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My ‘Spotlight’ on Carcinoid Syndrome may be easier reading for some but for those who wanted to dive into NET Specialist authored guidelines, this set from ENETS will be of use.

Scope.  Carcinoid Syndrome (CS) and Carcinoid Heart Disease (CHD) formally known as Hedinger Syndrome) are related in that the latter is a rare and complex cardiac complication occurring in patients with advanced NETs and CS.  CS is predominantly encountered in patients with well-differentiated NETs of intestinal origin, followed by lung NETs, and only in a minority of patients with pancreatic, ovarian, thymic, or unknown origin (UKO) NETs.  It follows that CHD only occurs in advanced NETs, when CS has been diagnosed, and there are normally elevated levels of 5-HIAA and NT-proBNP. 

Guidelines. This first ENETS guidance paper on CS aims to provide practical guidance for NET specialists in the diagnosis and management of CS, with special emphasis on CHD.

TABLE 1. Ten clinical questions for Carcinoid Syndrome (CS) and Carciniod Heart Disease (CHD) scenarios.

Q1: How should the clinician assess a patient with suspected CS and CHD at presentation, and during the follow-up?

Q2: What is the differential diagnosis of CS?

Q3: Which tools are needed to characterise severity of CHD?

Q4: Which are the principles/aims of treatment in a patient with CS?

Q5: How to manage CS?

Q6: What are the general recommendations for the initial management of CHD?

Q7: Do’s and don’ts in CS/severe CHD

Q8: What is the appropriate surgical management of CHD?

Q9: “Nonsurgical” interventional options for CHD

Q10: What are the advantages of MDT approach in improving the overall management of CS and CHD patients?

Now read the reference material below.

Reference

Click on the blue link below see read the discussion and the recommendations for each of those questions. 

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

 

First published: 25 April 2022

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