While it’s a long way off becoming reality, this is quite an exciting clinical trial. I have no idea if it will pick up Neuroendocrine disease but initially, patients with suspected oesophageal and stomach cancers will be asked to try the test. Later it will be extended to include prostate, kidney, bladder, liver and pancreatic cancers. It’s possible that Neuroendcorine tumours in these locations might be picked up or at least show up some abnormality that triggers further checks.
The fact that Cancer Research UK is involved gives me some confidence as they tend to back the strong horses.
I will keep this article live and track developments.
It’s no secret that Neuroendocrine Cancer can be difficult to diagnose. Although earlier diagnosis is improving (as reported in the SEER database report issued in 2017), there is still a lot of ground to cover. It’s also no secret that certain cancers are difficult to diagnose (NETs is one) and there are a number of reasons why this happens, including but not limited to: – they grow silently, they often produce vague symptoms which can be mistaken for much more common illnesses, and their complexity is not fully understood.
I wanted to cover two different aspects of the problem of finding NETs. Firstly, in finding the primary tumour so that the type of NET can be properly established – this drives the best treatment regime. Secondly in finding all the tumours, as this establishes the correct and most detailed staging declaration – this drives treatment plans and surveillance regimes that need to be put into place.
Hunting Tumours – Primary vs Secondary
It’s really important to determine which tumours are primary and which are secondary (metastasis). There’s a number of ways to help work this out and knowledge of NETs epidemiology studies can help.
Specialist Knowledge – certain things are known about the behaviour of NETs
Specialists and in particular NET specialists will be aware of the vagaries of NETs in terms of what tumours are normally a primary and which are normally secondary and many of the pitfalls involved in working that out. Many NETs will have metastasized to the liver at diagnosis, so whilst it is not impossible to have a primary liver NET, the vast majority of liver tumours found will be secondary (metastases). NET Specialists are more likely to have the experience than generalists. They know that the varying metastatic potential depending on the primary site clearly indicates differing biology and genetics across sites and they know that NETs are indeed a heterogeneous group of tumours. The differences cannot be explained by whether the NET is situated in the foregut, midgut, or hindgut. For example, Appendiceal NET is known to be less prone to metastasis. This may be due to the high rate of incidental ﬁndings during appendectomies, or because the appendix is an immunological organ where malignant cells can therefore be expected to be frequently recognized by the immune system.
The majority of the digestive tract is drained by the portal venous system, explaining the dominance of liver metastases in this group of tumours. This also explains our ﬁnding that many nervous system and bone metastases originate from NETs in the lungs. Disseminated tumour cells may directly reach the systemic circulation from the lungs, whereas if originating from the midgut region, they need to ﬁrst pass both the liver and the lungs.
As an example of this heuristic knowledge, one Swedish study indicated that two-thirds of peritoneal metastases will be attributed to Small Intestine NETs (SI NETs). SI NETs and Pancreatic NETs (pNETs) are the most likely to metastasize. The least likely sites to metastasize are the Appendix and Rectum. The same study indicated that in addition to the common metastatic locations of lymph nodes and liver, Lung NETs are more likely to metastasize to the brain and bone than other types. I believe the findings from this study more or less correlates to other information I’ve had access to and also confirms the technical behaviour paragraph above.
Multiple Primary Tumours
With NETs there are two scenarios:
1. Multiple primaries in same organ/location (multicentric). This is fairly common in small intestine (SI NETs), stomach/gastric NETs (gNETs), and also found in Lung and pNETs too. NET experts will be aware of the issue and know to look for the possibility. This is an important point with SI NETs as it’s quite long although held together by the mesentery, so a ‘Mark 1 eyeball’ can be more efficient in finding NETs in this organ than scans. There is a very well known surgical technique called “running the bowel” where they check the small intestine for signs of other primary tumours – they can do the same with the large intestine. Additional surgeries due to this lack of knowledge could come with significant morbidity. Multiple ‘nodes’ and ‘lesions’ are common in the thyroid.
2. Multiple primaries in different locations. This is common with Multiple Endocrine Neoplasia (MEN) syndromes (the name gives it away) and these may be metasynchronous. MEN1 for example can have tumours in what is called the ‘3 P’ locations, pituitary, pancreas and parathyroid. Of course MEN guys may also have multiple primaries in the same organ (multicentric). Read more about MEN by clicking here.
There’s probably a third scenario (for all cancers) and that is multiple primaries with different cancers (i.e a second, third and fourth cancer etc). Synchronous would be really unlucky but metasynchonous is more likely and there are many NET patients with a second cancer.
What else helps find a NET?
There are many other clues open to those involved in diagnosing a NET:
Patient. Very often the patient plays a big part of determining where the primary and other tumours might be by carefully describing symptoms.
Incidental Finds. NETs are very often found incidentally during trips to the ER/A&E and also during tests for something else. This is particularly the case with Appendiceal NETs and might explain why the average age of a patient is significantly lower in this type of NET.
Blood tests and Hormone Markers. We are not yet in a position where these types of tests can diagnose (but we are moving in that direction). In the case of unknown primaries (CUP), sometimes test results can help to find where some of these cancers started. With NETs, symptomatic patients can often test to confirm an elevated hormone marker which may narrow it down to a specific organ or gland. Read more here.
Scans and Endoscopies. Most cancers of a certain size may show up on conventional scanning such as CT, MRI and Ultrasound. Nuclear scans are now playing a bigger part in finding tumours which betray their location through functional behaviour by lighting up or glowing on these imaging devices. Endoscopies (e.g. gastroscopies, colonoscopies, even gastro intestinal pill cameras can be used) can help but like scans are not foolproof). Generally with NETs, if you can see it, you can detect it. Read more here.
Hereditary Conditions. Around 5-10% of NETs are hereditary in nature, mostly involving the MEN group of syndromes. Many of those people will know they are at risk of developing NETs and their doctors should know the most common locations for primary tumours associated with each gene. So a declared or suspected hereditary syndrome is useful in finding primary tumours if they exist and are proving difficult to find.
Biopsies. “Tissue is the issue”. Pathology can very often give really strong clues as to the type of NET and therefore the likely location of a primary tumour, for example additional tests such as immunostains. Many biopsies will come from secondary cancer (metastases), mostly the liver. Despite all the potential diagnostic routes above, the place the cancer started is sometimes still not found and this may lead to atypical diagnostic/treatment plans and in certain cases this might even include exploratory biopsies via surgery (invasive/minimally invasive), perhaps combined with opportunistic tumour removal if found during the procedure.
Staging. Simple staging can be given if locations of metastases are known. For example in the case of Liver metastases, the stage is automatically Stage 4. However, the full staging definition relies on knowing distant metastases, loco-regional metastases and the full Tumour/Node/Metastases (TNM) definition (size, spread, etc) cannot be fully complete without a primary. Read more here.
Cancers of Unknown Primary
Cancer is always named for the place where it started, called the primary site. Sometimes doctors can’t tell where a cancer may have started. When cancer is found in one or more places where it seems to have spread, but the site where it started is not known, it is called a cancer of unknown primary (CUP) or an occult primary cancer.
When you look at the ratio of all cancers, the figure for cancers of unknown primary (CUP) is quite startling. Depending on where you look the figure is around 2-10%. That doesn’t seem a lot but when you consider the amount of people diagnosed with cancer, the total figure must be staggering. Interestingly, Cancer Research UK say that 60% of CUP cases are in the over 75s. In another interesting Swedish study, doctors claimed that the rates of metastatic cases were higher with certain NETs than they were in their anatomical counterparts, reinforcing the dangerous and sneaky nature of NETs.
Despite quite advanced scanning and diagnostic testing currently in place, and the extensive knowledge of NET specialists, there can still be reasons for not being able to find the primary tumour:
The primary is just too small to be seen and is growing quite slow. Very small cancers might not cause symptoms or be seen on scans. This is a particularly relevant point with NETs.
The primary could be hidden in tissue in between different organs causing confusion about the actual primary location.
The body’s immune system killed the primary cancer. It’s also possible (but not common) that any secondary cancer (i.e. metastases) is still growing.
The tumour has become loose from its primary location and exited the body, e.g. from a wall of the bowel and excreted out in the stool.
The primary cancer was removed during surgery for another condition and doctors didn’t know cancer had formed. For example, a uterus with cancer may be removed during a hysterectomy to treat a serious infection.
I hope this is useful for many NET patients, particularly those who are looking for a diagnosis or looking for a primary tumour.
