Hedinger syndrome - Ronny Allan - Living with Neuroendocrine Cancer

28Apr 2023 by Ronny Allan 2 Comments

A spotlight on 5-HIAA

A Spotlight on NENs - Testing Series, Patient Advocacy

Background. It's important to note that not every type of Neuroendocrine Neoplasm will get the same tests due to the heterogenous nature of this cancer type. Some are more specific than others. A wide range of tests may be necessary at diagnosis if the type of NET is not clear. This post will cover one of the main tests to check for a common type of Neuroendocrine Tumour (NET) that is known to secrete excess amounts of the hormone Serotonin leading to symptoms indicative of Carcinoid Syndrome. These tumours are often labelled using the antiquated and misnomer term ‘Carcinoid Tumour’ but more and more healthcare organisations and specialists are avoiding use of this term for several reasons. Mainly because the word has been 99% removed from the widely accepted World Health Organisation…

30Oct 2018 by Ronny Allan 9 Comments

Neuroendocrine Cancer Hormonal Syndromes – a witch’s brew

Awareness

One of the key awareness messages for Neuroendocrine Cancer is the hormonal syndromes that can often accompany the diagnosis for many people. As it's a difficult disease to diagnose, many people struggle with these syndromes for some time before formal diagnosis of Neuroendocrine Cancer. Some continue to struggle after. Neuroendocrine Cancer can often be uncannily quiet, but the tumours can be 'functional' and over-secrete certain hormones to add or introduce symptoms that mimic many other diseases or conditions, such as Irritable Bowel Syndrome, Menopause, Heart disease and Asthma. In addition to common symptoms of flushing and diarrhea, others include generally feeling weak, fatigued, pain, agitated, anxious, dizzy, nauseous, acid reflux, skin irritation, anaemic, weight loss, weight gain, low blood sugar, high blood sugar, heart palpitations, headaches, sweating, high blood…

17Jul 2018 by Ronny Allan 8 Comments

Neuroendocrine Cancer: Fibrosis – an unsolved mystery?

Living with Neuroendocrine Cancer, Treatment

What happened to me?Since I was diagnosed in 2010, I've always known I've had a fibrosis issue in the retroperitoneal area, as it was actually identified on my very first CT scan, which triggered my diagnosis. Here's how the radiologist described it - "There is a rind of abnormal tissue surrounding the aorta extending distally from below the renal vessels. This measures up to 15mm in thickness". He went on to describe that "almost certainly malignant". The second and third scans would go on to describe as "retroperitoneal fibrosis" and "a plaque-like substance". Interestingly the fibrosis itself does not appear to 'light up' on nuclear scans indicating it was not cancerous (see below).I really didn't know what to make of this issue at diagnosis, although I did know the aorta…

28Sep 2016 by Ronny Allan 23 Comments

Neuroendocrine Cancer – tumour markers and hormone levels

A Spotlight on NENs - Testing Series, Patient Advocacy

BackgroundSome people on hear the terms "tumour markers" and "hormone levels" when they are diagnosed with Neuroendocrine Cancer. Most of them will have had a form of medical testing at some point in their life, i.e. the sampling and testing of blood, urine, saliva, stool or body tissue. In a nutshell, the medical staff are just measuring the content of a 'substance' and then taking a view whether this is normal or not based on pre-determined ranges. These tests are normally done as a physician's reaction to symptom presentation or maintenance/surveillance of an existing diagnosed condition. Sometimes, abnormal results will lead to more specialist tests.In cancer, these tests are frequently called 'markers'. Most tumour markers are made by normal cells as well as by cancer cells; however, they are produced at…

31Mar 2016 by Ronny Allan 20 Comments

Telotristat Ethyl (XERMELO®) – an oral treatment for Carcinoid Syndrome Diarrhea not adequately controlled by Somatostatin Analogues

Treatment

What is Telotristat Ethyl?Telotristat Ethyl is a significant introduction to the treatment of Carcinoid Syndrome diarrhea. It's the first addition to the standard of care in more than 16 years and the first time an oral syndrome treatment has been developed. The drug was previously known as Telotristat Etiprate but was changed to Ethyl in Oct 2016. 'Etiprate' was previously a truncation of 'ethyl hippurate'. The brand name is XERMELO® Who is the drug for?The drug may be of benefit to those whose carcinoid syndrome diarrhea is not adequately controlled by somatostatin analogues (Octreotide/Lanreotide). It doesn't replace somatostatin analogues - it is an additional treatment alongside.Where is it currently approved?The US FDA approved the drug 28 February 2017.On 19 September 2017,the European Commission approved Xermelo® (telotristat ethyl) for the treatment of carcinoid syndrome diarrhea in patients…

10Aug 2015 by Ronny Allan 16 Comments

The Syndromes of Neuroendocrine Cancer – Early Signs of a Late Diagnosis

Awareness

The Syndromes of Neuroendocrine Cancer - Early Signs of a Late Diagnosis One of the curious things about Neuroendocrine Cancer (NETs elsewhere in the text) is that it can very often exhibit one or more vague symptoms collectively known as a 'syndrome'. Syndrome is an apt word to describe these complications as the most general meaning in medical terms is a group of symptoms that together are characteristic of a specific disorder or disease". Having a syndrome can often be the difference between having a 'functional' condition or a non-functional' condition - see more below. This frequently makes Neuroendocrine Cancer very difficult to diagnose quickly. It's a very devious disease. It's NOT all about Carcinoid Syndrome! Most people think of Carcinoid Syndrome when they discuss NETs. Anyone suggesting that all…

02Jun 2015 by Ronny Allan 25 Comments

Neuroendocrine Cancer – don’t break my heart!

Awareness, Living with Neuroendocrine Cancer, Survivorship, Treatment

Neuroendocrine Cancer has certain unique features whereby tumours can produce one or more symptoms which are known collectively as a syndrome. Neuroendocrine Tumours secreting excess amounts of serotonin, can be accompanied by Carcinoid Syndrome which if not diagnosed and treated early enough, can lead to an additional complication known as Hedinger Syndrome (often known as Carcinoid Heart Disease (CHD)). However, very late diagnoses can present with CHD already in place.Excess serotonin, a hormone released by NETs into the bloodstream seems to be the prime and lead suspect for causing thick ‘plaques’ or fibrosis tissue within the heart muscle and damage to (mainly) the tricuspid and pulmonary valves on the right side of the heart which can become ‘tightly narrowed’ or ‘leaky’. It's very similar to the reasons for mesenteric and peritoneal fibrosis…

22Apr 2015 by Ronny Allan 41 Comments

Carcinoid vs Neuroendocrine

Awareness, Patient Advocacy

OPINION There's a constant debate regarding the validity of the term 'Carcinoid'. I've posted about this a few times and as far as I know, the debate has been raging for some years. EDIT MARCH 2022. The latest classification system for Lung Neuroendocrine Neoplasms (NEN) confirms the word "carcinoid" is now a choice - the WHO Lung Committee bottled it. I made my choice some years ago, I hope others follow suit. Read more about changes to Lung NEN by clicking here. EDIT APRIL 2020. The latest classification system for Neuroendocrine Neoplasms confirms the word "carcinoid" no longer forms part of the terminology used in Digestive System tumours (effectively removing the term from GEP NETs) - read more - click here Edit May 2020. So, what about other areas not included in…

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