Abstract
Simple SummaryPancreatic neuroendocrine tumors (pNET) are a heterogeneous and challenging entity, and today’s guidelines offer a variety of treatment modalities, while surgery has a clear role for patients with resectable tumors and early stages, advanced, or metastatic pNET may benefit from treatments that were evaluated in randomized controlled studies during the last year. With this review, we aim to provide an updated view on treatment options for metastatic pNET.Pancreatic neuroendocrine tumors (pNETs) are a vast growing disease. Over 50% of these tumors are recognized at advanced stages with lymph node, liver, or distant metastasis. An ongoing controversy is the role of surgery in the metastatic setting as dedicated systemic treatments have emerged recently and shown benefits in randomized trials. Today, liver surgery is an option for advanced pNETs if the tumor has a favorable prognosis, reflected by a low to moderate proliferation index (G1 and G2). Surgery in this well-selected population may prolong progression-free and overall survival. Optimal selection of a treatment plan for an individual patient should be considered in a multidisciplinary tumor board. However, while current guidelines offer a variety of modalities, there is so far only a limited focus on the right timing. Available data is based on small case series or retrospective analyses. The focus of this review is to highlight the right time-point for surgery in the setting of the multimodal treatment of an advanced pancreatic neuroendocrine tumor.
Highlights
Neuroendocrine tumors (NET) are a heterogeneous group of tumors with primary origin often located in the gastro-entero-pancreatic (GEP) tract
We address the question of the timing of modalities in the setting of advanced or metastatic Pancreatic neuroendocrine tumors (pNETs) treatment in the European
Chromogranin A (CgA), Synaptophysin and neuron-specific enolase (NSE) are widely used in clinical routines as diagnostic tumor markers. Since both CgA and NSE have a wide range in sensitivity (CgA 43–100%; NSE between 33% and 59%), as well in specificity (CgA 10–96%; NSE up to 80%) [22,23], further markers are currently investigated in clinical routines [24–26]
Summary
Neuroendocrine tumors (NET) are a heterogeneous group of tumors with primary origin often located in the gastro-entero-pancreatic (GEP) tract. The incidence has increased significantly in recent years, these tumors are still considered a rare entity. Pancreatic neuroendocrine tumors (pNET) show the worst prognosis at any stage or grade compared to midgut NETs [1,7], if p-NETs represent liver metastasis [8]. More than 60% of pNETs present in the advanced or metastatic stage at first diagnosis [9]. Over 40% of pNET become metastatic in the course of the disease, commonly by lymph nodes or liver metastasis [10], and patients with untreated liver metastasis show a 5-year survival rate of between 20% to 40% [11,12]. Compared to gastrointestinal NET, pNET rarely presents with classical neuroendocrine symptoms [13]. The initial presentation of pNET is unspecific unless the tumor is hormonally active or the patient represents extensive symptomatic liver metastasis
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