Neuroendocrine Cancer – at times, it can really be like looking for a needle in a haystack.
Eight years ago today, I was sat in front of a secondary care consultant, his speciality was colorectal. I asked specifically for this consultant for two reasons, firstly, he carried out a colonoscopy some 20 months previously which turned out to be negative. Secondly, my GP had referred me to the iron deficiency anaemia clinic, and they wanted to do ….. a colonoscopy. I changed that plan because this “non-issue” was dragging on; quite frankly I wanted it to be resolved quickly, and I wanted it to be resolved in my favour – after all, I wasn’t actually ill!
Rewind two months, I had an incidental set of blood tests ordered by a nurse following a routine visit to my local medical centre (……. “I think I’ve lost a bit of weight“). My haemoglobin was low (even lower on repeat testing). The GP compared my results to someone in their eighties with malnutrition. In hindsight, I should have been alarmed by that statement but instead I went on holiday to Barbados. Apparently low haemoglobin is a sign of iron deficiency anaemia. I suspected it would pass, either my blood results would revert to normal naturally, or they would after a prescription for some pills. That’s what normally happens, isn’t it? I was so indifferent to the issue, I even delayed the blood tests by three weeks.
Back to 8th July 2010 ….I hadn’t really given him many clues but within minutes of chatting with the secondary care consultant (who was armed with the results of the negative colonoscopy test), he said “what are you doing this afternoon“. I had no hesitation in saying “whatever you want me to do“. I’m still not getting it as I saw this as a chance to get an all clear, get some pills, get back to normal. To cut a long story short, the results confirmed I had a metastatic cancer. If you can see it, you can detect it.
Following the scan results, I had a dozen other tests to narrow it down to Neuroendocrine Cancer (eventually confirmed by biopsy). During these 2 weeks of tests, I finally confessed for the first time that I had been experiencing facial flushing and intermittent diarrhea. In those days, I wasn’t really in tune with my body.
I had been sitting on a beach in Barbados sipping piña coladas with my wife and neither of us had any inkling that I had a serious life threatening illness and that it had been growing inside of me for some years. Slow but sneaky? You betcha. They did some damage too – check out my treatment summary here.
I remain thankful to all those involved in the triggering of my ‘incidental’ diagnosis. The Nurse who ordered the ‘just to be sure’ blood tests, the GP who immediately referred me to secondary care (increased my chances of being diagnosed with cancer), the secondary care specialist who was instrumental in getting to the bottom of the problem in double-quick time.
My intransigence, denial and withholding vital symptoms from the doctors didn’t really help – there’s a lesson for all there.
On the day I was diagnosed, I hadn’t really thought about questions, the only one I actually remember asking was “how long do I have left to live” (I watch too many movies!). On the day of diagnosis and a period beyond, people tend to feel emotions of shock, denial, anger and sadness, before going on to accept their situation. Yes, I ‘googled‘ but not a great deal really – although some things I found did frighten me. I wish I had found this article way back then.
As things progressed in the weeks after ‘D-Day’, I started to work out the sort of things to ask but even then it was limited. I had been referred to an experienced NET team so I felt confident they would do whatever needed doing. In hindsight, I can now think of a quite a few questions I should have asked. That said, I suspect my team probably gave me the answers without having been asked the questions!
My blogging efforts have turned into a ‘Community’ of sorts. Consequently, I’m contacted daily from people finding me on the web. Many of these people are at the pre-diagnosis or initial phase. Many are undiagnosed. Most are looking for information and some sound like they are already at the ‘acceptance stage’; some are frightened about the future, some are angry because they think they are not being told important information and some also feel they have been messed about or ‘fobbed off’ by their doctors. Of course I’m happy to help but only after reminding them that I’m just a wee Scottish guy with the same disease!
I have to say that some people arrive on my site without a diagnosis but often seem to be very well prepared – the power of the internet I suspect. The questions I mostly get involve finding experts and then what questions to ask them.
As an extra bonus to this post, I offer you a starting point for the best places I know for finding NET expertise:
One US center is now the first to achieve a European NETs Center of Excellence accreditation – read more hear about University of Iowa Holden Comprehensive Cancer Center – click here
NANETS have listed “NET Centers” here – NANETS NET Centers and Clinics
The NET Research Foundation as they also have a ‘Doctor Database’ section which differs slightly from CCF below.
Dr. Shereen Ezzat at Princess Margaret in Toronto (PMH)
Dr. McEwan, The Cross Clinic, Alberta?
Dr Kavan at Montreal Jewish General Hospital (Oncology)
Dr Buteau / Beauregard at Quebec Hotel Dieu (Radiation Oncology (PRRT, Ga68)
Dr Rivera at Montreal General Hospital (Endocrinology)
Dr Metrakos at the Montreal Royal Victoria Hospital (Surgeon) sees a lot of NET patients
On the French side Dr Andre Roy at the CHUM in Montreal (surgeon) also sees a lot of NET patients
Dr. Jamil Asselah also treats net patients. He is an oncologist….Quebec
Michael Sawyer at Cross Clinic in Alberta Edmonton.
Drs. Parkins, Card, and Paseka at the Tom Baker CC in Calgary.
London Ontario: Dr. David Laidley, Dr. Robert Reid in the Neuroendocrine Clinic at London Regional Cancer Program and Dr. Daryl Gray, Surgeon.
Russia – Clinical Oncology Research Institute, N. N. Blokhin RCRC RAMS, Address: 24, Kashirskoye sh., Moscow, 115478, RF. NET specialist Alla Markovich
In my Group – ask other patients: Click here to join.
Neuroendocrine Cancer – 10 questions to ask your specialist
Many people ask me what sort of questions to ask and because NETs is such a diverse bunch of diseases, that leads to me ask them a series of questions to ascertain what they might consider asking. I’m not surprised to find some are unable to answer my questions and so those then become some of their questions to ask!
Also, questions don’t end at the diagnosis phase, they continue and in fact, some of the answers to the questions below, may bring up new questions in your mind. Some of these questions can be asked time and time again in the event of issues downstream.
If you’re currently confused about the essential facts of your condition, you’re not alone. In a recent study, almost half of cancer patients did not know basic stuff such as grade and stage of cancer, and after their initial treatment, whether they were free of disease or in remission.
For those entering or are recently just beyond the diagnostic phase, you may find certain questions cannot yet be answered without further test results etc. However, if the answer is not yet known for whatever reason, at least you have it on your list for follow up appointments. Consequently, I’ve constructed this list of questions that should function as a generic set. There may also be ‘specific to country’ questions such as insurance cover in addition to this suggested list. Of course, some of you may not want the answer to so certain questions. That’s perfectly understandable, so don’t ask!
1. Where is my primary tumour and what type of NET is it?
This is a fundamental question and it’s likely many will already have some inkling about location and perhaps a type. The difference between NETs and other types of cancer is the primary can be found wherever there are Neuroendocrine cells rather than a specific part of the anatomy in terms of naming the type of cancer, i.e. a NET of the pancreas is not Pancreatic Cancer.
The type of NET is key as it will drive a lot of other stuff including treatment. Location and type of NET are not always aligned, for example, you may have a NET in your Pancreas but there are several types of Pancreatic NET (or pNET) and these may depend on identification of a particular hormone (see syndrome below). Many NETs are non-functional (there is no oversecreting hormone).
For some the primary will not yet be found (i.e. cancer of unknown primary or CUP). There may also be multiple primaries.
2. What is the grade and differentiation of my tumour(s)?
Another fundamental question as this defines the aggressiveness of the disease and is absolutely key in determining overall treatment plans. Treatment plans for poorly differentiated can be very different from well differentiated. Read more here – Grading and here – Benign or Malignant
3. What is the stage of my disease?
Fundamental to understanding the nature of your disease. Stage confirms the extent of your disease, i.e. how far has it spread. Again this will drive treatment plans and long-term outlooks. Scans are really important in determining the Stage of your cancer – check out my scans post here. Read more here on Staging
4. Do I have a NET Syndrome?
Many NET patients will have been experiencing symptoms prior to diagnosis, perhaps for some time. It’s possible these symptoms form part of what is known as a ‘Syndrome’ and there are several associated with NETs. Syndromes are mostly caused by the effects of over-secretion of hormones from the tumours, a hallmark of Neuroendocrine disease. Carcinoid Syndrome is the most common but there are many more depending on the primary location. Read more here – NET Syndromes.
