As featured by Neuroendocrine Cancer UK (formerly NET Patient Foundation)
It’s well known that Neuroendocrine Cancer can often be difficult to diagnose condition. However, what is less well known is the impact it has on those who are diagnosed. I’m one of the lucky ones, even though I still ended up with distant metastases. It does feel odd to say that having distant metastasis is lucky!
I consider my diagnosis to have been incidental as they were not investigating cancer – I suspect that’s the route for many cancer patients. I also think I was lucky because I had instant access to Neuroendocrine Cancer specialists and got quick treatment, and my follow up and support from a specialist centre were in place. I cope, but I wouldn’t say it’s easy living with metastatic and incurable cancer. As an advocate for a large online community, I do see many people struggle to get the same experience I had. I’m in no doubt that more must be done to help with earlier diagnosis, access to specialists and access to the right therapies at the right time. People must then be followed up with robust and expert-led surveillance and support regimes, and for the long term. This must become the normal experience; this must become the minimum standard of care.
This cancer is unlike others where the primary tumours are only located in a single organ. With Neuroendocrine disease, the primary tumour(s) can be found in many different organs and many different parts of the body. The aggressiveness of these tumours can range from indolent right up to very aggressive types. In aggressive types of cancer, stage is normally a key differentiator in prognostic terms, but with the less aggressive types of Neuroendocrine Cancers, often the grade can be more significant than the stage. There are a significant number of patients who have the lower grades but the highest stage and this is one of the huge challenges for physicians and patients to reconcile. An individual’s experiences will rely on their stage, grade, age, general physical condition and type of Neuroendocrine Neoplasm (a term encompassing well-differentiated Neuroendocrine Tumours and poorly differenced Neuroendocrine Carcinomas). Statistics indicate that early-stage diagnoses are on the increase, this is great news. But let’s be under no illusions, many people continue to be diagnosed late, often because of the relatively silent nature of the disease and often because of misdiagnosis and delay. More needs to be done to address both of these 2 issues.
Frequently, a correct diagnosis is just the beginning of a patient’s trauma. A late-stage diagnosis can still save a person’s life, but it can also present new challenges that will need specific attention to maintain quality of life. All these accumulative events and circumstances can create huge uncertainty, often with no signs of hope or a way out for the patient. Some of these events and circumstances will be sequential, but for many, they may appear simultaneously and within a relatively short period of time. For some, it can be like a “Perfect Storm” and even those who find themselves in the seemingly tranquil ‘eye’ of the storm, may still feel trapped and uncertain of what lies ahead. These ‘storm’ effects comprise:
- Diagnostic Difficulties – People often assume the symptoms they are experiencing are things they can treat themselves with over the counter drugs before finally seeking professional help. This could go on for some time, it’s a pretty deceiving disease. After several visits, and despite your concerns, your doctors may diagnose you with something else. In extreme cases they could become so frustrated that nothing serious is obvious, they might even start to think it’s all in your head. It’s even possible that some people will be referred to secondary care only be referred back to primary care still undiagnosed. The result of these delays is a diagnosis at a later stage and for some an additional diagnosis of an associated ‘syndrome’ in addition to cancer. Fortunately, this doesn’t happen all the time, thanks to improvements in awareness and the work of organisations such as Neuroendocrine Cancer UK and Specialist organisations.
- The Tumour Effect – Like many cancers, it can grow in your body without you knowing, without you having any reasons to seek intervention by healthcare professionals. It finds places to hide mainly the small intestine, appendix, lungs, stomach, pancreas, rectum and a host of other less common places. Once it has established in the primary location(s), it will try to break out via your blood and lymphatic systems, as cancer does. In indolent cases, it may not do this very fast but as it can be quiet, it will do this over several years. This cancer wants to establish other bases in your liver, your lymph nodes, your bones and any other place it can get to. If a person is lucky, their disease may be discovered incidentally while being investigated for something else, sometimes this is at an early stage but often it happens at a later stage. If a person is unlucky, the silence will continue until the tumours announce their presence via symptoms caused by growth, often in an emergency at a hospital and often leading to diagnosis at the later stages of cancer.
