An interactive, practical toolkit for all those living with Neuroendocrine Cancer
PATIENT HANDBOOK
An interactive, practical toolkit
for all those living with Neuroendocrine Cancer
©️ Neuroendocrine Cancer UK 2023 Charity Registration Number 1092386
01. • What is Neuroendocrine Cancer? • How are Neuroendocrine Cancers formed? • The difference between normal cells and cancer cells - Explained • Is Neuroendocrine Cancer genetic? • Does primary site matter? • Staging and Grading (including differentiation) • What is meant by the terms “Functioning” and “Non-Functioning”? • What is meant by the terms ‘metastatic’ or ‘secondary cancer’? • Mythbusters - Section 01 UNDERSTANDING NEUROENDOCRINE CANCER 02. • Signs & Symptoms of Neuroendocrine Cancer - Before & After Diagnosis • The physical presence of a cancer - it’s size, site and position • Pain • Mythbusters: Pain • Functional signs, symptoms and syndromes • Carcinoid Syndrome - Explained • Syndromes explained • Complications or Consequences of Neuroendocrine Cancer SYMPTOMS OF NEUROENDOCRINE CANCER
• Carcinoid Heart Disease - Explained • Carcinoid Crisis Disease - Explained • Paraneoplastic Syndromes - Explained
03.
TESTS & SCANS
• Introduction to Section 3 - Tests & Scans • Shared Decision Making and Consent • Medical Consent - Explained • Blood and Urine Tests • Getting your results - Explained • Advances in blood tests • Tests using Scopes • Scans • Histopathology • Your Appointment • Cancer Screening Programmes
04.
SPECIALIST CARE & SUPPORT
• Introduction to Section 4 - Specialist Care & Support • Your rights as a patient • So WHO are the right people? • The importance of a multidisciplinary team (MDT) approach • Specialist Neuroendocrine Cancer Multidisciplinary Team Meetings (MDMs) • Specialist services, where are the right people and
when should I see them? • Support available to you • Referral Pathway • The importance of a specialist care
05. TREATMENT DECISION MAKING & PREPARATION • Treatment Decision Making & Preparation • Treatment decision-making the role of the MultiDisciplinary Team (MDT) • Key influences in MDT treatment decision making • Questions the team may discuss in the meeting, 06. TREATMENTS FOR NEUROENDOCRINE CANCER when reviewing results available for you • What happens after an MDT meeting? • FAQs - Treatment Decision-Making • Genetics, Genomics, Precision Medicine and Personalised Care • FAQs - Genetics & Genomics • Side effects & potential consequences of treatment • Treatments available for Neuroendocrine Cancer • Enhanced Supportive Care • Surgical Approaches • Endoscopic Therapies • Somatostain Analogues • Chemotherapy & Targeted Molecular Medical Treatments • Radiation Based Treatment inc. Targeted Molecular Radiation Therapies • Interventional Radiology • Clinical Trials • Considerations & Preparing for Treatment • Questions you may have • Reducing risks
07.
FOLLOW UP CARE
• Follow Up Care - Introduction • Examples of Follow Up Care • Expert Clinical Guidelines and where to find them • Points of contact roles • What can help to ensure you have a point of contact throughout your follow on care
08.
LIVING WITH NEUROENOCRINE CANCER
• Introduction to Section 8 Living with Neuroendocrine Cancer • A “Roller-Coaster of Emotions and Feelings” • Finding ways to cope • Relationships: Family and Friends • Children • For Family & Friends • Work & Neuroendocrine Cancer • Finances & Neuroendocrine Cancer • Driving • Travel & Neuroendocrine Cancer • The Future • Progressive disease & End of Life Care Planning • End of Life Care • Practical Considerations • This is my husband, Andrew Cheetham In memory of Andrew, written by his wife, Faye
09.
PRINTABLES
A QUICK GUIDE This Virtual Patient Handbook is designed for those living with neuroendocrine cancer and their loved ones. As a way of presenting large amounts of information in an engaging and interactive way, we have designed the Handbook to include a mixture of text and audio-visual content in the hopes that it may be more accessible to a range of learning styles within our community. Each section of the Handbook focuses on a unique part of living with neuroendocrine cancer, from understanding what it is, to symptoms, tests and treatments, specialist care and guidance for living with it. The Handbook is designed to be experienced in tandem with the Neuroendocrine Cancer UK website so you will find links throughout this book which direct you to additional resources online. Within this Handbook you will also encounter a range of digital resources. As a quick reference, they will each have an identifying logo / 'key' which is shown next to their descriptions overleaf. **If viewing on a mobile device, the Handbook is best viewed with your phone in landscape mode so you can see the double-page spread. You can also zoom in where necessary and links will highlight**
VIDEO SUMMARY
These short videos summarise the contents of longer, in depth informational videos that are located on the Neuroendocrine Cancer UK YouTube page. Below each video you will find the relevant link to visit the full video
PODCAST SUMMARY
These short videos summarise the contents of longer, in depth, informational podcasts. You will find a link to the podcast page on the Neuroendocrine Cancer UK website below each video
EXPLAINER VIDEOS
These informational short videos consist of narrated visual content on a range of subjects
For further information and resources LINK TO NCUK WEBSITE
Link to a relevant piece of research SPOTLIGHT ON RESEARCH
NEWS FEATURE
Link to a relevant news item on the Neuroendocrine Cancer UK website
LINK TO EXTERNAL WEBSITE
Link to a relevant report or information hosted on an external website (not Neuroendocrine Cancer UK)
HELLO & WELC
Welcome to the Neuroendocrine Cancer UK Patient Handbook, an interactive, practical toolkit for all those living with and affected by, neuroendocrine cancer. A diagnosis of cancer is one of the greatest challenges anyone can face. Being diagnosed with a rare or uncommon cancer, such as neuroendocrine cancer, can have additional consequences, not least in terms of awareness, early diagnosis and access to expertise. Feelings of isolation, fear, uncertainty and anxiety are not uncommon - especially when you are newly diagnosed.This toolkit has been written to provide you with information you may find helpful. We would like to take this opportunity to thank Faye Cheetham for her incredible fundraising in memory of her husband, Andrew. It is thanks to Faye and all those who donated and continue to support the charity, that this Handbook has been made possible. The handbook’s digital format will enable us to update the enclosed information for years to come, ensuring we can maintain an up-to-date expert and research informed resource for all affected by neuroendocrine cancer. We hope this handbook honours Andrew’s memory and serves as a lasting legacy - and acknowledgement - of his family’s incredible generosity and support. You can find Faye, and Andrew’s, candidly honest love story at the conclusion of SECTION 08.