5. What is my treatment plan, and what are the factors that will influence my eventual treatment? When will I start treatment
This is a very complex area and will depend on many factors. Thus why your specialist may not have the answers to hand. Decisions on treatment are normally made by some form of Multi-disciplinary Team (MDT). Many people diagnosed with cancer expect to be whisked away to an operating theatre or chemotherapy treatment. However, for many this is not what actually happens. Depending on what testing has been done up to the actual diagnosis, it’s possible that even more testing needs to be done. Additionally, for those with an accompanying syndrome, this will most likely need to be brought until control before certain treatments can be administered; and even then, there may be checks to make sure the treatment will be suitable. Sometimes it’s a case of ‘Hurry up and wait’. My first treatment was 6 weeks after diagnosis and that was designed to control my syndrome ready for surgery which was undertaken 14 weeks after diagnosis. It’s also possible you will be placed on a ‘watch and wait’ regime, at least to begin with.
6. Can you comment on the potential for my type of NET to be related to any familial or genetic aspects of cancer?
A small percentage of NETs are hereditary/genetic in nature. This is mostly associated with those who have Multiple Endocrine Neoplasms (MEN) syndromes and a few other less common types of NET including Pheochomocytoma / Paraganglioma(Pheo/Para) and Medullary Thyroid Carcinoma (MTC) (the familial version of MTC is often referred to as FMTC). However, please note this does not mean that all those diagnosed with pancreatic, parathyroid, pituitary, Pheo/Para and MTC tumours, will have any hereditary or genetic conditions, many will simply be sporadic tumors.
7. Will you be able to get rid of all my disease?
This is a really difficult question for any specialist, even a Neuroendocrine expert. All published articles on NETs will say they are a heterogeneous collection of diseases (i.e. consisting of dissimilar entities) which makes this question (and others) difficult. I have read articles written by the world’s foremost NET experts and they all have the word ‘curative’ mentioned in various places. So I guess in particular scenarios with certain NETs, and if the disease is caught early enough, that possibility exists. However, for many, the disease could be incurable, particularly where there is distant metastasis. But, the disease has many treatment options for most types and for many it is possible to live as if it were a chronic condition. I call it ‘incurable but treatable’. Read more here – Incurable vs Terminal
8. What Surveillance will I be placed under?
Again, this is very individual in NETs and is mainly dependent on type of NET, grade and stage and how the patients reacts to treatment. This may not be known until you have undergone your initial treatment. For example, surveillance scans can be any period from 3 months to 3 years depending on tumour type(location) and stage/grade. Marker testing tends to average around 6 monthly but could be more or less frequently depending on what’s going on. Read more here – click here
9. Will I receive support and specialist advice after my treatment?
Let’s not be afraid of the word ‘Palliative’, it does not always mean ‘end of life’ care. Another example is nutrition. Many people with NETs, the condition in combination with the side effects of treatment may necessitate an alteration of diet and this is a very individual area. I would also emphasise that dietitians not well versed in NETs might not offer the optimum advice. Read more – My Nutrition Series.
10. How will treatment affect my daily life?
This is a question that many people miss but it’s becoming more important as we all live longer with cancer Again, this may not be possible to answer immediately but perhaps this question could be reserved once you know which treatment(s) you will be receiving. All treatment comes with side effects and can last for some time or even present with late effects after some years. The ‘consequences’ of cancer treatment need to be factored in earlier so that the necessary knowledge and support can be put in place. See also Unmet Needs for NET Patients
I suspect others will have suggestions for this list so feel free to submit these to me. I quite often refresh my posts over time.
Scanning is a key diagnostic support and surveillance tool for any cancer. Even though you have elevated bloods or urine (….or not), a picture of your insides is really like a thousand words…. and each picture has a story behind it. Scanning can be a game changer in the hunt for tumours and although scans do not normally confirm the cancer type and grade, they certainly help with that piece of detective work and are key in the staging of the cancer.
When I read stories of people in a difficult diagnosis, I always find myself saying ‘a scan might resolve this’ and I always suggest people should try to get one. Even in the case of a story about late diagnosis or a misdiagnosis, I find myself thinking ‘if only they had done a scan earlier’. Despite what you read on NET forums, a CT scan will be able to find some evidence of tumour activity in 90-95% of cases. However, some are cunningly small or hiding and it can be like trying to find a needle in a haystack.
However, scans are not an exact science…..not yet! Apart from human error, sometimes tumours are too small to see and/or there are issues with ‘pickup’ (i.e. with NETs, nuclear scans need efficient somatostatin receptors). The differences between scan types are more quality (sensitivity) related as new technologies are introduced.
As for my own experience, I was very lucky. I managed to get a referral to a specialist early on in my diagnosis phase. He looked at the referral notes and said “what are you doing this afternoon“. I replied “whatever you want me to do“. He didn’t know I had cancer but his instincts led him to believe he needed to see inside my body, he wanted to scan me. The scan results were pretty clear – I had a metastatic Cancer and further checks were now needed to ascertain exactly what it was. So I took my seat on the roller coaster. Medicine is not an exact science (not yet anyway) but here’s something I believe is a very common occurrence in all cancers – If your doctors don’t suspect something, they won’t detect anything.
There’s frequent discussion about the best types of scans for different types of NETs and which is best for different parts of the anatomy. There’s also different views on the subject (including in the medical community), However, a few well known facts can be gleaned from authoritative NET sources:
Computed Topography (CT)
CT scans are often the initial imaging study for a patient presenting with signs or symptoms suggestive of many cancers including NET. These studies are most useful for disease staging and surgical planning as they provide excellent anatomic detail of the tumors themselves and surrounding structures. Primary NETs (GI and lung NETs) and their metastases are generally hyperenhancing with IV contrast and are best seen in the arterial phase of a triple phase CT scan.
In primary NETs, the average sensitivity of a CT scan is 73%. CT scans have even better sensitivity in detecting NET metastases, as they demonstrate 80% sensitivity for liver metastases (but see MRI below) and 75% sensitivity for other metastases (non-liver). This modality is also useful when the primary tumor site is unknown. In one single-institution retrospective study, it was the most common study ordered to look for an unknown primary tumor site and was able to uncover the primary in 95% of cases.
Magnetic resonance imaging (MRI)
MRI is the best conventional study to detail liver metastases in NETs. It is not as useful as CT for the detection of primary small bowel lesions or their associated lymphadenopathy, but is good for the detection of primary pancreatic NETs. A study comparing MRI, CT and standard somatostatin receptor-based imaging (OctreoScan) reported 95.2% sensitivity for MRI, 78.5% sensitivity for CT and 49.3% sensitivity for the OctreoScan in detecting hepatic metastases. MRI also detected significantly more liver lesions than the other two modalities.
You may see something called Magnetic Resonance Cholangiopancreatography (MRCP). Magnetic resonance cholangiopancreatography (MRCP) is a special type of magnetic resonance imaging (MRI) exam that produces detailed images of the hepatobiliary and pancreatic systems, including the liver, gallbladder, bile ducts, pancreas and pancreatic duct.
The primary role of conventional ultrasound in neuroendocrine disease is detection of liver metastases and estimation of total liver tumor burden. This technique has the advantages of near-universal availability, intraoperative utility, minimal expense and lack of radiation. Most examinations are performed without contrast, which limits their sensitivity (compared with CT and MRI). I know in my own situation, US was used as a quick check following identification of multiple liver metastasis during a CT scan. I’ve also had US used to monitor distant lymph nodes in the neck area but always in conjunction with the most recent CT scan output.
Endoscopic Ultrasound (EUS)
With increased access to endoscopy, NETs in the stomach, duodenum, and rectum are increasingly incidentally detected on upper endoscopy and colonoscopy. Patients are frequently asymptomatic without any symptoms referable to the a NET (i.e. non-functional). EUS has also been used to survey patients at increased risk of developing pancreatic NETs. For example, patients with multiple endocrine neoplasia (MEN). They are also frequently used in conjunction with biopsies using fine needle aspiration (FNA) guided by EUS.
Somatostatin receptor-based imaging techniques
Somatostatin is an endogenous peptide that is secreted by neuroendocrine cells, activated immune cells and inflammatory cells. It affects its antiproliferative and antisecretory functions by binding to one of five types of somatostatin receptors (SSTR1- SSTR5). These are G-protein coupled receptors and are normally distributed in the brain, pituitary, pancreas, thyroid, spleen, kidney, gastrointestinal tract, vasculature, peripheral nervous system and on immune cells. Expression of SSTRs is highest on well-differentiated NETs. Somatostatin receptor type 2 is the most highly expressed subtype, followed by SSTRs 1 and 5, SSTR3 and SSTR4.
