ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Neoplasms: Systemic Therapy - Chemotherapy

Garcia-Carbonero, Rocío; Rinke, Anja; Valle, Juan W; Fazio, Nicola; Caplin, Martyn; Gorbounova, Vera; O’Connor, Juan Manuel; Eriksson, Barbro; Sørbye, Halfdan; Kulke, Matthew H.; Chen, Jie; Falkerby, Jenny; Costa, Frederico; Herder, Wouter W. de; Lombard‐Bohas, Catherine; Pavel, Marianne

doi:10.1159/000473892

Cited by 102 publications

(109 citation statements)

References 56 publications

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“…The available data on outcomes from palliative chemotherapy are from retrospective data. Most guidelines advocate the use of platinumbased chemotherapy combined with etoposide as firstline palliative chemotherapy [6,49,52,53]. The optimal duration of such treatment has not been established.…”

Section: Palliative Chemotherapymentioning

confidence: 99%

Unmet Needs in High-Grade Gastroenteropancreatic Neuroendocrine Neoplasms (WHO G3)

et al. 2018

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Gastroenteropancreatic (GEP) neuroendocrine neoplasms (NEN) are classified based on morphology and graded based on their proliferation rate as either well-differentiated low-grade (G1 to G2) neuroendocrine tumors (NET) or poorly differentiated high-grade (G3) neuroendocrine carcinomas (NEC). Recently, a new subgroup of well-differentiated high-grade pancreatic tumors (NET G3) has been defined. The GEP NEN G3 group consisting of both NEC and NET G3 has recently been shown to be a quite heterogeneous patient group concerning prognosis and treatment benefit, depending on factors such as the primary tumor site, differentiation, proliferation rate, and molecular alterations. In this review we discuss the existing data on diagnostics, treatment, and biomarkers in this patient group, the unmet needs, and the future perspectives.

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Section: Palliative Chemotherapymentioning

confidence: 99%

Unmet Needs in High-Grade Gastroenteropancreatic Neuroendocrine Neoplasms (WHO G3)

et al. 2018

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“…Other novel targeted agents evaluated in phase 2 trials (e.g., bevacizumab, axitinib, or pazopanib) remain investigational (pending further validation in phase 3 studies and subsequent licensing) and will not be covered by these guidelines. SOC guidelines for systemic chemotherapy will be covered in a separate chapter [2]. …”

Section: Introductionmentioning

confidence: 99%

ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Neoplasms: Systemic Therapy - Biotherapy and Novel Targeted Agents

et al. 2017

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Systemic therapies established in the management of patients with neuroendocrine tumors (NETs) include somatostatin analogs and interferon-α, also referred to as biotherapy. Recent randomized controlled studies have extended the knowledge on the frequency of side effects associated with biotherapy. More recently, novel targeted drugs, such as the mammalian target of rapamycin inhibitor everolimus and the multiple tyrosine kinase inhibitor sunitinib, have been introduced in the management of NETs. Although targeted drugs are generally well tolerated, with most adverse events being of mild to moderate severity and manageable, novel targeted drugs exhibit a distinct adverse event profile that warrants guidance for appropriate diagnostic and therapeutic management. This is particularly important given the widespread and potentially long-term use of everolimus in a broad spectrum of NETs and of sunitinib in pancreatic NETs. This review will focus on the most relevant toxicities associated with biotherapy and novel targeted drugs and on their management. For each drug class indication, administration and dosing schedule, most frequent adverse events, actions and dose adjustments for adverse events as well as their monitoring are presented. This review further covers the evaluation of treatment effect, patient information, drug interactions, and information on pregnancy.

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“…According to the WHO classification 2010, a poorly differentiated neuroendocrine neoplasm with a Ki-67 index >20% is defined as a neuroendocrine carcinoma (NEC) and hence of grade G3 [2-6]. Increasing evidence suggests that G3 neuroendocrine neoplasms are not a homogeneous entity and can be further subclassified into biologically relevant subgroups [7]. …”

Section: Introductionmentioning

confidence: 99%

Extremely Long Survival under Combined Immunotherapy in a Metastatic Functional Neuroendocrine Neoplasia Patient

Schmidt

Wiedenmann

2017

Neuroendocrinology

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Treatment and prognosis of neuroendocrine neoplasia depends on tumor size, stage, grade, resectability, and extent of distant metastasis. In most cases a multimodality approach including surgical, locally invasive procedures, peptide-guided radioreceptor therapy (PRRT), and medical therapies represent the mainstay of treatment in advanced disease. In the reported case, a 68-year-old man was diagnosed in 2010 with an initially functional (histamine) neuroendocrine tumor of gastric type III, G2, stage IVB, cT4cN1cM1 (hepatic, peritoneal, nodal, osseous), including a hepatic tumor load of 25%. Intensive multimodality approaches including combined immunotherapy (vaccination and PD-1/CTLA-4 blockade) led to a survival of 8 years until now with a high quality of life and minimal residual disease (only a single, small paragastric recurrence) despite the dedifferentiation of the tumor into a neuroendocrine carcinoma G3 (Ki-67 of 80%) including a nonfunctional stage.

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ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Neoplasms: Systemic Therapy - Chemotherapy

Cited by 102 publications

References 56 publications

Unmet Needs in High-Grade Gastroenteropancreatic Neuroendocrine Neoplasms (WHO G3)

Unmet Needs in High-Grade Gastroenteropancreatic Neuroendocrine Neoplasms (WHO G3)

ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Neoplasms: Systemic Therapy - Biotherapy and Novel Targeted Agents

Extremely Long Survival under Combined Immunotherapy in a Metastatic Functional Neuroendocrine Neoplasia Patient

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