COME
Our Values
• Promote equality and equity of care for all neuroendocrine cancer patients in the UK • Promote excellence in support, care, treatment and research • Promote a fair allocation of national resources and inclusion in national policies and strategies for all cancer types • Promote collaborative working and build an accessible, approachable, fraternity across all healthcare • Deliver honesty, transparency and integrity to promote confidence and trust in NCUK
ABOUT YOU
ABOUT US
CONTACT DETAILS
Helpline: 0800 434 6476 Office: 01926 883 487 (Tuesday-Thursday 10am-4pm)
Office contact form Click here
Office email: office@nc-uk.org www.neuroendocrinecancer.org.uk
DISCLAIMER: Neuroendocrine Cancer UK is not a clinical or healthcare service - it is an information and support organisation. This handbook (and all it contains - including videos and podcasts) is for informational or educational purposes only, and does not substitute expert professional medical advice or consultations with expert healthcare professionals. We, therefore, strongly recommend that you always seek the advice of your doctor or other qualified healthcare provider with any questions you may have regarding a medical condition or treatment, including diet, before undertaking a new healthcare regimen or altering your current healthcare regimen. We also recommend that you never disregard expert professional medical advice or delay in seeking it because of something you may have read, watched or heard in this handbook.
SECTION 01 UNDERSTANDING NEUROENDOCRINE CANCER
You will find the following information in Section 01:
• What is Neuroendocrine Cancer? • How are Neuroendocrine Cancers formed? • The difference between normal cells and cancer cells - Explained • Is Neuroendocrine Cancer genetic? • Does primary site matter? • Staging and Grading (including differentiation) • What is meant by the terms “Functioning” and “Non- Functioning”? • What is meant by the terms ‘metastatic’ or ‘secondary cancer’? • Complications or Consequences of Neuroendocrine Cancer • Mythbusters - Section 01
SECTION 01 REFERENCES
We were able to provide this free information resource due to the wonderful donation made by Faye Cheetham. if you would like to donate towards our services please click here.
What is Neuroendocrine Cancer?
Description & overview
Each year in the UK, around 6,000 people are diagnosed with a neuroendocrine cancer. It can occur in people of any age or gender and can develop in many places throughout the body, but what exactly is neuroendocrine cancer?
UNDERSTANDING NEUROENDOCRINE CANCER
Click here to visit our website and read more about neuroendocrine cancer
The Neuroendocrine System
Our bodies are made up of billions of cells including neuroendocrine cells. In health, neuroendocrine cells, help regulate our bodily functions by releasing small molecules that circulate throughout the body, such as neuropeptides, hormones, cytokines and chemokines (see bullet points below). They are present in endocrine glands but can also be found, more diffusely, throughout the body, including just below the surface layer of the skin. The neuroendocrine system (NES) has been described as the interface between the body’s nervous and endocrine systems. Because of this, it has a role to play across an entire spectrum of bodily functions – from digestion and breathing to how we think, feel, and behave. • Neuropeptide – acts as neurotransmitter, allowing messages to be passed throughout the body • Hormone - a regulatory substance that can stimulate specific cells or tissues into action to maintain normal body function • Cytokine - crucial to the growth and activity of immune system cells and blood cells • Chemokine – central to the development and balance of the immune system
How are Neuroendocrine Cancers formed? Neuroendocrine cancer, (known medically as malignant neuroendocrine neoplasm or NEN), was first described as a specific disease in the mid-1800’s. In 1907, the term ‘Carcinoid’ was applied – from the German word for “cancer-like”. This term became very popular amongst the medical community of the time, as it was believed that neuroendocrine cancer behaved very differently to common cancers. It was thought that all neuroendocrine cancers were very indolent, that is, very slow growing and unlikely to spread or behave in the same way as other malignancies. However, 20 years later, Obendorfer revised his view, concluding that ‘carcinoids’ could indeed behave like other cancers, albeit at different growth rates.
Click here to watch the full video on understanding Neuroendocrine Cancer
By the 1950’s. it was more widely accepted that ‘Carcinoids’ could indeed behave like common cancers. While many may takes years to develop and then grow slowly, others could behave more aggressively - and that all have the potential to spread to other parts of the body, regardless of their rate of growth However, how or why, neuroendocrine cancers occur is still not fully understood. But we can apply some of the knowledge we have about normal cells and common cancer cell development, to try to explain how they grow and behave.