It must be noted that even the most modern scans are not an exact science. Radionuclide scans are like conventional imaging, they can be subject to physiological uptake or false positives, i.e. they can indicate suspicious looking ‘glows’ which mimic tumours. This article explains it better than I can – click here.
The ubiquity of SSTRs on NET cell surfaces makes them ideal targets for treatment (e.g. Somatostatin Analogues (Octreotide/Lanreotide) and PRRT), but also for imaging. There are two primary types of somatostatin receptor-based imaging available:
Octreoscan – In111 based
The most common (currently) is the OctreoScan or Somatostatin Receptor Scintigraphy (SRS), which uses the ligand 111In-DPTA-D-Phe-1-octreotide and binds primarily to SSTR2 and SSTR5. In its original form, it provided a planar, full body image. In modern practice, this image is fused with single photon emission computed tomography (SPECT) and CT. This takes advantage of the specificity of the OctreoScan and the anatomic detail provided by SPECT/CT, improving OctreoScan’s diagnostic accuracy. These improvements have been shown to alter the management in approximately 15% of cases, compared with just OctreoScan images. In primary tumors, the OctreoScan’s sensitivity ranges from 35 to 80%, with its performance for unknown primary tumors dipping beneath the lower end of that range (24%). Its ability to detect the primary is limited by the size but not SSTR2 expression, as tumors less than 2 cm are significantly more likely not to localize but do not have significantly different SSTR2 expression than their larger counterparts.
Octreoscan – Tc99m based
In one study, it was shown that sensitivity and negative predictive
values of Tc-99m-Octreotide scan is significantly higher than that of CT
and MRI. Using Tc-99m instead of In-111 had several advantages that
include better availability, cheaper and higher quality images. In
addition, to less radiation exposure to both patients and nuclear
medicine personnel. In the absence of Ga68 PET, this could prove a reliable alternative. Please note this scan is completed in a single day vs In111 Octreotide time of 2-3 days.
Ga68 PET (or SSTR PET in general)
The newest somatostatin receptor-based imaging modality, although it has been around for some time, particularly in Europe. The most common of these labeled analogs are 68Ga-DOTATOC, 68Ga-DOTANOC and 68Ga-DOTATATE. They may be known collectively as ‘SSTR-PET’. Additionally, the DOTATATE version may often be referred to as NETSPOT in USA but technically that is just the commercial name for the radionuclide mix.
These peptides are easier and cheaper to synthesize than standard octreotide-analog based ligands, boast single time point image acquisition compared to 2 or 3 days with Octreoscan. Its superior spatial resolution derives from the fact that it measures the radiation from two photons coincidentally. SPECT, in comparison, measures the gamma radiation emitted from one photon directly. This results in different limitations of detection – millimeters for 68Ga-PET compared with 1 cm or more for SPECT. There are a few choices of ligands with this type of imaging, but the differences lie primarily in their SSTR affinities – all of the ligands bind with great affinity to SSTR2 and SSTR5. 68Ga-DOTANOC also binds to SSTR3. Despite these differences, no single 68Ga ligand has stood out as the clear choice for use in NETs. As with standard somatostatin receptor-based imaging, these 68Ga-PET studies are fused with CT to improve anatomic localization.
Comparison studies between 68Ga-PET and standard imaging techniques (CT, OctreoScan) have universally demonstrated the superiority of 68Ga-PET in detection of NET primary tumors and metastases. Two early studies compared 68Ga-DOTATOC to standard somatostatin imaging (SRS)-SPECT and CT. Buchmann et al. reported that 68Ga-DOTATOC detected more than 279 NET lesions in 27 patients with histologically proven NETs, whereas SRS-SPECT detected only 157. The greatest number of lesions were detected in the liver. 68Ga-DOTATOC found more than 152 hepatic lesions, while SRS-SPECT found only 105, resulting in a 66% concordance rate between the two modalities. The concordance for abdominal lymph nodes was worse at 40.1%. Cleary these advantages are going to impact on treatment plans, some needing to be altered. In addition, 68Ga-DOTA PET imaging can be used to determine which patients might benefit from use of Somatostatin Analogues (Octreotide/Lanreotide) and PRRT – you can read more about this integrated and potentially personalised treatment in my article on ‘Theranostics‘ – click here.
It’s worth pointing out that SSTR PET is replacing previous types of radionuclide scans, mainly Octreoscan (Indium 111) and is not replacing conventional imaging (CI) such as CT and MRI etc. Whilst SSTR-PET has demonstrated better sensitivity and specificity than CI and In-111, there are specific instances in which SSTR-PET is clearly preferred: at initial diagnosis, when selecting patients for PRRT, and for localization of unknown primaries. For patients in which the tumor is readily seen on CI, SSTR-PET is not needed for routine monitoring. The Journal of Nuclear Medicine has just published “Appropriate Use Criteria for Somatostatin Receptor PETImaging in Neuroendocrine Tumors” which gives guidance on it’s use – issued by the Society of Nuclear Medicine and Molecular Imaging (SNMMI).
Other PET Scans
18-Fluoro-Deoxy-Glucose PET (FDG PET) is used to detect malignancy for a variety of tumor types. Unfortunately, its utility has not been borne out in NETs, as the majority of NETs tend to be relatively metabolically inactive and fail to take up the tracer well. However, high-grade NETs are more likely to demonstrate avid uptake of 18FDG, giving these scans utility in identifying tumors likely to display more aggressive behavior.
The use of Fluoro-18-L-Dihydroxyphenylalanine (18F-FDOPA) in PET was developed in the 80’s for the visualisation of the dopaminergic system in patients with degenerative disorders, such as Parkinson’s Disease and related disorders. The ﬁrst publication on the use of 18F-FDOPA PET for brain imaging was in 1983, which was followed by many others on the use of 18F-FDOPA PET for the diagnosis of Parkinson’s disease. Years later, in 1999 the ﬁrst publication on the use of 18F-FDOPA PET for imaging of neuroendocrine tumour appeared. The value of 18F-FDOPA PET has now been proven for the diagnosis and staging of many neuroendocrine tumours, brain tumours and congenital hyperinsulinaemia of infants.
18F-FDOPA is accurate for studying well differentiated tumours. However the difficult and expensive synthesis have limited its clinical employment. It currently can be successfully used for imaging tumours with variable to low expression of somatostatin receptors (SSTR) such as Medullary Thyroid Carcinoma, Neuroblastoma, Pheochromocytoma), and others that cannot be accurately studied with Somatostatin SSTR scans such as the OctreoScan (Somatostatin Receptor Scintigraphy (SRS)), which uses the ligand 111In-DPTA-D-Phe-1-octreotide or the newer 68Ga DOTA-peptides. Read more about the use of 18F-FDOPA in ‘endocrine tumours’ here. Please bear in mind that more recent Ga68 PET studies may supersede some of the data mentioned. If in doubt ask your specialist.
Radioiodinated (123I) metaiodobenzylguanidine (MIBG) is an analog of norepinephrine that is used to image catecholamine-secreting NETs such as pheochromocytomas, paragangliomas and glomus tumors. It can also be used to look for Neuroblastoma in children. In patients with functional pheochromocytomas or paragangliomas, this modality has a sensitivity of 90% and positive predictive value of 100%. However, it has limited use in Gastrointestinal (GI) NETs, as this modality was positive in only 49.1% of patients. In the same cohort of patients, OctreoScan was positive in 91.2%. As an imaging tool, this study is best used to confirm a diagnosis of pheochromocytoma or paraganglioma and define the extent of metastatic disease in these tumors. (Note – the Ga68 PET is rising in prominence though). Its most practical use in GI NETs may be to determine whether patients with metastases may benefit from treatment with 131I-MIBG (a form of radiotherapy). Please bear in mind that more recent Ga68 PET studies may supersede some of the data mentioned. If in doubt ask your specialist.
Parathyroid Scan – Sestamibi
Sestamibi scanning is the preferred way in which to localize diseased parathyroid glands prior to an operation. This parathyroid scan was invented in the early 1990’s and now is widely available. Sestamibi is a small protein which is labeled with the radio-pharmaceutical technetium99 (Tc99m). This very mild and safe radioactive agent is injected into the veins of a patient with hyperparathyroidism (parathyroid disease) and is absorbed by the overactive parathyroid gland. Since normal parathyroid glands are inactive when there is high calcium in the bloodstream, they do not take up the radioactive particles. When a gamma camera is placed over the patient’s neck an accurate picture will show the overactive gland. Only the overactive parathyroid gland shows up…a very accurate test.
