The incidence and prevalence of pancreatic neuroendocrine neoplasms are steadily increasing. These tumors are highly heterogeneous, with treatment options ranging from observation to surgery, and various medical therapies. The choice of treatment is influenced by factors such as tumor stage, grade (proliferative activity), and the presence of hormone-related syndromes. Endoscopic ultrasound (EUS) is becoming increasingly valuable for assessing pancreatic neuroendocrine neoplasms, offering detailed morphological, vascular, and functional information through techniques such as contrast enhancement and elastography. It also allows biopsies that are useful for both histopathological and molecular analyses. These tumors are highly heterogeneous, with treatment options ranging from observation to various medical therapies and surgery. Recent data suggest that small, non-functioning PanNENs with low proliferation rates may be safely monitored, whereas more aggressive or functioning tumors typically require surgery. EUS-guided ablation is a promising alternative for patients with functional pancreatic neuroendocrine neoplasms who are unsuitable for surgery, although randomized trials are needed. In non-resectable pancreatic neuroendocrine neoplasms, treatment options include somatostatin analogs, targeted therapies (e.g., everolimus, sunitinib), chemotherapy, and radioligand therapy. This review discusses key factors in planning personalized treatment strategies for pancreatic neuroendocrine neoplasms.
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http://dx.doi.org/10.1002/ueg2.12710 | DOI Listing |
Cureus
December 2024
Department of Hepatobiliary and Pancreatic Surgery, Pontificia Universidad Católica de Chile, Santiago, CHL.
Pancreatoduodenectomy and distal pancreatectomy are standard treatments for various pancreatic pathologies. These procedures involve radical resection and a significant loss of pancreatic tissue, which can lead to exocrine and/or endocrine pancreatic insufficiency. In selected cases of benign tumors or those with low malignant potential, central pancreatectomy can be performed with acceptable morbidity and mortality rates.
View Article and Find Full Text PDFCurr Oncol Rep
January 2025
Neuroendocrine Tumour Unit, ENETS Centre of Excellence, 1st Department of Propaedeutic and Internal Medicine, Laiko Hospital, National and Kapodistrian University of Athens, Agiou Thoma 17, Athens, 11527, Greece.
Purpose Of Review: The purpose of this review is to outline the current knowledge on epidemiology, diagnosis and management of neuroendocrine neoplasms (NENs) that develop in the context of Von Hippel-Lindau (VHL) syndrome.
Recent Findings: Pancreatic NENs develop in 8-17% of VHL patients (vPNENs) and are mostly multi-focal, cystic and non-functioning. Surgical resection is recommended for vPNENS > 3 cm that exhibit higher metastatic potential or in tumors with short doubling time while in the 20% of cases with metastatic disease the HIF-2 A inhibitor belzutifan is considered a promising option.
Endocr Pract
January 2025
Department of Endocrinology, Metabolism and Internal Diseases, Poznan University of Medical Sciences, 49 Przybyszewskiego Str., 60-355 Poznan, Poland.
Objectives: Neuroendocrine tumors (NETs) constitute a diverse group of tumors. NETs are often diagnosed late, due to nonspecific symptoms. Second Primary Malignancies (SPMs) have been reported in up to 25% of NETs and their incidence has been described as negative predictor of OS.
View Article and Find Full Text PDFBMJ Case Rep
January 2025
General Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India
Fibrolamellar hepatocellular carcinoma (FL-HCC) is a primary liver tumour presenting at a young age. Aggressive surgery of FL-HCC is the mainstay of management unlike other malignancies where metastatic stage precludes curative surgery. There are limited reports of response of FL-HCC to systemic therapies predominantly owing to its rarity.
View Article and Find Full Text PDFPathologica
October 2024
Pancreatic and Digestive Endocrine Surgical Research Group, Department of Surgery, Oncology and Gastroenterology, University of Padua, Padua, Italy.
An asymptomatic 79-year old woman presented with a 40 mm pancreatic cystic lesion, located in the pancreatic body-tail and consistent with branch-duct intraductal papillary mucinous neoplasm (BD-IPMN) without "high risk stigmata". During a 4-year follow-up period, imaging showed no mural nodules or main pancreatic duct dilation, and serum CEA and CA19.9 were within normal range.
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