Click here to watch the full video on the Endocrine & Neuroendocrine Systems
Normal Cells • Normal cells develop and grow in a controlled manner • There is a consistency in cell size and shape, and cells are organised into discrete tissues • They may have a lower level of dividing cells and cell tissues are clearly demarcated • They have small, uniformly shaped nuclei and a relatively large cytoplasmic volume • Normal cells do what they are programmed to do, then die off to be replaced by new cells. They have a lifespan. If damaged they try to repair themselves or die off • They tend not to travel to other parts of the body • If they have a particular function this will occur normally • They may have normal specialised features and have normal presentation of cell surface markers • If they do grow beyond normal size they tend to push against neighbouring cells, rather than through them
Cancer Cells
• Cancer cells grow in an uncontrolled way
• There is variation in cell size and shape and disorganised arrangement • There may be a large number of dividing cells and poorly defined tumour boundaries • There is a large, variable shaped nuclei and relatively small cytoplasmic volume • There is a lack of cell repair and replacement or death, which can result in tumour formation of immature, abnormal (cancerous) cells • Cancer cells will travel to other parts of the body and settle (metastasise) • If they have a particular function, this can become abnormal or altered • There is a loss of normal specialised features and elevated expression of certain cell markers • When they do grow beyond their normal size, they will invade neighbouring cells and structures (infiltration)
The difference between normal cells and cancer cells - Explained
HISTOPATHOLOGY Click here to listen to the Not Just NE Cancer podcast with Consultant Histopathologist, Dr Claudia Mestre
Click here to watch the full video on Terminology & Classification
Understanding How Type 1 Gastric Neuroendocrine Tumours Develop CLICK HERE to visit our website and read a past update from Mark Pritchard – Professor of Gastroenterology at the University of Liverpool and Head of the Department of Cellular and Molecular Physiology
Male/female Endocrine System: image adapted from Stock Photo ID: 197574979 BIGSTOCK https://www. bigstockphoto.com/
Is Neuroendocrine Cancer genetic? Most neuroendocrine cancers occur ‘sporadically’, this means that there is no known genetic (inherited) link to how or why they have developed. However, there are a number of rare inherited genetic conditions that may increase someone’s risk of developing a neuroendocrine cancer, these include: • Multiple Endocrine Neoplasia MEN types 1-4 • Familial medullary thyroid cancer (FMTC) • Familial phaeochromocytoma +/- paraganglioma (Adrenal gland related tumours) • Parathyroid hyperplasia
• Familial isolated pituitary adenoma (FIPA) • Succinate dehydrogenase (SDH) disorders
• Von-Hippel Lindau • Neurofibromatosis 1
Click here to listen to the Not Just NE Cancer podcast, with Jo Grey, CEO of AMEND
Does primary site matter? Primary site refers to where your neuroendocrine cancer started to grow. Primary site matters because it can influence how and why symptoms may occur. It can also make a difference to what treatment is used - for example, whether surgery is possible or not, due to position of tumour and whether surrounding tissue or structures may be affected. CLICK on the link at the bottom of this page to find out more about primary and secondary cancer sites.
PRIMARY & SECONDARY SITES Click here to visit our website and read more
Staging and Grading (including differentiation)
Stage Looking at where your neuroendocrine cancer is can tell us whether it (has spread to other places in the body). Staging is usually assessed by scans / histology. Commonly, there are four stages – but the criteria for each stage can Stage I: Early stage Stage II: Localised infiltration Stage III: Regional infiltration Stage IV: Advanced stage CLICK HERE t o find out more about your primary cancer site
Click HERE to find out more about Staging and Grading
t is ‘early stage’ (limited to the area in which it starts) or ‘advanced’
vary depending on the primary site:
Grade Examining neuroendocrine cancer cells under a microscope can provide vital information about how it is likely to behave, in terms of growth and potential to spread. It is also important in treatment decision-making - to ensure the most appropriate anti-cancer therapy is offered. It is vitally important that this grading examination is undertaken by an expert - a histopathologist with experience in examining neuroendocrine cancer. Histopathologists are expert doctors who are responsible for diagnosing and studying disease in tissues and organs, including cancer. They carefully examine samples, taken by aspiration, biopsy or surgery, to determine a diagnosis. There are 2 key factors that can help accurately grade neuroendocrine cancer: 1. differentiation: which refers to cell appearance 2. mitotic rate: which refers to how quickly or slowly cancer cells are dividing and growing
Neuroendocrine carcinomas (NECs) are treated differently to neuroendocrine tumours (NETs). Click HERE to find out more about NECs.
Differentiation refers to how different the cancer cell looks like compa this information it is possible to divide neuroendocrine cancers into 2 m neuroendocrine carcinomas: Neuroendocrine tumours (NETs) are well-differentiated cancers. Th change, the neuroendocrine cancer cells that form the tumour, have k cell. Their mitotic rate can vary from slow to rapid. Neuroendocrine carcinomas (NECs) are poorly-differentiated cance become very abnormal, with obvious features of cancerous change, lo They are also distinct by cell size - and can be further classified as eit consistently high. Mitotic rate: is a measure of how fast cancer cells are dividing and growing. To find the mitotic rate, the number of cells actively dividing in a certain amount of cancer tissue, is counted. This may be reported in histopathology reports using the abbreviation MiB-1 or Mitoses per 10 HPF. However, for most neuroendocrine cancers, histopathologists use Ki67 to ‘stain’ or identify actively dividing cancer cells: Ki67 is a protein that is present during all of the active stages of the cell cycle, a valuable marker of proliferation (cell division and growth), often expressed as a percentage (%). You may see the term Ki67 followed by a percentage, used in your clinical letters or reports. The higher the Ki67% the more rapidly the cells are dividing and growing.