The Sestamibi scan will display the hyperactive gland which is causing hyperparathyroidism in about 90 percent (90% sensitivity) of all patients. If the Sestamibi does show the hyperactive gland it is almost always correct (98-100% specificity). It takes approximately two hours to perform the Sestamibi scan after it has been injected. Pictures of the neck and chest are usually taken immediately after the injection and again in 1.75 to 2.0 hours (shown above). Newer techniques allow for more complete two and three dimensional images to be obtained of a patient’s neck. This technique is called SPECT scanning (Single Proton Emission Computerized Tomography) but it is usually not necessary.
Skeletal Scintigraphy (bone scan)
Quite often, bone metastases in NETs will be found via conventional imaging or special to NET nuclear scans such as Ga68 PET or MIBG. However, a bone scan can often find them or confirm findings of scans looking for NETs.
Skeletal scintigraphy is a special type of nuclear medicine procedure that uses small amounts of radioactive material to diagnose and assess the severity of a variety of bone diseases and conditions, including fractures, infection, and cancer.
Nuclear medicine imaging procedures are non-invasive and — with the exception of intravenous injections — usually painless medical tests that help physicians diagnose and evaluate medical conditions. These imaging scans use radioactive materials called radiopharmaceuticals or radiotracers. Radioactive energy emitted from the radiotracer is detected by a special camera or imaging device that produces pictures of the bones called scintigrams. Abnormalities are indicated by areas of abnormal bone that take up more or less of the radiopharmaceutical which appear brighter or darker than normal bone on the scintigram.
Because nuclear medicine procedures are able to image the functions of the body at the molecular level, they offer the potential to identify disease in its earliest stages as well as a patient’s response to therapeutic interventions. In fact, a bone scan can often find bone abnormalities much earlier than a regular x-ray exam.
Taking the camera inside and directly to the Tumour
Of course there are other ways to “see it” via several types of Endoscopy procedures – taking the camera to the tumour. Read my article about this by clicking here
A look to the future of PET Scans
Just imagine something which is 40 times better than current PET scan technology? That’s what the scientists are working on now. Here’s an example called “EXPLORER“. Clearly there are more answers required in order to see if this is suitable for use with NETs (i.e. will it work with our radionuclide tracers etc) but it is very exciting and like something out of Star Trek. A little bit of me is worried about ‘overdiagnosis’ so interpretation of something that detailed will be very important to avoid unnecessary worry. Read more here and there is a later update here. Check out this cool video of the 3D images:
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Scanning is a key diagnostic and surveillance tool for any cancer. Even though you have elevated bloods or urine (….or not), a picture of your insides is really like a thousand words…. and each picture has a story behind it. Scanning can be a game changer in the hunt for tumours and although scans can’t (yet) confirm the cancer type and grade, they certainly help with that piece of detective work and are key in the staging of the cancer.
When I read stories of people in a difficult diagnosis, I always find myself saying ‘a scan might resolve this’ and I always suggest people should try to get one. Even in the case of a story about late diagnosis or a misdiagnosis, I find myself thinking ‘if only they had done a scan earlier’. Despite what you read on NET forums, a CT scan will normally find some evidence of most tumour activity.
However, scans are not an exact science…..not yet! Apart from human error, sometimes tumours are too small to see and/or there are issues with ‘pickup’ (i.e. with NETs, nuclear scans need efficient somatostatin receptors). However, technology is improving all the time and you can read about this in my blog Neuroendocrine Cancer – Exciting times Ahead.
As for my own experience, I was very lucky. I managed to get a referral to a specialist early on in my diagnosis phase. He looked at the referral notes and said “what are you doing this afternoon”. I replied “whatever you want me to do”. He wanted to scan me. He didn’t know I had cancer but his instincts led him to believe he needed to see inside my body. The scan results were pretty clear – I had a metastatic Cancer and further checks were now needed to ascertain exactly what it was. So I took my seat on the rollercoaster. Here’s something I always say I believe is so much better than the impractical early diagnosis messages that seem to pervade our community: If your doctors don’t suspect something, they won’t detect anything and I believe this is a very frequent outcome of many diagnoses for many cancers (not just NETs).
There’s frequent discussion about the best types of scans for different types of NETs and even for different parts of the anatomy. This is correct and there’s also different views on the subject (including in the medical community), However, a few well known facts that can be gleaned from authortative NET sources. I found this useful video summary from the NET Patient Foundation describing the different scans for NET Cancer and what to expect. Worth a look.
Sooner we can all get access to the latest radionuclide scans the better!
When I was diagnosed with metastatic Neuroendocrine Cancer on 26 July 2010, I just wanted them to hurry up and fix my body so I could get back to normal. My expectations of speed turned out to be wildly inaccurate and in hindsight, I was also wildly naive. You see, with Neuroendocrine Cancer, particularly well-differentiated, low or medium grade tumours, it sometimes doesn’t work as fast as you would think and there are good reasons for that.
The complexity of the condition needs some consideration as the physicians work up a treatment plan. I’m quite happy and content they took their time, rather than rush into the wrong decisions. If you think about it, this is an advantage with low and medium grade NETs……you normally have some time to get the ducks in a line (and there are some pretty important ducks in that line).
Here’s a very short video discussing this during a patient video shoot: Click here.
I had a confirmed biopsy result following some incidental CT scans and other tests. However, they now needed further checks and marker tests to work out the extent of the disease. So the timeline leading up to major surgery ended up like this:
Diagnosis: 26 July 2010. Met with Oncologist. Grade 2 Small Intestine NET with distant metastasis (Stage 4)
Chromogranin A and 5HIAA: submitted 28 July. Results received 13 August – both elevated, indicating and confirming tumour bulk and function status respectively
Octreotide Scan:17-19 August. Report issued 24 August – confirmed CT plus additional distant hotshots. Also confirmed my tumour receptors were avid to somatostatin analogues.
Daily Octreotide Injections: Started 9 September to control syndrome (derisk surgery)
Referred to NET Multi-Disciplinary Team (MDT): 15 September – they now had sufficient data to form a treatment plan.
Holiday: Late September (it was booked and I felt OK, why not!)
Further MDT assessment and consultations: 1- 7 October
Bland Liver Embolisation: 19 October
First Surgery: 9 November – to remove primary and debulk local and regional spread.
You can read the rest of my treatment background here.
So it took 75 days from diagnosis to opening me up to remove the first batch of tumours. With reasonably slow-growing tumours, that isn’t really a long time when you consider they had probably been growing inside me for several years. I’m sure others waited even longer.
Sometimes rushing straight into the operating theatre isn’t really the best option. I’m still here!
There’s a saying that the patient is the most underused person in healthcare and I think there’s a lot of truth in that. However, I would suggest with Neuroendocrine Cancer, it’s less true than for many other cancers. There are so many NET Cancer patients out there who know quite a lot about their cancer, and in some detail. Even the great Dr Liu once said that NET Patients frequently know more about NET Cancer than their doctors.
If you go onto Twitter, if you go onto Facebook, if you read newspaper stories, you will find cancer patient stories in abundance and they will normally be patients diagnosed with the big 4 cancers. This is not surprising as these tend to affect more people. However, the ratio of NET Cancer patient stories still does not seem to be right. I’m not ‘dissing’ breast, lung, bowel and prostate cancer patients, all credit to them for pushing their cancer awareness – respect!
I truly believe that patient stories, whether they are written, presented live or recorded for mass media, are an extremely valuable tool in spreading awareness of NET Cancer. A ‘human being’ talking is a thousand times more potent than the endless stream of ‘memes’ and cartoons that seem to pervade our community – one reason why I don’t use them on my own site. It’s also the reason why I always jump at the opportunity to tell my story, because it’s real, it’s factual and I’m sensing an increasing willingness from the medical and healthcare communities to use patients in this way. Quite right too, patients have a lot to offer.
I’ve been video’d several times in the past 12 months and one day you might actually get to see those, there are some contractual reasons why I cannot yet share them with you. It’s quite a scary thing to do and I found it mentally exhausting – but very worthwhile.
