Hedinger syndrome Archives

26 Apr 2026

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Spotlight on Small intestine Neuroendocrine Neoplasms (siNENs)

A spotlight on NENs, A spotlight on NENs - Types, Patient AdvocacyCarcinoid heart disease, Carcinoid syndrome, Hedinger syndrome, Ileum, Jejunum, Mesenteric Fibrosis, Retroperitoneal Fibrosis, Small intestine NET (SI NET), Terminal ileum

DisclaimerThis Spotlight is for general education and reassurance only. It cannot replace personalised advice from your own medical team, who understand your individual history, imaging, pathology, and treatment needs. Neuroendocrine tumours vary widely in behaviour, presentation, and management, and guidance may evolve as new evidence emerges. If you have questions about your diagnosis, symptoms, or treatment plan, please discuss them directly with your specialist NET team. Small intestine neuroendocrine neoplasms (siNENs) - (Jejunum + ileum — excluding duodenum) Small intestine neuroendocrine neoplasms (siNENs) arise in the mid‑gut, specifically the ileum and jejunum. These two segments form the core of “small intestine…

01 Dec 2024

By Ronny Allan

23 Comments

Neuroendocrine Cancer – tumour markers and hormone levels

A Spotlight on NENs - Testing Series, Patient Advocacy131I-Metaiodobenzylguanidine (mIBG), 5-HIAA, 5-HT, 5-HTP, 5-Hydrotryptophan (5-HTP), 5-Hydroxyindoleacetic Acid (5-HIAA), 5-Hydroxytryptamine (5-HT), Adrenocorticotropic Hormone (ACTH), Biopsy, C-peptide, Calcitonin, Carcinoid crisis, Carcinoid heart disease, Carcinoid syndrome, Catecholamines, Chromaffin cells, Chromogranin, Chromogranin A (CgA), Chromogranin B (CgB), Chromogranin C (CgC), Cortisol, Electrolytes, Endocrine, Fasting Gut Endocrine Profile, Flushing, Gastric NET, Gastrin, Gastrin pH, Gastrinoma, Gastroesophageal reflux disease (GERD), Genetic testing, Glucagon, Glucagonoma, Hedinger syndrome, Histamine, Hormones, Hypertension, Insulin, Insulinoma, MEN1 syndrome, Metanephrines, Molecular markers, NETest, Neuroendocrine, Neuroendocrine cancer, Neuroendocrine carcinoma (NEC), Neuroendocrine tumour (NET), Neurokinin A (NKA), NKA, NT-proBNP, Pancreastatin, Pancreatic cancer, Pancreatic NET (PanNET), Pancreatic polypeptide (PP), Pheochromocytoma, Plasma 5HIAA, PPoma, Proinsulin, Proton pump inhibitors (PPI), Red blood count (RBC), Renal insufficiency, Serotonin, Serum serotonin, Somatostatin, Somatostatinoma, Substance K, Substance P, Tachycardia, Tachykinin, VIPoma, Wheezing, Zollinger-Ellison Syndrome

Originally published 2016, updated October 2025 Background Some people hear the terms "tumour markers" and "hormone levels" when they are diagnosed with Neuroendocrine Neoplasms (NENs). That might be confusing or even alarming at the time. Most people diagnosed with cancer 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. With NENs, 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…

01 Sep 2024

By Ronny Allan

20 Comments

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

TreatmentCarcinoid crisis, Carcinoid heart disease, Carcinoid syndrome, Carcinoid tumour, Diarrhea, Hedinger syndrome, Neuroendocrine tumour (NET), Quality of LIfe (QoL), Serotonin, Somatostatin analogues, Telotristat ethyl (Xermelo), TPH1, TPH2, Tryptophan hydroxylase (TPH), Xermelo (Telotristat ethyl)

Republished and reformatted and reviewed 1st September 2024. Original published 31st March 2016. 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…

14 Jun 2024

By Ronny Allan

16 Comments

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

A spotlight on NENs - Syndromes, Awareness5-HIAA, Achlorhydria, Adrenocorticotropic Hormone (ACTH), Alcohol, Angioma, Carcinoid crisis, Carcinoid heart disease, Carcinoid syndrome, Carcinoid tumour, Carney-Stratakis syndrome (CSS), Cerebellum, Cushing's disease, Cushing's syndrome, Dermatitis, Diet, Familial medullary thyroid cancer (FMTC), Fat malabsorption, Functional tumour, Gastrinoma, Glucagon, Glucagonoma, Haemangioblastoma, Hedinger syndrome, Hereditary pheochromocytoma/paraganglioma syndromes, Hypercalcemia, Hypercortisolism, Hypokalemia, Insulinoma, Medullary thyroid cancer (MTC), MEN1 syndrome, MEN2 syndrome, MEN2A syndrome, MEN2B syndrome, NET syndromes, Neuroendocrine cancer, Non-functional tumour, Paraganglioma, Paraneoplastic syndromes, Pheochromocytoma, Pituitary gland, PPoma, SDHA, SDHAF2, SDHB, SDHC, Somatostatin analogues, Somatostatinoma, Surgery, Sweet's syndrome, The 5 E's, Verner-Morrison syndrome, VIPoma, Von Hippel-Lindau (VHL) Syndrome, Von Recklinghausen's disease, Wermer syndrome, Whipple's triad

There are some curious things about Neuroendocrine Cancer (NETs elsewhere in the text on the basis that a hormonal syndrome is extremely rare in Neuroendocrine Carcinoma (NEC)). One of them 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. This frequently makes NET …

14 May 2024

By Ronny Allan

8 Comments

Neuroendocrine Cancer: Fibrosis – an unsolved mystery?