ared to what it should look like as a normal cell. Using main groups, these are neuroendocrine tumours or
his is because although abnormal, with evidence of cancerous kept many of the characteristics of a normal neuroendocrine
ers. This is because the neuroendocrine cancer cells have osing many of the features of a normal neuroendocrine cell. ther small cell or large cell carcinomas. Their mitotic rate is
Q&A Session ‘High Grade’ with Professor Nick Reed
Grading differences NETs are well-differentiated cancers with different grade categories. NECs are poorly-differentiated cancers - a distinct group with a consistently high Ki67 - and therefore are not routinely assigned a grade (though some may call them Grade 3). NET gradings: • Grade 1 is a well-differentiated cancer with a Ki67 less than 3% • Grade 2 is a well-differentiated cancer with a Ki67 between 3 and 20% • Grade 3 is a well-differentiated cancer with a Ki67 more than 20%
(b)
(a)
(c)
(d)
Histology Image Source: Cuthbertson, Shankland & Sririjaskanthan (2023) Clin Med)
Ki-67 staining of neuroendocrine cancer: (a) grade 1 NET (ileum), (b) grade 2 NET (lung, atypical carcinoid), (c) grade 3 NET (pancreas) (d) NEC (small cell lung cancer)
However, the exact cut-off criteria between G1-3 NETs may differ depending on primary site. For example, in lung - where well-differentiated tumours are often referred to as either typical or atypical carcinoids, rather than NETs - the cut off is: • Typical carcinoid: A well-differentiated lung neuroendocrine cancer with a Ki67 of less than 5% would be considered low-grade. • Atypical carcinoid: A well-differentiated lung neuroendocrine cancer with a Ki67 between 5 - 30% may be considered moderate-grade. • NEC: poorly-differentiated, either small or large cell carcinomas, with a Ki67 more than 30%.
La Rosa (2023) Endocrine Pathology (2023) 34:79–97)
The importance of accurate grade cannot be over- emphasised as it is a key determinant of both treatment planning and decision-making, alongside prognosis (where prognosis is the prediction of the most likely course of disease as well as the treatment response)
What is meant by the terms “Functioning” and “Non-Fu Functioning or Non-functioning refers to the hormone or chemical prod • Non-functioning neuroendocrine cancer cells usually retain their abi • Functioning neuroendocrine cancer cells produce and release abno they are usually responsible for. This abnormal release can result in hormone-associated symptoms / s Please Note: Functioning is a term that may also be used to describe nuclear medicine scans (e.g., Gallium68 Dotatate or FDG-PET scans)
In summary: Neuroendocrine Tumours (NETs) are: • well-differentiated cancers
• may be graded 1-3, depending on their Ki67% • may be Stage 1-4, depending on whether they are early or advanced stage • may be functioning or non-functioning Neuroendocrine Carcinomas (NECs) are: • poorly-differentiated cancers • have high Ki67 (above 20%) • may be small cell or large cell type in appearance • may be Stage 1-4, depending on whether they are early or advanced stage • are usually non-functioning (n.b. paraneoplastic syndrome may occur)
unctioning”? duction and release by neuroendocrine cancer cells. ility to release normal amounts of hormone or chemicals. ormal, that is higher than normal, amounts of the hormone or chemical syndromes. whether your cancer can it be seen on ‘functional imaging’ such as ).
Click here to find out more about Secondary (Metastatic) Neuroendocrine Cancer
What is meant by the terms ‘metastatic’ or ‘secondary cancer’?
Terminology is important. “Metastatic cancer” refers to the spread of primary cancer cells from the place where they first formed (primary site) to another part of the body. These ‘secondary sites’ or ‘disease spread’ are called metastases. This is distinctly different to those who may have 2 different types of cancer at the same time - for example, a bowel cancer AND a neuroendocrine cancer. Both are primary cancers, and the term “secondary primary cancer” may be used. How do metastases occur? Metastases occur when cancer cells break away from the original (primary) cancer, and travel through the blood or lymph system, to form a new site of disease in another organ or part of the body. This new site of disease, called a metastasis, will be formed by the same kind of cancer cells as those found in the primary site. For example - if your primary site is the small bowel and it has spread to the liver - the metastases in the liver will be formed by cancer cells that have come from the small bowel. They will ‘small bowel liver metastases’.
Development of Cancer Image Source: https://www.cancer.nsw.gov.au/about-cancer/cancer-basics/ what-is-cancer
Different neuroendocrine cancers affect people in different ways. This • where they are (primary site and stage), • differentiation • how slowly or rapidly they are growing - mitotic rate / Ki67% • whether they are functioning or non-functioning • whether they are associated with symptoms or not • and whether they have a genetic link or not They can also appear to behave differently when other factors are in p • other health conditions - incl another cancer • age and well-being • access to expert healthcare • access to disease-appropriate treatments • responses to treatment • decisions / choices made regarding care and treatment (by healthca Although neuroendocrine cancers may share similar characteristics, th treated, can be different between people - even if they have a similar d
can depend on:
play, for example;
are professionals and the person diagnosed) he diagnosis, the way the cancer may behave and the way it may be diagnosis (site, stage, grade).