I was therefore delighted to find this recently published group of videos from Cure Connect. Within the clips, there are 2 patients stories, one Pancreatic NET (pNET) and one Carcinoid and they are interspersed and integrated by input from NET specialist Dr. Reidy-Lagunes (a very knowledgeable and enthusiastic speaker). Each clip is only around 5 minutes long so not too taxing. The pNET patient, Michael, is a great supporter of my blog and one of the first NET patients I met on twitter. I’m very thankful to him for alerting me to the videos. Dr Reidy-Lagunes is fast becoming a ‘fav’ of mine and I note she emphasises some of the things I’ve been consistently saying in my blogs; i.e. this cancer can be treated and it’s not as rare as people think.
Another bonus is the addition of Carcinoid Cancer Foundation (CCF) and my friend Grace Goldstein. CCF is the largest and most respected NET Cancer organisation on the planet and Grace works tirelessly to spread awareness and help patients including me! CCF was the first site I found and remains my go-to site today.
Well done Michael and Brenda. Thanks also to Dr. Reidy-Lagunes, Grace Goldstein/CCF and Cure Connect for once again highlighting our cancer.
As it’s Testicular Cancer Awareness Month, I thought I’d share a personal story with you. This is something regarding my own diagnosis and something as yet unpublished. I don’t tend to share some very personal stuff but this is on the boundary of that rule and there are some important messages to be teased out. For those who follow my blog in detail, you will remember the post entitled “Neuroendocrine Cancer – Signs, Suspicions, Symptoms, Syndromes, Side-Effects, Secondary Illnesses, Comorbidities, and Coincidences”. As you can see from the title, I got hooked on a bunch of synonyms (small s) that represent the difficulty in sorting out what can be attributed to Neuroendocrine Tumours (NETs) and what might be something else. You’ll note they all begin with the letter ‘S’ except ‘Comorbidities’ and ‘Coincidences’. These 2 were actually retrospective add-ons to the blog title and there is a potential overlap between both.
Life is full of coincidences and I’m certain this is also the case with issues NET patients have from time to time. There is a high possibility that some things which were going to happen health-wise before NETs came along, will most likely still happen and it can often seem like the NETs have some causal effect. As my friend Dr Eric Liu says ‘Even NET patients get regular illnesses’.
I also suspect the same thing can happen pre-diagnosis and if you’re unlucky, during the diagnostic phase. This sort of event has the potential to confuse an already confusing diagnosis! So here’s a story about my ‘COINCIDENCE’ which eventually turned out to be a ‘COMORBIDITY’.
At the beginning of 2010 (remembering my diagnosis was July that year), I did what all men should regularly do – I checked my ‘chaps’ for lumps. Sometime in January, I got the feeling my left ‘chap’ was bigger than the right and I monitored that for a few days. Eventually, it was patently obvious there was an abnormality. I immediately went to my GP and he diagnosed a hydrocele. Apparently these are quite common with men. He was able to quickly work this out by shining a torch through the offending gonad area and as the light came out the other side, this was confirmation it was excess fluid. He said it might go away on its own but explained there were medical procedures to correct it including fine needle aspiration (not normally a permanent fix) or surgical repair (the most permanent fix). I left it for a few weeks and as time passed, the size of my left ‘chap’ increased. It became really uncomfortable and painful so I asked to be referred to a specialist. Bear in mind at this point, I still didn’t know I had Neuroendocrine Tumours burrowing away inside me for years.
Fast forward 1 month, the hydrocele is not yet sorted and I’m speaking to a specialist having been referred for a low hemoglobin score (the trigger for my NET diagnosis). At this point, I’m convinced there is a connection and amongst the plethora of tests and checks, the specialist also carried out a fine needle aspiration of my left ‘chap’ (I can hear the male audience wincing). The fluid was sent off for testing and subsequently returned negative. My left ‘chap’ was now back to normal (every cloud…..). By the way, the hydrocele returned around 2 months later. I eventually got the date for my hydrocele surgical procedure (hydrocelectomy) but decided to postpone it to sort out another little matter …… Cancer!
I eventually got it repaired in Sep 2011 after 14 months of NET treatment and had no issues since. Now…… I can almost hear the cogs turning …… the testes are an endocrine organ etc. I’ve been through this too and I was still suspicious for a year after diagnosis. However, I’ve been categorically told there is no connection and there is nothing showing on ultrasound, CT scan or Octreoscan. 4.5 years later, I’m happy there was no connection 🙂
However, I did my duty, I checked my chaps, found an issue and fortunately it was nothing too serious. Crap timing though!
I’ve mentioned ‘luck’ a few times in the past month following some more ‘cancerversary’ milestones – these tend to make me reflect on my experience. Even though I was metastatic at diagnosis, I think of myself as lucky on the basis that my tumours were found by ‘chance’, or to be more accurate, found following an innocuous set of circumstances. Click here to hear me talk about my diagnosis.
As we know, Neuroendocrine Cancer can sometimes be very difficult to discover and diagnose. However, sometimes with a bit of luck or a chance event, it can be intercepted and can then often lead to a much better outlook for the person concerned. But sometimes there is also a cost and I don’t just mean financial (although that is also a very real problem). Despite me thinking I had been lucky, the ‘little suckers’ had burrowed their way into many places and I now deal with those consequences following significant treatment to get rid of as many as possible.
With my blogging and advocate activity, I get to hear other people’s stories, some of which have tweaked my emotions from ‘man style leaky eyes’ to wide-eyed surprise and astonishment, but very occasionally with smiles. I had one such exchange with Mary who subsequently agreed to let me use her story which immediately caught my eye because it not only triggered a wide range of emotions, but made me reflect on the cost aspect I described above.
Mary’s is a lung NET patient and her tumour was caught early. Although it was a totally chance discovery, it was in really unfortunate circumstances. Her brother Dan was fighting leukaemia and needed a life saving stem cell transplant. During the checks for her suitability as a donor, the lung NET was discovered. Clearly a very worrying time for Mary as she had gone to the hospital to try to save her brother’s life and ended up being admitted with her own cancer diagnosis and was therefore unable to take any part in the donation. I cannot begin to imagine how that felt for the whole family. Fortunately Mary’s sister was also found suitable and was able to donate, Their brother later had a successful transplant but unfortunately his cancer recurred and he passed away a short while later.
That’s an amazing albeit sad story but it invokes a wide range of emotions. It’s also a very inspiring story about a family coming together in time of crisis. Mary went to hospital that day to try to save Dan’s life and despite her own diagnosis, she still felt guilty that she was unable to fulfil that task. However, before his passing, Dan let it be known that he must have gotten sick to save her life. That’s a heart-warming thought – RIP Dan.
I’m very thankful to Mary who agreed to let me publish her story here. It was actually featured a couple of years ago in their local newspaper – you can read it here – <Click here>
I’d love to hear from others who had a lucky or chance tumour find.
I talk often about my diagnosis but not about an ‘incident’ which occurred almost immediately prior to being formally told.
I was well into the ‘diagnostic phase’, having had all sorts of tests including a liver biopsy. I vividly remember thinking these tests were a ‘nuisance’, I was far too busy and I didn’t even feel ill. In hindsight, I was fortunate to have had such a thorough bunch of physicians who diagnosed me with metastatic Neuroendocrine Cancer in about 6 weeks ‘flash to bang’. I intentionally use a phrase associated with ‘quick’ because in the world of Neuroendocrine Cancer, 6 weeks is ‘warp speed’.
So why was I admitted to hospital during the diagnostic phase? Because I was stupid. In fact I was double-stupid. Firstly, despite having had to undergo a liver biopsy and a referral to an Oncologist, I was in a dismissive frame of mind and was blanking out any thought that I actually had cancer. I didn’t have time for it, I was far too busy. I’m in control! Secondly, despite being told to take it easy after the liver biopsy, I ignored that advice because I was far too busy getting on with a normal life. After all, this is just another test hurdle and I’ll get the all clear. Other people get Cancer but not me.
On the weekend following the liver biopsy, the family came round, so I decided to do normal things like lifting one of my grandsons up (as one does) and I prepared the BBQ which involved lifting a 13.5kg cannister of gas from the garage onto the patio. Why not? I didn’t have anything wrong with me and I didn’t even feel ill.
However, as that Saturday afternoon progressed so did the pain; and to the point that I knew I had to seek help. To cut a long story short, I was eventually admitted to hospital for what was to be diagnosed as a bleed on my liver at the biopsy site. Oh how the mighty fall.