Living with Neuroendocrine Cancer, Treatment5-HT, Advanced neuroendocrine cancer, Carcinoid heart disease, Carcinoid syndrome, Carcinoid tumour, Dermal fibrosis, Desmoplasia, Hedinger syndrome, Hormones, Hydrocele, Hydronephrosis, Intestinal ischaemia, Mesentery, Neuroendocrine cancer, Neuroendocrine tumour (NET), Peritoneal carcinomatosis, Pleural Fibrosis, Pulmonary fibrosis, Retroperitoneal Fibrosis, Retroperitoneum, Scleroderma, Serotonin, SI NET, Small intestine NET (SI NET)

What happened to me? Since I was diagnosed in 2010, I've always known about a fibrosis issue in my retroperitoneal area. It was identified on the 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". Followed by a statement saying that it was "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…

05 May 2024

By Ronny Allan

27 Comments

Neuroendocrine Cancer – don’t break my heart!

Awareness, Living with Neuroendocrine Cancer, Survivorship, TreatmentCarcinoid heart disease, Carcinoid syndrome, ECG, Echocardiogram, Heart disease, Hedinger syndrome, Hormones, Neuroendocrine, Neuroendocrine cancer, NT-proBNP, Patent foramen ovale (PFO), Quality of LIfe (QoL), Serotonin, SI NET, Small intestine NET (SI NET), Somatostatin analogues, Surgery

Neuroendocrine Cancer has certain unique features whereby tumours can produce one or more symptoms which are known collectively as a syndrome. Some doctors may refer to this as a functional tumour. Neuroendocrine Tumours secreting excess amounts of serotonin sufficiently to develop a syndrome currently called Carcinoid Syndrome, which if not diagnosed and treated early enough, or the levels of serotonin ae not controlled following diagnosis, can lead to an additional complication known as Hedinger Syndrome (also known as Carcinoid Heart Disease (CHD)). However, late diagnoses can present with CHD already in place and in some cases, may have led to…

20 Apr 2024

By Ronny Allan

41 Comments

Carcinoid vs Neuroendocrine

Awareness, Patient AdvocacyAppendiceal NET, Benign tumour, Carcinoid, Carcinoid crisis, Carcinoid heart disease, Carcinoid syndrome, ENETS, Gastric NET, Gastro-entero-pancreatic neuroendocrine tumour (GEP-NET), Gastrointestinal NET (GI-NET), Hedinger syndrome, Karzinoide, Lung NETs, NET syndromes, Neuroendocrine cancer, Neuroendocrine carcinoma (NEC), Pathology, Pheochromocytoma, Rectal NET, Serotonin, Siegfried Oberndorfer, Small intestine NET (SI NET), World Health Organisation (WHO)

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. This post was originally generated in April 2015 (and hasn't changed that much!). EDIT MARCH 2024. There is now a consolidated Endocrine/Neuroendocrine Tumour classification system (Blue Book). Still in print but you can see a summary of changes by clicking here. Didn't provide clarity for all NENs. EDIT MARCH 2022. The latest classification system for Lung Neuroendocrine Neoplasms (NEN) confirms the word "carcinoid" is now a choice when…

20 Jan 2024

By Ronny Allan

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

A spotlight on NENs - Syndromes, Latest ENETS GuidelinesCarcinoid heart disease, Carcinoid syndrome, Hedinger syndrome

Author's commentMy '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…

20 Nov 2023

By Ronny Allan

0 Comments

Neuroendocrine Cancer – Clinical Esoterica

AwarenessCarcinoid crisis, Carcinoid syndrome, Desmoplasia, Gastrinoma, Glucagonoma, Hedinger syndrome, Hormones, Incurable cancer, Insulinoma, Neuroendocrine, Neuroendocrine cancer, Neuroendocrine tumour (NET), Paraganglioma, Pheochromocytoma, PPoma, Serotonin, Somatostatinoma, Thorson-Bioerck syndrome, Vasoactive intestinal peptide (VIP), Verner-Morrison syndrome, VIPoma, Whipple's triad, Zollinger-Ellison Syndrome

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…

28 Apr 2023

By Ronny Allan

2 Comments

A spotlight on 5-HIAA

A Spotlight on NENs - Testing Series, Patient Advocacy5-HIAA, 5-HT, Carcinoid heart disease, Carcinoid syndrome, Carcinoid tumour, Diagnosis, Functional tumour, Hedinger syndrome, Neuroendocrine cancer, Neuroendocrine tumour (NET), Plasma 5HIAA, Serotonin

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’…

30 Oct 2018

By Ronny Allan

9 Comments

Neuroendocrine Cancer Hormonal Syndromes – a witch’s brew

AwarenessCarcinoid crisis, Carcinoid syndrome, Desmoplasia, Gastrinoma, Glucagonoma, Hedinger syndrome, Hormones, Incurable cancer, Insulinoma, Neuroendocrine, Neuroendocrine cancer, Neuroendocrine tumour (NET), Paraganglioma, Pheochromocytoma, PPoma, Serotonin, Somatostatinoma, Thorson-Bioerck syndrome, Vasoactive intestinal peptide (VIP), Verner-Morrison syndrome, VIPoma, Whipple's triad, Zollinger-Ellison Syndrome

It’s a difficult disease to diagnose, e.g. many people struggle with undiagnosed syndromes for some time before formal diagnosis of Neuroendocrine Cancer. Many continue to struggle after. They hide in plain sight – sometimes the syndromes can provide clues ……… but they can still fool your doctors. Let's change that with more awareness which can lead to earlier diagnosis. 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…

Tag: Hedinger syndrome