Mythbusters - Section 01
Myth #1 - It’s Carcinoid - “not really cancer”. “Ca
“Carcinoid” is an outdated term - and is based on work undertak for carcinoid or cancer-like) was first used by Obendorfer (1907) Less well publicised is that some 20 years on, he revised his opi these tumours could indeed invade and spread and were indeed The term “carcinoid” is no longer recommended, because it fails cancers harbour. The term is also confusing, because it promote carcinoid syndrome, when most do not.
Myth #2 - Neuroendocrine Cancer is a slow-gro
Neuroendocrine cancers are not all the same. There are 2 distin all NECs are rapidly growing, NETs can show different rates of g
Myth #3 - Neuroendocrine Cancers are incurab
As with most other cancers, diagnosed accurately and at an earl
More information on understanding neuroendocrine cancer is
arcinoids are benign”
ken more than 100 years ago, when the term ‘Karzinoide’ (German ). inion and concluded that although rate of growth may be different, d cancers - however the term Carcinoid persists. s to convey the malignant potential that most neuroendocrine es the misconception that all neuroendocrine cancers produce the
owing cancer
nct classifications: NETs and NECs based on differentiation, and while growth, from slow to rapid.
ble
ly stage, neuroendocrine cancers are potentially curable.
s within this handbook - you can also visit our website HERE.
Myth #4 - Incurable means terminal
Many people diagnosed with neuroendocrine cancer will have incur Many live well - and for many years following diagnosis - although t access to specialist care and treatment(s) and any other health con A “terminal illness” is defined as “a disease or condition which can’t is very difficult, especially with so many advances in care and treatm someone might live or die.
UK law (England and Wales) only recognise believes that their death “can be reasonabl This “six-month rule” was not intended to b timescale has no clinical meaning in most It was introduced into law to exempt termi period for benefits.
Click here to read the All-Party Parliamentary Group for Terminal Illness (2019) report TO READ MORE...
rable disease - but this does not mean that they are “terminally ill”. this does depend on the site, grade and stage of their cancer, their nditions they may have. t be cured and is likely to lead to someone’s death” - however, it ments, to determine how long, following an incurable diagnosis,
es that a person is terminally ill if their clinician ly expected within six months”. be a wider definition of terminal illness and the cases.
inally ill people from the six-month qualifying
Myth #5- Neuroendocrine Cancers only occur in the gut (small bowel)
Strange one this, but it’s certainly out there. Whilst the small bowel is one of the most common sites for neuroendocrine cancer to start, other sites include the lungs, pancreas, skin - indeed anywhere in the body that neuroendocrine cells exist.
Myth #6 - Only the stage of your Neuroendocrine Cancer defines how serious it is This is another myth it’s important to tackle upfront. The stage of your neuroendocrine cancer can give important information about the primary site and whether it has spread. However, other factors such as how active it is (grading), what the tumour cells look like under a microscope (differentiation) and your overall health, alongside tumour staging, will determine how serious it is. So please make sure you always chat to your care team to get the full picture about your condition.
Myth #7 - Sugar feeds cancer. You should avoid / only eat . .
To address the number of myths and misinformation about the “cancer-related” foods would fill a book, there is an industry about this - a money-generating, potentially harmful, largely unregulated industry. So read any information carefully - and always seek expert advice from your specialist team and/or qualified dietitian before making any changes. There is no one specific diet suitable for everybody who has neuroendocrine cancer and sugar provides energy for ALL cells, not just cancer cells. While too much sugar can cause general health problems, e.g. obesity / diabetes, we all need a source of energy. If you have cancer then eating well matters - good nutrition, a healthy, well-balanced diet, can help. You can find out more about evidence-based, dietitian informed, nutrition on our website HERE
SECTION 02 SYMPTOMS OF NEUROENDOCRINE CANCER BEFORE & AFTER DIAGNOSIS
You will find the following information in Section 02:
• Signs & Symptoms of Neuroendocrine Cancer - Before & After Diagnosis • The physical presence of a cancer - it’s size, site and position • Pain • Mythbusters: Pain • Functional signs, symptoms and syndromes
• Carcinoid Syndrome - Explained • Carcinoid Crisis Disease - Explained • Carcinoid Heart Disease - Explained • Syndromes explained • Paraneoplastic Syndromes - Explained
SECTION 02 REFERENCES
We were able to provide this free information resource due to the wonderful donation made by Faye Cheetham. if you would like to donate towards our services please click here.
In this section we will look at 3 key types of symptoms associated with neuroendocrine cancer - that may occur before / after diagnosis: The physical presence of a cancer - it’s size, site and position The effects of functional neuroendocrine cancers - that is, signs, symptoms and syndromes associated with neuroendocrine-related hormone excess : these affect up to 40% of people with neuroendocrine cancer Paraneoplastic signs, symptoms / syndromes / “oncological emergencies” - the effects of cancer itself and the body’s response to it - approximately 1 in 5 people with a cancer, of any type, may develop a paraneoplastic syndrome.
Signs & Symptoms of Neuroendocrine Cancer - Before & After Diagnosis
Click here to watch the full video from David, discussing his symptoms prior to diagnosis
Q&A Session ‘Gastrointestinal Symptoms’ with Dr Raj Srirajaskanthan
The physical presence of a cancer - it’s size, site and position Where a neuroendocrine cancer develops, it’s position and how big it is can affect what symptoms may develop. These symptoms include obstruction and/or pain.