On the positive side, I got another bunch of tests including scans as confirmation (….a second opinion from a different hospital). However, it was the wake-up call I needed to take it seriously. I was discharged on the Monday in time for my very first Oncology appointment with my wife Chris in attendance. For the first time, we were officially told I had Cancer – it was much more than just a ‘scare’. For me, the denial was over, indicating that I was never actually in control of what was happening to me.
Finally some food for thought …… In hindsight, I made the serious mistake of not talking to anyone about my denial and I suspect that led to me acting stupidly.
It really is OK to talk about Cancer
p.s. I’m now slightly mellower about Cancer 🙂 You might say I’m back in control?
Last year I wrote a series of blogs on the ‘coping’ side of cancer, one of which was about still being able to have a laugh. This was my way of saying no matter how tough life is, you need to stay positive and maintain your sense of humour. When I think back to some of the treatments I’ve had, I sometimes have a little laugh even although I wasn’t laughing at the time! My favourite ‘treatment laugh’ is the ‘suppository story’ which occurred in hospital shortly after my first major surgery – it wasn’t funny at the time but I smile when I think back to it. On a similar subject, I had a colonoscopy around 21 months prior to my actual NET Cancer diagnosis. Like the guy in the story below, I don’t remember a thing. However, what I do vividly remember (and clearly so did he!), is that the preparation for the procedure can be a ‘challenge’. I can vouch for that.
I came across this real but anonymised journal which you may enjoy and hopefully have a little laugh too. I suspect those who have had a colonoscopy (or two) will enjoy it more than others! I suddenly realised colonoscopies can be funny on the basis I laughed out loud reading this. The quotes from doctors at the end are hilarious!
I called my friend Axxx, a gastroenterologist, to make an appointment for a colonoscopy. A few days later, in his office, Axxx showed me a color diagram of the colon, a lengthy organ that appears to go all over the place, at one point passing briefly through Minneapolis. Then Axxx explained the colonoscopy procedure to me in a thorough, reassuring and patient manner. I nodded thoughtfully, but I didn’t really hear anything he said, because my brain was shrieking, ‘HE’S GOING TO STICK A TUBE 17,000 FEET UP YOUR BEHIND!’
I left Axxx’s office with some written instructions, and a prescription for a product called ‘MoviPrep,’ which comes in a box large enough to hold a microwave oven. I will discuss MoviPrep in detail later; for now suffice it to say that we must never allow it to fall into the hands of America’s enemies.
I spent the next several days productively sitting around being nervous. Then, on the day before my colonoscopy, I began my preparation. In accordance with my instructions, I didn’t eat any solid food that day; all I had was chicken broth, which is basically water, only with less flavor. Then, in the evening, I took the MoviPrep. You mix two packets of powder together in a one-liter plastic jug, then you fill it with lukewarm water. (For those unfamiliar with the metric system, a liter is about 32 gallons). Then you have to drink the whole jug. This takes about an hour, because MoviPrep tastes – and here I am being kind – like a mixture of goat spit and urinal cleanser, with just a hint of lemon.
The instructions for MoviPrep, clearly written by somebody with a great sense of humor, state that after you drink it, ‘a loose, watery bowel movement may result.’ This is kind of like saying that after you jump off your roof, you may experience contact with the ground. MoviPrep is a nuclear laxative. I don’t want to be too graphic, here, but, have you ever seen a space-shuttle launch? This is pretty much the MoviPrep experience, with you as the shuttle. There are times when you wish the commode had a seat belt. You spend several hours pretty much confined to the bathroom, spurting violently. You eliminate everything. And then, when you figure you must be totally empty, you have to drink another liter of MoviPrep, at which point, as far as I can tell, your bowels travel into the future and start eliminating food that you have not even eaten yet.
After an action-packed evening, I finally got to sleep. The next morning my wife drove me to the clinic. I was very nervous. Not only was I worried about the procedure, but I had been experiencing occasional return bouts of MoviPrep spurtage. I was thinking, ‘What if I spurt on Axxx?’ How do you apologize to a friend for something like that? Flowers would not be enough.
At the clinic I had to sign many forms acknowledging that I understood and totally agreed with whatever the heck the forms said. Then they led me to a room full of other colonoscopy people, where I went inside a little curtained space and took off my clothes and put on one of those hospital garments designed by sadist perverts, the kind that, when you put it on, makes you feel even more naked than when you are actually naked.
Then a nurse named Exxxx put a little needle in a vein in my left hand. Ordinarily I would have fainted, but Exxxx was very good, and I was already lying down. Exxxx also told me that some people put vodka in their MoviPrep. At first I was ticked off that I hadn’t thought of this, but then I pondered what would happen if you got yourself too tipsy to make it to the bathroom, so you were staggering around in full Fire Hose Mode. You would have no choice but to burn your house down. When everything was ready, Exxxx wheeled me into the procedure room, where Axxxx was waiting with a nurse and an anesthesiologist. I did not see the 17,000-foot tube, but I knew Axxx had it hidden around there somewhere. I was seriously nervous at this point. Axxxx had me roll over on my left side, and the anesthesiologist began hooking something up to the needle in my hand. There was music playing in the room, and I realized that the song was ‘Dancing Queen’ by ABBA. I remarked to Axxx that, of all the songs that could be playing during this particular procedure, ‘Dancing Queen’ had to be the least appropriate. ‘You want me to turn it up?’ said Axxx, from somewhere behind me. ‘Ha ha,’ I said. And then it was time, the moment I had been dreading for more than a decade. If you are squeamish, prepare yourself, because I am going to tell you, in explicit detail, exactly what it was like……………
I have no idea. Really. I slept through it. One moment, ABBA was yelling ‘Dancing Queen, feel the beat of the tambourine,’ and the next moment, I was back in the other room, waking up in a very mellow mood. Axxx was looking down at me and asking me how I felt. I felt excellent. I felt even more excellent when Axxx told me that It was all over, and that my colon had passed with flying colors. I have never been prouder of an internal organ.
On the subject of Colonoscopies……..
Colonoscopies are no joke, but these comments during the exam were quite humorous!!!!! A physician claimed that the following are actual comments made by his patients (predominately male) while he was performing their colonoscopies:
1. ‘Take it easy, Doc. You’re boldly going where no man has gone before!’ 2. ‘Find Amelia Earhart yet?’ 3. ‘Can you hear me NOW?’ 4. ‘Are we there yet? Are we there yet? Are we there yet?’ 5. ‘You know, in Arkansas, we’re now legally married.’ 6. ‘Any sign of the trapped miners, Chief?’ 7. ‘You put your left hand in, you take your left hand out…’ 8. ‘Hey! Now I know how a Muppet feels!’ 9. ‘If your hand doesn’t fit, you must quit!’ 10. ‘Hey Doc, let me know if you find my dignity.’ 11. ‘You used to be an executive at Enron, didn’t you?’
And the best one of all:
12. ‘Could you write a note for my wife saying that my head is not up there’
thanks for listening
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The sooner any cancer can be correctly diagnosed, the better chances of a curative scenario for the person concerned. However, some cancers are in the ‘difficult to diagnose’ category. Neuroendocrine Tumours (NETs) are in this category due to the vague symptoms which may be mistaken for other diseases and routine illnesses. This is one of the reasons there have been many lengthy diagnostic delays. In many cases, it can be very quiet leading to incidental diagnosis at an advanced stage. It’s SNEAKY!
In some cases it can be a little bit noisy. For example, some of the most common misdiagnoses appears to be Irritable Bowel Syndrome (IBS), asthma, or menopause. Patients complain of abdominal pain, wheezing, shortness of breath, diarrhea, flushing, palpitations and a whole host of other minor issues. There are even extreme cases where patients have been told they may have a mental illness following constant visits to their local doctors. These cases can lead to months or even years of delay from the onset of symptoms, with around half of patients diagnosed at an advanced stage of disease and metastases at initial presentation.
I’m not totally up to speed on what happens overseas, but in the UK, you normally only have 10 minutes (5 in some scenarios) to see a doctor (although my own experience is that many ignore that rule and if they sense a big issue). No matter how hard we push, this can sometimes be insufficient to diagnose or even suspect NET cancer (or indeed many other diseases).
A NET Cancer diagnosis is more likely to be as a result of a number of visits where a picture can emerge and even then, a referral to a ‘symptom’ specialist may not necessary lead to an immediate discovery of a NET cancer. In fact, a ‘nothing serious found’ referral back from secondary care/specialist to a person’s GP/PCP is not uncommon.