- The Syndrome Effect – As if that wasn’t bad enough, some of the sub-types of this cancer is known to be able to oversecrete hormones and peptides which can mimic routine illnesses and conditions including (but not limited to) Irritable Bowel Syndrome (IBS), asthma, general abdominal upset, diarrhoea, diabetes, rosacea, menopause and heart disease. Depending on the type of tumour, these present as common symptoms, some of which include flushing, diarrhoea, generally feeling weak and fatigued, pain, agitation, anxiety, dizziness, nausea, acid reflux, skin irritation, anaemic, weight loss/gain, low blood sugar, high blood sugar, heart palpitations, headaches, sweating, high blood pressure. So even when it becomes ‘noisy‘ and you seek professional help, the disease is so complex that it can often fool the host and the physicians into believing these are just routine illnesses needing routine approaches. Meanwhile, the tumours continue to grow and spread, often reaching an incurable state. There are several associated syndromes with Neuroendocrine Cancer, depending on the offending hormone(s) and in some cases the primary location(s). Many people focus on serotonin as the culprit hormone but there are other oversecreting hormones that are specifically associated with one of the Neuroendocrine Cancer syndromes and certain organs in the body, including (but not limited to) Insulin, Glucagon, Gastrin, Vasoactive Intestinal Peptide (VIP), Pancreatic Polypeptide (PP), Somatostatin, Adrenaline and Noradrenaline, Cortisol, Calcitonin, Adrenocorticotropic hormone (ATCH), Parathyroid hormone (PTH) and many more. The disease is also associated with hereditary syndromes which predispose a person to neuroendocrine tumours, these include 4 types of Multiple Endocrine Neoplasia (MEN), and familial versions of Medullary Thyroid Carcinoma, Pheochromocytoma and Paraganglioma. Other less common hereditary connections include Von Hippel-Lindau syndrome, neurofibromatosis type 1 and tuberous sclerosis complex.
- Post diagnosis discoveries – Even at diagnosis, things can suddenly get worse as specialist scanning, blood/urine testing and biopsies are conducted to discover the grade and stage of the patient’s disease. Further metastasis can be discovered via nuclear scans and for some, the primary location remains undiscovered. The most common syndrome associated with Neuroendocrine Cancer is currently known as ‘carcinoid syndrome’, predominantly caused by the over secretion of the hormone serotonin. Those who remained unaware of their disease or were diagnosed late, could now potentially find two further issues caused by high levels of this hormone. Firstly, a phenomenon known as desmoplasia which in the extreme results in a ‘fibrous mess’ in the mesentery and less commonly in the retroperitoneum. These issues were potentially involved in pre-diagnosis pain and discomfort in the abdominal area, at worst causing obstructions and damage to major organs and blood vessels, which may have led to the diagnosis. Secondly, a similar scenario involving serotonin can introduce fibrosis to the right side of your heart causing even more life-threatening issues to contend with on top of cancer. Other syndrome types are also capable of introducing significant disease morbidity.
- Post diagnosis side effects and long-term consequences – Diagnosis and initial treatment over, the patient’s and physician’s tasks are not over. A late-stage diagnosis means the disease is essentially incurable. While most will be ‘incurable but treatable‘, this does introduce additional challenges to the mix. Many of those in the lower stages and lower grades may need ongoing support and surveillance for a considerable period. Moreover, the side effects of surgery and other therapy types have the potential to introduce new treatment-induced side effects for many including (but not limited to); malabsorption, vitamin and mineral deficiencies, diarrhoea, diabetes, thyroid issues and many more. It may feel like the patient never recovers from the diagnosis with the patient unable to work out what a new normal is supposed to look like.
- Coping – The sheer amount of testing and surveillance required, the uncertainly about the prognostics, the consequences of the therapy, the worry about the impact on work, finances, family, (including any genetic worries) all have the potential to lead to anxiety and more serious mental illness issues in the long term, all add to this ‘Perfect Storm‘. Quite often these peripheral issues go unnoticed, untreated and essentially ignored. Fortunately, organisations such as Neuroendocrine Cancer UK are trying to ensure these issues are brought ‘front and centre’ via their ‘Mind the Gap’ campaign. However, it’s true to say we are only seeing the tip of the iceberg, there’s much more to do in this area.