Obstruction: Example 1: Lung Neuroendocrine Cancer
A cancer that starts to grow peripherally, that is, in a position away from the main structures, such as the bronchus, may not cause any symptoms - until (or unless) it grows larger or spreads elsewhere.
Whereas a cancer that starts to grow centrally, that is, in or near to the bronchus, can cause obstructive symptoms, such as a cough and/or wheeze.
Lung image adapted from Diagram 1: The Lungs, Neuroendocrine Cancer UK Lung Booklet p4. https://www.neuroendocrinecancer.org. uk/wp-content/uploads/2020/06/503906-LUNG-BOOKLET-v3.pdf
LUNG NEUROENDOCRINE CANCER Click here to visit our website and read more about lung neuroendocrine cancer
Example 2: Pancreatic Neuroendocrine Cancer
As with lung or any other site, if the cancer starts peripherally, that is, from any main structures - eg tail of the pancreas, it may not cause an immediate signs or symptoms. However, if the cancer develops in the head of the pancreas it may ca blockage or interfere with the drainage of either (or both) the pancrea bile duct. • If the bile duct becomes blocked / obstructed, bile cannot reach the duodenum, so builds up and higher levels of bilirubin develop in the blood, which can lead to jaundice (a yellowing of the whites of the e and skin), fevers and/or steatorrhoea*. • If the pancreatic duct becomes blocked / obstructed, pancreatic juic which contain bicarbonate and digestive enzymes, may not reach th duodenum, which can lead to malabsorption of food and steatorrho Steatorrhoea* : bowel movements that may be difficult to flush away, a pale and oily appearance and can be especially foul-smelling! Not all obstructive symptoms may be related to cancer - infection, inflammation or obstruction due to stones eg gallstones - may also be causes of jaundice, pain and other symptoms.
NEUROENDOCRINE CANCER OF THE PANCREAS Click here to visit our website to read more
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Pancreas image adapted from https://upload.wikimedia.org/wikipedia/commons/0/0d/ Normal_pancreas_.jpg
ces, he oea*.
Tumours that develop in the head or neck of the pancreas may cause pain, bile / pancreatic duct obstruction - leading to inflammation and/or jaundice, bloating / steatorrhea. Tumours in the body or tail of the pancreas may cause epigastric pain (like heartburn or dyspepsia / acid reflux) or back pain (radiating from front of the body or centred between or just below the shoulder blades)
have
e
Click here to watch the full video from Professor Martyn Caplin
Click here to watch the full video from Mike Tadman, Specialist Nurse
*Syndrome, from its Greek roots, basically means "running together”. In medicine a syndrome refers to a group of signs and symptoms that occur together and characterise a particular disease, disorder or health condition. (Treatment related symptoms, including side-effects, will be discussed in the Treatments section)
Example 3: Small Bowel Neuroendocrine Cancer
The small bowel (or small intestine) is the longest part of the digestive system. It is only about as wide around as a middle finger (approximately 1 inch or 2.5 centimetres) and is, on average, 600 centimetres in length in an adult (we need at least 200 centimetres to maintain normal function).
Small Bowel image adapted from https://www.myhsn.co.uk/top-tip/what-does-the-small-bowel-do-5-functions
The small bowel is anchored within the abdomen by a double fold of peritoneum (lining of the abdomen) called the mesentery. Lymph nodes and the blood supply to the intestines run through it. If the bowel becomes kinked or blocked - you may experience symptoms of bowel obstruction.
In small bowel neuroendocrine cancer - it may not be the primary tumour itself that causes pain / obstruction - but either an inflammatory, reactive response (called desmoplasia) / spread of tumour cells to nearby lymph nodes (which then clump together) and may be identified on scans as a mesenteric mass.
If you experience symptoms of bowel obstruction you should be reviewed, as soon as possible, by a medical team - even if by the time you are seen, symptoms have eased off. If your nearest A&E is not your specialist centre - notify your specialist team you are going to A&E and which one (or ask attending staff in the department to do so). A bowel obstruction requires urgent medical attention to not only alleviate the cause, but also to prevent perforation (where part of the bowel wall may be break down, releasing bowel contents into the abdomen) - and to protect the rest of the bowel from damage due to impaired blood supply, nerve damage / lymphatic system issues.
SMALL BOWEL NEUROENDOCRINE CANCER Click here to visit our website to read more
Symptoms may include pain, cramping, diarrhoea / constipation, inability to pass wind, bloating, nausea / vomiting.
If you have a small bowel neuroendocrine cancer or have been told you are at risk of bowel obstruction you can obtain one of our free wallet cards by contacting Neuroendocrine Cancer UK by phone – 01926 883487 or order one through the website by clicking on the link below.
