival. The epidemiology, prognosis, and established and novel prognostic markers of PETs are reviewed.
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Thorvardur R Halfdanarson, Joseph Rubin, Michael B Farnell, Clive S Grant, and Gloria M Petersen
Alaa Sada, Travis J McKenzie, Adrian Vella, Michael J Levy, and Thorvardur R Halfdanarson
Localized insulinoma is an uncommon entity that can result in substantial morbidity due to the associated hypoglycemia. Recent studies have suggested an increase in the incidence of insulinoma in recent decades that may possibly be secondary to increased awareness, incidental diagnoses, and better diagnostic methods. Diagnosing and localizing insulinoma within the pancreas can be challenging, but advances in nuclear imaging may improve diagnostic accuracy. Delays in diagnosis are common, but once a localized insulinoma is diagnosed and appropriately treated, the long-term prognosis is excellent. Surgical resection is considered the standard of care management option for localized insulinoma, but tumor ablation with endoscopic ultrasound guidance has also been shown to be an effective and safe method for therapy.
Mojun Zhu, Karl R Sorenson, Rebecca Liu, Bonnie E Gould Rothberg, and Thorvardur R Halfdanarson
Pancreatic neuroendocrine tumors (PNETs) encompass a diverse group of malignancies marked by histological heterogeneity and highly variable clinical outcomes. We performed a systematic review on potential prognostic biomarkers in PNETs by searching the PubMed database. A total of 472 manuscripts were reviewed in detail, of which 52 multivariate studies met the inclusion criteria proposed by the Reporting Recommendations for Tumor Marker Prognostic Studies. These altogether analyzed 53 unique targets, and 36 of them were statistically associated with survival.
Jennifer R Eads, Thorvardur R Halfdanarson, Tim Asmis, Andrew M. Bellizzi, Emily K Bergsland, Arvind Dasari, Ghassan El-Haddad, Michael Frumovittz, Joshua Meyer, Erik Mittra, Sten Myrehaug, Eric Nakakura, Nitya Raj, Heloisa P Soares, Brian Untch, Namrata Vijayvergia, and Jennifer A Chan
High grade neuroendocrine tumors (G3 NET) and neuroendocrine carcinomas (NEC) are a rare disease entity for which there is a limited amount of prospective data available. It has also been relatively recent that a distinction has been made between G3 NET and NEC, further complicating the approach for management. Due to the overall lack of data in this space, much of the practice for treatment of these tumors is based on expect opinion. The North American Neuroendocrine Tumor Society (NANETS) is an organization composed of experts who specialize in the management of these tumors. In an effort to provide further guidance to the oncology community in regard to the management of these rare tumors, NANETS convened a panel of experts from medical oncology, surgery, interventional radiology, nuclear medicine, radiology, pathology and radiation oncology to address critical issues surrounding the management of G3 NET and NEC that would benefit from expert consensus opinion. Here we present the recommendations from this panel of neuroendocrine experts in regard to the management of high grade, extra-pulmonary neuroendocrine tumors (G3 NET) and neuroendocrine carcinomas (NEC).
Nicola Fazio, Lorenzo Gervaso, Thorvardur R Halfdanarson, Mohamad Sonbol, Rachel A Eiring, Sara Pusceddu, Natalie Prinzi, Benedetta Lombardi Stocchetti, Simona Grozinsky-Glasberg, David J Gross, Thomas Walter, Patrick Robelin, Catherine Lombard-Bohas, Samuele Frassoni, Vincenzo Bagnardi, Lorenzo Antonuzzo, Clotilde Sparano, Sara Massironi, Fabio Gelsomino, Alberto Bongiovanni, Nicoletta Ranallo, Salvatore Tafuto, Maura Rossi, Mauro Cives, Ibrahim Rasul Kakil, Hytam Hamid, Alessandra Chirco, Michela Squadroni, Anna La Salvia, Jorge Hernando, Johannes Hofland, Anna Koumarianou, Sabrina Boselli, Darina Tamayo, Cristina Mazzon, Manila Rubino, and Francesca Spada
We conducted a retrospective/prospective worldwide study on patients with neuroendocrine neoplasms (NENs) and a molecularly proven SARS-CoV-2 positivity. Preliminary results regarding 85 patients of the INTENSIVE study have been published in 2021. Now we are reporting the 2-year analysis.Here, we are reporting data from consecutive patients enrolled between 1 June 2020, and 31 May 2022. Among the 118 contacted centers, 25 were active to enroll and 19 actively recruiting at the time of data cut-off for a total of 280 patients enrolled. SARS-CoV-2 positivity occurred in 47.5% of patients in 2020, 35.1% in 2021, and 17.4% in 2022. The median age for COVID-19 diagnosis was 60 years. Well-differentiated tumors, non-functioning, metastatic stage, and gastroenteropancreatic (GEP) primary sites represented most of the NENs. COVID-19-related pneumonia occurred in 22.8% of the total, with 61.3% of them requiring hospitalization; 11 patients (3.9%) needed sub-intensive or intensive care unit therapies and 14 patients died (5%), in 11 cases (3.9%) directly related to COVID-19. Diabetes mellitus and age at COVID-19 diagnosis > 70 years were significantly associated with COVID-19 mortality, whereas thoracic primary site with COVID-19 morbidity. A significant decrease in both hospitalization and pneumonia occurred in 2022 vs 2020. In our largest series of NEN patients with COVID-19, the NEN population is similar to the general population of patients with NEN regardless of COVID-19. However, older age, non-GEP primary sites and diabetes mellitus should be carefully considered for increased COVID-19 morbidity and mortality. Relevant information could be derived by integrating our results with NENs patients included in other cancer patients with COVID-19 registries.