There is no harm in targeting GP/PCP but they are more likely to be following instincts by referring due to symptoms rather than in the game of diagnosing obscure cancers which look like other things. I’m a big believer in primary care being the trigger for ‘something‘ which will hopefully be figured out at secondary care where you are more likely to get access to scans etc. There is data to suggest that ‘symptom specialists’ frequently refer patients back to primary care, indicating the education at secondary care level may be a better focus for NET awareness. NETs are dangerous despite the normally indolent course. It has a propensity to metastasize meaning that the chance of a curative scenario is vastly decreased for many. It can kill if left untreated.
One of the most discussed and debated Cancer issues is late diagnosis. Cyberspace is full of disturbing stories and many different cancers are involved. Some cancers are much more difficult to diagnose than others and this increases the need for more awareness and education campaigns.
Under-diagnosed or Under-reported?
Like many other Cancers, Neuroendocrine Cancer (known as Neuroendocrine Tumors or NETs) is one of a number of ‘difficult to diagnose’ conditions with some of its variants more difficult than others. It’s a less common form of cancer but with a fast rising incidence rate, possibly the fastest rising incidence rate of all cancers. In fact, its fast rising incidence rate has been a positive in some ways, contributing to awareness and the introduction of new treatments. In some respects, the incidence rate increase is due to people knowing more about it (…… particularly medical staff), diagnostic tools have improved; and critically …….. the correct ICD codes are being applied by physicians to enable accurate cancer incidence data (although this is probably still far from being 100% accurate in favour of NETs). In short, it’s been under-diagnosed and under-reported for decades.
Consequently, it’s not as ‘rare’ as we have been consistently told. For example, in UK, the latest figures from Public Health England indicate an annual incidence rate of 9/100,000 – to put that into perspective, one patient every 2 hours and above the rare threshold of 5/100,000. In USA, the latest SEER database figures indicate that the prevalence figure extrapolated to 2017 has accelerated beyond 200,000 (i.e. not rare). Eminent NET specialists in USA are saying it’s not rare and one centre is even suggesting there are 200,000 undiagnosed cases in that country, also adding that the autopsy rate of (so-called) ‘carcinoid‘ finds is four times higher than the documented diagnosis figure.
Is patient and patient advocate organisation reported data accurate?
Statistics indicate that many patients are initially diagnosed with something else and it occasionally takes some time to be formally diagnosed with NETs. However, it’s wrong to suggest this happens to most NET patients. I’ve heard many stories from many people who have had a speedy diagnosis, even those where the cancer had advanced silently to a metastatic stage (I myself am in that category). Unfortunately these don’t tend to be mentioned a lot on social media and they don’t tend to be the people who complete questionnaires for NET patient surveys. Another oddity in survey data is that you can still see several specialists within a short period of time and have an excellent diagnostic experience – it’s the time that is important not the number of people you see in between. It’s also a myth to suggest that you only need to see one doctor to be diagnosed with any cancer. The fourth person I saw officially told me I had cancer but the gap was only 2 months (half of that was my own procrastination). This happens with many cancers, NETs is not special in this regard.
The problem with some of the NET Cancer survey statistics is that the reach is nearly always drawn from a limited audience and therefore the data can be skewed, particularly when the target collection is in the main from patient forums or groups where the ratio of problematic diagnoses is high. Offering these patients a ‘platform’ disguised as a survey is like ‘situating the appreciation’. On certain forums, it can be like pushing at an open door. This is why I currently have little faith in NET patient surveys. It’s a difficult area but we need a new model for capturing the whole spectrum of patient opinion. One positive statement from the recent SEER database study mentioned above…… the increase in incidence is partly due to earlier diagnosis. Clearly there’s more work to be done but it helps to dispel the myth that every NET patient was misdiagnosed for years. No medical corroboration is done, i.e. if a patient says they were misdiagosed, that doesn’t mean that’s an accurate statement from a medical perspective. I have it on good authority that some people who were diagnosed with IBS actually did have IBS, but it was masking the NET, the same is probably true for other symptoms/conditions. PCP/GP guidelines for diagnosing IBS clearly need updating.
Sure, some people will be misdiagnosed and that is no different to many difficult to diagnose conditions. But to suggest this is the most common outcome is way off beam. There are thousands of incidental diagnoses of NETs when checking for other issues – these don’t make good headlines though thus why we don’t hear much about it. Only a very small percentage of NET patients are on forums so it’s not a good measure of how many people got a quick diagnosis vs the opposite. Only a very small percentage of people are on the mailing lists for NET Advocate organisations, so in reaction to patient surveys put out by NET advocate organisations, who will also use patient forums to disseminate, neither are those a good measure of who got a quick diagnosis and the opposite. Ergo, NET patients surveys are always wrong.
So how did I fare with my own diagnosis? I’ve always thought myself luckier than many. I suspect the best I could have hoped for was diagnosis about 20 months prior to ‘D-Day in July 2010. My problem at the time was so vague that I could see myself there wasn’t much justification for expensive tests (i.e. scans). Moreover, by the time I got to see a specialist in 2008, the problem had settled and I was content. Even my second referral to specialists in May 2010 was random as I hadn’t initially intended to say I’d lost a ‘few pounds’ in weight whilst at a routine clinic. Fortunately, I had a pretty thorough and professional nurse who made me have a blood test ‘just to be sure’. My GP immediately referred me to a specialist. The referral specialist was pretty much on the ball. He was looking at a (then) 55-year-old fit and healthy looking male presenting with low haemoglobin – boom! CT scans, ultrasounds, blood tests, the works – except he could not pin down the exact cancer type until I mentioned facial flushing. Eureka, he knew and correctly predicted the results of the forthcoming liver biopsy. It must be said that even if he didn’t know or hadn’t heard of Neuroendocrine Cancer, the biopsy was key to finding out but the scan was the trigger. However, the damage was done and I now live with an incurable metastatic cancer. And I believe the damage was done back at my first issue in 2008. Despite this, I still feel lucky because I’m not dead. I do often wonder what would have happened had I not had that initial blood test. Check out this video of me explaining my diagnosis.
Luck plays a part, so does patient intransigence.
Sometimes with Cancer, you need a bit of luck and I never really think of my diagnosis as late, just unlucky to begin with (not helped by my own indifference to illness) and then geographically lucky as the diagnostic ball starting rolling. It had silently metastasized and perhaps that’s just how the cookie crumbles with silent illnesses in the absence of a whole population screening programme. I’m fairly certain future detection and screening will help find most cancers/conditions earlier as technology and science progresses – but we are not there yet.
I am contacted almost daily from someone who is experiencing flushing and diarrhea but they have not been diagnosed with NETs. Many are quite up to date on the condition but lack any clinical evidence of NETs. It’s not cancer is not really a diagnosis so I feel for these guys who just need a diagnosis of something. If you are reading this and think you may have NETs, read this articlewhere I offer you some advice.
You don’t actually need to be a NET specialist to trigger a diagnosis of a NET
I was helped by three other things:
The nurse who sent me for a ‘just to be sure‘ blood test was not a NET expert but she was doing her job in a thorough manner and triggered my eventual diagnosis.
The GP was not a NET expert but he analysed the blood results, considered my healthy outlook; and then used his instincts and training to send me to a specialist (i.e. he was suspicious of ‘something’).
The investigating specialist was aware of NET Cancer but although he knew I had cancer, he was not suspecting NETs until I said I was having occasional flushing (something I hadn’t mentioned to the nurse or the GP). But he was suspecting ‘something’ and in the end, he did detectsomething through scans and then confirmed it through a biopsy.
Should we expect every single doctor wherever they are, and whatever their experience, to be able to diagnose a NET at first visit?
‘If you don’t suspect it – you won’t detect it’ is a great NET cliché – but simply not practical to expect at primary care and IMHO almost impossible. There are 200 different cancer types and some have a bunch of sub-types. And at primary care level, you can add another 10,000 non-cancer conditions. It’s impossible for anyone to know everything about every single condition, let alone every single cancer BUT ……. a referral for something else can very often be a trigger for a diagnosis of Cancer. In fact, I suspect this is a very frequent scenario which often fails to make the patient survey data. A picture of your insides is key, regardless of what your physician is suspecting. If you can see it, you can normally detect it.
In short, you don’t really need to suspect NETs to detect it. Awareness is really important but it needs to be realistic to be taken seriously.
……if your Doctors don’t suspect something, they won’t detect anything!