EMERGENCY WALLET CARDS CLICK HERE TO ORDER
Pain
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Pain - Terminology & overview of pain types
There are things that can affect how you feel pain, such as fear, anxiety, depression, a lack of sleep / not being able to carry on with usual activities including work. Feeling fatigued, weak / being anaemic - alongside hormone excess related symptoms - can also affect how pain is experienced. ‘Painkillers’ (pain relieving medicines) are possibly the main treatment that most people expect to be given when they have pain, but they may not be the right or only effective therapy. Finding the right treatment for you is important, and identifying the cause of your pain can help in deciding on what treatment / intervention may be most effective for you.
r for your doctor or nurse to understand the pain you are feeling and eviate it, they may ask : re does it hurt? n does it hurt? e.g. time of day, after activity, after eating t does it feel like?* s anything make it feel better or worse? e.g. heat, cold, eating / not g does your pain affect your daily life and activities? d question* above can be difficult to answer - here are some words that help:
Click here to watch the full video from Dr Ros Taylor, discussing pain
The correct dose of any medication is the minimum dose that produce maximum benefit. Guidelines primarily recommend medication(s) that • can be taken by mouth (rather than injection) • can be taken regularly • are appropriately prescribed for your specific needs “Painkillers” may not completely stop or “kill” the pain but may help alle Other therapies or interventions that may help manage pain include: • Treating the underlying cause - e.g., alleviating constipation • Other medications - e.g., anti-spasmodics can help with cramping, v supplements to correct deficiencies • Relaxation, mindfulness, yoga or similar activities • Movement / gentle exercise can help with musculoskeletal and bone
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In dealing with pain, you may have certain preferences regarding pain-relieving medications - this may be as a result of previous experience (positive or negative) or expectations of care. You may prefer to use complementary interventions, for example, relaxation and/or acupuncture. Other interventions including the treatment(s) you may receive for your cancer may reduce pain.
Click here to listen to the Not Just NE Cancer podcast with Maxine, Simon, Michelle and Debra discussing symptoms
Don’t feel you have to put up with pain: talk with your care team about how you would prefer to manage your pain when or as it occurs If you have had an adverse reaction, an intolerance or allergy to certain medications - please ensure your medical team is aware of this. You should also be able to discuss any concerns you may have about potential about side effects. Pain can be the body’s way of telling us that something is not quite right - as previously mentioned, if you are able to discuss what you’re feeling - both physically and mentally - with your neuroendocrine cancer team, pain team or palliative care (symptom control) team, this can help to identify the cause and tailor treatment more effectively. NB: Not all pain may be due to your cancer, for example, bone pain may be experienced if your vitamin D and/or calcium levels are abnormal. Other causes, such as gallstones, other health conditions / over-exertion or injury, need to be considered to ensure your pain is treated effectively and appropriately.
Click here to listen to this bonus episode of the Not Just NE Cancer podcast with Sally, Neuroendocrine Cancer UK Ambassador, speaking about pain
You can also hear Steve’s podcast with NCUK CEO Catherine Bouvier in our “Not just NE Cancer” series – “Anxieties, Mindfulness and Neuroendocrine Cancer” by clicking HERE. This may be particulalry helpful as Steve turned to mindfulness after suffering chronic pain.
Mythbusters: Pain
Myth #1 - Cancer-related pain is inevitable
Pain is not inevitable. Is it likely? Maybe. Research about patient experiences, has shown that whilst more than two-thirds of people, living with cancer, experience pain at some point leading up to or after diagnosis - the type and degree of pain varies significantly - as do the causes. More than 25%, even at a very advanced stage of cancer, say they have experienced little or no pain at all.
Myth #2 - There’s nothing that can be done to relieve cancer-related pain
There are a range of options to help treat cancer-related pain - doctors can choose from a wide range of pain relief medications, therapies / techniques, that can help - depending on the type and severity of pain. Good communication between you and your care team is vital to ensure that a full assessment is undertaken to understand the root causes of your pain and how it is experienced - so that the right approach and intervention(s) are agreed upon to help you to relieve and manage your pain.
Myth #3 - Palliative care is only for the dying
Palliative care is an often misunderstood specialty, with many thinking it only applies to those who are nearing the end of life. However, in practice, palliative or ‘enhanced supportive’ care is an incredibly useful resource for anyone living with a serious illness, such as neuroendocrine cancer. It focuses on providing support and symptom management strategies throughout ALL stages of disease. This approach, which is used - even where cure is possible - focuses on care for the whole person: mind, body, and spirit.
Myth #4 - Morphine is addictive
When correctly prescribed, taken and reviewed, regular use of morphine, or similar opioids, does not lead to addiction. However, long-term use can lead to an increased tolerance - this may lead to a need to increase the dosage or, consider changing the specific medication to an alternative, in order to achieve the same pain-relieving benefit. But tolerance is not addiction* *Addiction has been defined as a compulsion, or lack of control over doing, taking or using something to the point where it could be harmful to you.
The National Institute for Health and Care Excellence (NICE) has listed conditions where it believes medically prescribed cannabis products might be helpful. Click here to visit nice.org.uk to read in full
Myth #5 - CBD oil / medical cannabis
According to the NHS: There are many cannabis-based products available to buy online, but their quality and content is not known. They may be illegal in the UK and are potentially dangerous. Though some products are available to buy legally as food supplements from health stores, there's no guarantee these are of good quality or evidence to prove they provide any health benefits. Medicinal cannabis, available on prescription may only benefit a very small number of patients. Neither Neuroendocrine Cancer UK nor Cancer Research UK have an organisational policy on the legal status of cannabis, its use as a recreational drug, or its medical use for diseases other than cancer. But we are supportive of properly conducted scientific research into cannabis and its derivatives that could benefit cancer patients in managing symptoms and we will continue to monitor developments in the fields and evidence as it emerges.
Myth #6 - Doctors in the UK won’t prescribe CBD oil or medical cannabis for cancer-related pain In the UK, medical use of cannabis was legalised in November 2018, but it is only legal when prescribed by a specialist consultant - GPs are not currently allowed to prescribe cannabis-derived medicines. NHS guidance states that medical cannabis should only be prescribed when there is clear published evidence of its benefit and other treatment options have been exhausted.
