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.
The cancer can often be uncannily quiet, but the tumours can be ‘functional’ and over-secrete certain hormones to add or introduce symptoms which 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, lose weight, gain weight, low blood sugar, high blood sugar, heart palpitations, headaches, sweating, high blood pressure. Its main trick is to prevent you from being correctly diagnosed and it’s pretty good at it. For those looking for a diagnosis, it can be very frightening.
One or more of the NET syndromes can be a weird concoction of strange, powerful or terrifying ingredients, designed to make you very ill; and doctors will be confused.
So called Carcinoid Syndrome
Certain types of Neuroendocrine Cancer were once referred to by the out of date term of ‘Carcinoid‘ – now correctly referred to as a NET prefixed by its anatomical primary location. However, for the time being, the term Carcinoid Syndrome which can be associated with these types of NET persists. The classic carcinoid syndrome includes flushing (80%), diarrhea (70%), abdominal pain (40%), valvular heart disease (20% since the introduction of somatostatin analogues), telangiectasia (25%), wheezing (15%), and pellagra-like skin lesions (5%).
Carcinoid syndrome, first described in 1954 by Thorson and co-workers, has the following features: malignant neuroendocrine tumour of the small intestine, normally with metastases to the liver, sometimes with valvular disease of the right side of the heart (pulmonary stenosis and tricuspid insufficiency without septal defects), peripheral vasomotor symptoms, bronchial constriction, and an unusual type of cyanosis. One year later, Dr. William Bean gave the following colorful description of carcinoid syndrome:
“This witch’s brew of unlikely signs and symptoms, intriguing to the most fastidious connoisseur of clinical esoterica—the skin underwent rapid and extreme changes resembling in clinical miniature the fecal phantasmagoria of the aurora borealis.”
Certain Neuroendocrine Tumours are known to be capable of over secreting (amongst others) the vasoactive substance called serotonin – mainly midgut. It’s commonly thought that serotonin is the cause of the flushing, but this is only partially correct, the flushing also results from secretion of kallikrein, the enzyme that catalyzes a conversion to bradykinin, one of the most powerful vasodilators known.
A very common component of carcinoid syndrome is diarrhea, probably caused by the increased serotonin, which greatly increases peristalsis, leaving less time for fluid absorption. In the extreme this syndrome can cause a pellagra-like condition, probably due to the diversion of large amounts of tryptophan from synthesis of the vitamin B3 (Niacin), which is needed for NAD production (oxidized form of B3).
Serotonin is also thought to cause fibrotic lesions of the endocardium (Hedinger Syndrome), particularly on the right side of the heart resulting in insufficiency of the tricuspid valve and, less frequently, the pulmonary valve and, uncommonly, bronchoconstriction. Other fibrosis issues arising from excess secretion of serotonin include mesenteric and retroperitoneal desmoplasia which have the potential to dangerously obstruct important vessels and cause general discomfort at best.
Carcinoid Syndrome is one of the most powerful and dangerous ‘witch’s brews’.
But it’s not all about (so called) carcinoid syndrome
Other witch’s brew flavours include the group of NET syndromes associated with pancreatic NETs with over-secretions of Insulin, Glucagon, Gastrin, Vasoactive Intestinal Peptide (VIP), Pancreatic Polypeptide (PP) and Somatostatin. Read more about these and other syndromes here.
It gets everywhere ……
One of the most scary witch’s brews is the group of symptoms associated with one of the most uncommon types of NET, the catecholamine and metanephrine (adrenaline and noradrenaline) secreting tumours known as Pheochromocytoma and Paraganglioma. These tumours are likely to cause a barrage of symptoms such as High blood pressure, Heavy sweating, Headache, Rapid heartbeat (tachycardia), Tremors, Paleness in the face (pallor) and Shortness of breath (dyspnea).
All of the above is a diagnostic nightmare for those who have the symptoms and remain undiagnosed – no fun for the doctors either – this why we need so much more awareness and education – it’s one of the key aims of all my social media sites. Another aim of my sites is to support those who are diagnosed as these symptoms can continue following diagnosis and treatment. Many NET patients need constant surveillance and follow-up, many for life. Neuroendocrine Cancer can be like the ‘Perfect Storm’ and definitely cause trouble (double toil and trouble).
This is a very spooky disease, it will slowly grow without you knowing, it will mess with your body and mind, and if left alone to plot its devious and destructive course, it will kill. Some are faster growing but they have the same traits – they just kill faster.
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Ronny Allan: Living with Neuroendocrine Cancer during COVID-19 restrictions 10 – A story of the Wall, Swans, Dundee and New Forest History
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Living with Neuroendocrine Cancer during COVID-19 restrictions 11 – a story of Walking the Wall, coping strategies and the London Ga68 PET
Episode 11 of my Living under COVID-19 series. A story of the Walking the Wall, coping strategies and the London Ga68 PET 30th May 2020