European Neuroendocrine Tumor Society (ENETS) 2022 Guidance Paper for Carcinoid Syndrome and Carcinoid Heart Disease

Abstract

Data regarding the diagnosis, management, and follow-up of carcinoid syndrome (CS) and carcinoid heart disease (CHD) were identified by searches of the MEDLINE database using specific terms in human studies: CS; CHD; screening; epidemiology; diagnosis; treatment; prognosis. The search results were supplemented by manual searching of relevant journals, reference lists in key articles and other appropriate documents, and expert opinion. All recommendations are offered on the basis of the best available evidence, supplemented by the authors' experiences in managing CS and CHD. Each recommendation for treatment will have a level of evidence and grade of recommendation as per the GRADE system (adapted in Infectious Disease society This first ENETS guidance paper on CS aims to provide practical guidance for NEN specialists in the diagnosis and management of CS, with special emphasis on CHD.

PRESTO 2: An International Survey to Evaluate Patients’ Injection Experiences with the Latest Devices/Formulations of Long-Acting Somatostatin Analog Therapies for Neuroendocrine Tumors or Acromegaly

“…Acromegaly is usually caused by excess production of growth hormone as a result of a benign tumor (adenoma) in the pituitary gland [ 3 – 5 ]. NETs are a heterogeneous group of slow-growing neoplasms that occur in diffuse neuroendocrine cells and that are accompanied, in some patients, by carcinoid syndrome [ 6 , 7 ]. There are currently two proprietary (branded) injectable LA SSAs available for first-line treatment of NETs and acromegaly: Somatuline® (lanreotide) Autogel/Depot (Ipsen)—herein referred to as LAN—and Sandostatin® (octreotide) Long-Acting Release (Novartis)—herein referred to as OCT. LAN is provided as a ready-to-use prefilled syringe for administration by deep subcutaneous injection once every 28 days [ 8 , 9 ].…”

Circulating Neuroendocrine Tumor Biomarkers: Past, Present and Future

“…Additionally, in patients diagnosed with small intestine NEN, bronchopulmonary NEN or when suspecting carcinoid syndrome, it is recommended to measure 5-HIAA in urine (at least two samples, collected over 24 h period each) [ 8 , 161 , 162 ]. Though not a NET biomarker sensu stricto, the N-terminal prohormone of brain natriuretic peptide (NT-proBNP) should be measured for the diagnosis and monitoring of carcinoid heart disease in carcinoid syndrome patients [ 163 ]. In patients presenting with symptoms characteristic of functional NETs, we recommend measuring the hormones linked with the suspected syndrome (as shown on Table 2 ).…”

A Clinical Guide to Peptide Receptor Radionuclide Therapy with 177Lu-DOTATATE in Neuroendocrine Tumor Patients

“…In a systematic review, symptomatic improvement after PRRT was observed in 74% of patients with diarrhea and in 6% of patients with flushing [ 43 ]. Together, these results have positioned PRRT with 177 Lu-DOTATATE as a viable option for refractory CS [ 44 ]. In addition, case reports have shown improvement of symptoms and echocardiographic parameters of carcinoid heart disease after PRRT, which is a severe symptom of CS [ 45 ].…”