Let’s be clear, Neuroendocrine Cancer actually wants to kill you. Without intervention, it will continue to plot its devious and destructive course, it will kill slowly but surely. Some are faster growing but they have the same traits, if left alone they just kill faster. Fortunately, due to earlier diagnosis, increasing awareness amongst the general public and healthcare professionals, and advances in medical science, the situation is improving slowly, but much more work needs to be done. Going forward, Neuroendocrine Cancer patients need quality of life markers in addition to tumour and hormone markers.
Awareness plays a critical part in finding these tumours earlier in order that treatment and support can begin. Share this post and potentially save a life.
Footnote to the article title:
A perfect storm is a rare combination of events or circumstances creating an unusually bad situation. The term is most well known as the title to the 1997 Sebastian Junger non-fiction book, The Perfect Storm which is about a fishing-boat crew encountering a confluence of several coincidental weather events at sea.
Ronny Allan – Short Biography
Ronny Allan was diagnosed with metastatic Neuroendocrine Cancer in 2010. He resides in Hampshire UK with his wife close to his own family and dotes on his four grandsons. He enjoys family life, travel, reading, walking cycling and watching football. Ronny blogs about cancer and his survivorship experience via his website RonnyAllan.NET . The blog has been featured by several international organisations including Cancer Knowledge Network (CKN), Cure Magazine, Macmillan Cancer Support, WEGO Health, NET Research Foundation and Neuroendocrine Cancer UK. He is also a strong global advocate for Neuroendocrine Cancer awareness via his associated Facebook and Twitter sites.
He was the 2016 Winner in Best in Show Community, 2018 Winner Best in Show Blog, and Lifetime achievement in 2021, all in the WEGO Health Awards. In total so far, he’s had over 20 nominations for these awards proceeding to the finals on 6 occasions. He’s currently a member of the Strategic Advisory Board at Multimed Inc (publishers of Cancer Knowledge Network and Current Oncology), the Ipsen Patient Advisory Board and in 2019 and 2022 tenures, the WEGO Health Patient Leader Advisory Board. In June 2019, his blog broke through the 1 million views mark and then 2 million in October 2022.
Ronny is a regular speaker telling his patient story and other contributions to Neuroendocrine Cancer, including to audiences in Europe and North America. Ronny is passionate about modernising Neuroendocrine Cancer awareness and taking it to new audiences. He’s also a big believer in the patients included concept and his sites are accredited with Patient Included status.
Featured by Neuroendocrine Cancer UK (formerly NET Patient Foundation) here.
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9 thoughts on “Neuroendocrine Cancer: The Perfect Storm”
great article i was diagnosed with nueroencrodine cancer of the cervix mixed with adenocarcinoma dec 2017 have been NED for 2 yearsbut live in fear everyday of it returning. Thanks for help spreading the word
Great article Ronny, the heading sums it up in nutshell, and a good one to share when being asked what it’s like to live with this. As we all know trying to explain the details and how if affects us is no easy task.
Also, it was good to finally meet you the other week too😀
Yes, always good to meet up offline!
I was diagnosed last year, following a blockage in the small bowel, I am feeling well and seem to spend all my time at the hospital or planning around my monthly injections. You have given me so much hope and it’s is interesting to read your article as a sufferer. My husband and I try to lead our life to the full, travelling and going out etc. Giving us both a quality to our lives. Thankyou for writing about your Perfect Storm
thanks Lorraine, stay well
Ronny and others – I was diagnosed with pNET in 2005. Long story; sometimes it feels like a short time. I still hear from every clinic and oncologist and specialist I see, the words “quality of life”. You use it freely as well. For all these years, I have yet to be able to find a personal definition for me. Seems basically indescribable to me. Just intersted in what you and others might say or how they define the “quality of life” dilemna.
Probably a personalised thing, maybe even self assessable/definable in some cases. In the most practical terms, it’s a series of checks done on patients alongside routine scans and bloods etc. There’s work going on to define a set of checks for NETs. Defining them is the easy part, getting centres to implement them is more difficult (£££).
A fantastic article Ronny!! Thank you.