Pancreatic Neuroendocrine Tumors: an Update

Abstract

Pancreatic neuroendocrine tumors (pNETs) are rare and comprise only 1-2 % of all pancreatic neoplastic disease. Although the majority of these tumors are sporadic (90 %), pNETs can arise in the setting of several different hereditary genetic syndromes, most commonly multiple endocrine neoplasia type 1 (MEN1). The presentation of pNETs varies widely, with over 60 % having malignant distant disease at the time of initial diagnosis involving the liver or other distant sites. Functioning pNETs represent approximately 10 % of all pNETs, secrete a variety of peptide hormones, and are responsible for several clinical syndromes caused by profound hormonal derangement. Surgery remains the cornerstone of therapy and the only curative approach. It should be pursued for localized disease and for metastatic lesions amenable to resection. Multimodality therapies, including liver-directed therapies and medical therapy, are gaining increasing favor in the treatment of advanced pNETs. Their utility is multifold and spans from ameliorating symptoms of hormonal excess (functional pNETs) to controlling the local and systemic disease burden (non-functional pNETs). The recent introduction of target molecular therapy has promising results especially for the treatment of progressive well-differentiated G1/G2 tumor. In this review, we summarize the current knowledge and give an update on recent advancements made in the therapeutic strategies for pNETs.

Malignant pancreatic tumor other than solid pseudopapillary tumor in pediatric patients

“…Approximately 10% of PNETs are functional, with insulinomas being the most common. [ 8 ] PNETs can also be classified as either well differentiated or poorly differentiated, with most being well differentiated and nonfunctional. The Ki-67 index of neuroendocrine patients reported in this study was low, indicating that the tumors were well differentiated.…”

“…The majority of PNETs are sporadic (90%) but can also be associated with genetic syndromes, such as multiple endocrine neoplasia type 1. [ 8 ]…”

“…PNETs are classified as either functional or nonfunctional and are mostly malignant depending on the production and secretion of pancreatic endocrine hormones. Approximately 10% of PNETs are functional, with insulinomas being the most common [8] . PNETs can also be classified as either well differentiated or poorly differentiated, with most being well differentiated and nonfunctional.…”

“…The Ki-67 index of neuroendocrine patients reported in this study was low, indicating that the tumors were well differentiated. The majority of PNETs are sporadic (90%) but can also be associated with genetic syndromes, such as multiple endocrine neoplasia type 1 [8] …”

Recent Advances in the Management of Endocrine Malignancies Associated with Hereditary Hyperparathyroidism Syndromes

“…are small, multifocal and microscopic [69], and larger pNETs tend to be associated with higher rates of lymph node and hepatic metastases [70][71][72][73][74]. Although many of the tumors associated with the MEN1 syndrome are benign, non functional pNET and thymic NET are two NETs in MEN1 with high malignant potential [75].…”