Carcinoid Crisis Symptoms Can be life-threatening Symptoms may be similar to Carcinoid Sundrome but are much more severe: • SEVERE FLUSHING • PALPITATIONS • FAST, IRREGULAR HEART RATE • LOW BLOOD PRESSURE • CONFUSION • BREATHING DIFFICULTY
Myth #7 - Everyone with Neuroendocrine Cancer has or will develop Carcinoid Syndrome Less than half of all neuroendocrine cancers are associated with abnormal hormone release. Neuroendocrine cancer-related syndromes, not just carcinoid syndrome, occur due when neuroendocrine cancer causes changes to the cell resulting in the abnormal production and excess release of specific peptides / hormones by neuroendocrine cancer cells. These syndromes occur in approximately 40% of all neuroendocrine tumours, rarely occurring in neuroendocrine carcinomas.
EMERGENCY WALLET CARDS CLICK HERE TO ORDER
Myth #8 - Carcinoid Crisis is just a ‘flare up’ of Carcinoid Syndrome No. Carcinoid crisis is a life-threatening occurrence that requires immediate medical intervention. It is rare, and whilst those most at risk are people who already have carcinoid syndrome, it may also occur in patients with no history of carcinoid syndrome. The exact reason for carcinoid crisis is unclear, though some believe it may be related to a sudden, excess release of carcinoid syndrome associated hormones, the exact cause is unclear. Carcinoid crisis, if it occurs, is most associated with surgical or medical interventions - for example as general anaesthesia (GA) is started / manipulation of the tumour (such as surgery, ablation or biopsy). Therefore careful planning of any GA and/or intervention is required.
Expert medical guidance, for healthcare professionals, is available from UKINETs here Further information on related complications, including carcinoid crisis can be found HERE
Myth #9 - Carcinoid Syndrome is the only cause of diarrhoea
No. There are many causes of diarrhoea in those who have a neuroendocrine cancer - carcinoid syndrome is only one. Others include: Infection, food intolerance, irritable bowel disorders e.g. crohns or colitis, pancreatic enzyme insufficiency (PEI), small intestine bacterial overgrowth (SIBO), short-bowel syndrome, bile salt malabsorption (BSM), menstrual cycle, anxiety, medications...the list is long. If you have diarrhoea then it is important to talk this through with your specialist team, being as descriptive as possible: how often it occurs, when it occurs, what colour it is. This can help to identify the cause and allow for appropriate treatment. NB not everyone with a neuroendocrine cancer has diarrhoea - indeed many may experience constipation. Bowel health is important - so if your usual bowel habit changes - let your specialist team know. Further information on factors that may cause diarrhoea and constipation can be found in the MENETS “Nutrition and Neuroendocrine Cancer” booklet, available through our website by clicking HERE
FUNCTIONAL SIGNS, SYMPTOMS AND SYNDROMES
In Section 1 we talked about functioning and non-functioning neuroen production and release by neuroendocrine cancer cells. Non-functioning neuroendocrine cancer cells usually retain their abilit Functioning neuroendocrine cancer cells produce and release abnorm responsible for. This abnormal release can result in symptoms / syndr The most frequently talked about syndrome associated with neuroend
Carcinoid Syndrome - Explained
ndocrine cancers and how this refers to the hormone or chemical
ty to release normal amounts of hormone or chemicals. mally higher levels of the hormone or chemical they are usually romes. docrine cancers is carcinoid syndrome.
Carcinoid Syndrome Carcinoid syndrome (CS) is the most frequently experienced hormonal syndrome by those with certain functioning NETs and is defined by chronic diarrhoea / flushing in the presence of elevated levels of serotonin [or its metabolite 5-hydroxyindolacetic acid (5-HIAA)]. Atypical carcinoid syndrome (ACS) may be experienced by those with NETs originating in the lung, stomach, duodenum / ovary. ACS is characterised by a patchy, intensely red, flush, sweating, itching, wheezing, salivary gland swelling, eye-watering (lacrimation) / cardiovascular instability e.g., hypotension (low blood pressure).
Syndromes explained
The following syndromes are explained in depth on the Neuroendocri Cancer UK website. Simply click on the link at the bottom of the page out more. • Clinical hypoglycaemia • Glucagonoma syndrome • Zollinger-Ellison syndrome (ZES) • Werner-Morrison Syndrome (WDHA) - may also be known as VIPo pancreatic cholera syndrome Rarer Pancreatic and/or Duodenal NET-related syndrom include: • Somatostatinoma • ACTHoma • PTHrPoma • Ppoma Other NET hormone-related symptoms and syndromes include: • GRHoma • Pheochromocytoma and paraganglioma (PPGL) symptoms and syn • Calcitoninoma • Neurotensinoma
SYNDROMES EXPLAINED Click here to visit our website and read more about neuroendocrine cancer- related syndromes
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Click here to watch the full video about hormone syndromes from Professor John Newell-Price
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Q&A Session ‘Hormones’ with Dr John Ayuk
Complications or Consequences of Neuroendocrine Cancer
In medicine, a complication (or consequence) means an additional health problem that may occur if a disease is left untreated or as a result of a disease that may continue to develop despite treatment. Complications can be serious and may require urgent medical attention.
A potential consequence of neuroendocrine cancer, specifically carcinoid syndrome, is the development of carcinoid heart disease (CHD). CHD is a NC complication.
Click here to listen to the Not Just NE Cancer podcast with Professor Steeds, Cardiac Consultant
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