Judy Golz is a retired registered nurse — she’s also a neuroendocrine cancer survivor. Like many nurses who get cancer, their experience in working in the healthcare industry possibly helps get a quicker diagnosis, possibly because they can recognise symptoms and likely differential diagnoses and it possibly helps knowing how the healthcare system works.
But with uncommon and complex diseases, it’s not always that straightforward, even for a nurse or any other healthcare professional. But Judy makes an excellent point about the quantity and quality of medical information now out there, including for Neuroendocrine Cancer and suggested she would be in a better place for self-diagnosis today.
Two things spring to mind about this story. Neuroendocrine Cancer is such a complex disease, it can often be hard for healthcare professionals to diagnose themselves. Secondly, since my diagnosis in 2010, there is no doubt a lot more information out there and I’d like to think I’ve played my part in that.
Sometimes, a blog post comes along and it just resonates! I got chatting with the author who has given me permission to post it here. Shari Berman is a two-time cancer survivor. She was diagnosed with Hodgkin’s Lymphoma at age 25, days after returning from her honeymoon and a second time with breast cancer 8 years later. I posted her full CV below.
Her post “25 Life Lessons From a Two-Time Cancer Survivor” is a fantastic summary of a positive approach to life, despite a cancer diagnosis (or in Shari’s case, two). I’ve seen some similar quotes before but Shari has collated them into one very powerful list. I’m not suggesting they all apply to everyone but perhaps even a ‘pick n mix’ approach would be useful. For example, I couldn’t do number 8 – they make me sneeze! (sorry Shari!)
The first 10 are here, you can see the others by linking below:
1. Life is short. Don’t wait for the perfect moment to DO or SAY something important to you.
2. Death is not failure. It is part of life and the more we accept that fact the more we can live intentionally and without as much fear.
3. A simple act of kindness can make someone’s day and leave a lasting impression.
4. You have the ability to make an impact. Leave your mark. Share your experience, your knowledge with others.
5. “Hope” is powerful.
6. If you don’t know what to say to someone in crisis, try saying “I don’t know what to say. But I want you to know I am thinking of you.”
7. You are stronger than you think. Trust me, you are.
8. Life is better with a dog
9. Trust your gut. Intuition is powerful.
10. It is important to take a “time-out”. Walking clears your head.
Read the remainder here, you won’t be disappointed. CLICK HERE to continue
Read Shari’s CV here:
Shari graduated from Cornell University and worked as a Human Resources Manager in the financial services industry for 15 years. After losing her mother to lung cancer she decided to spend her time advocating for patients and lending her perspective and expertise as patient, long-term survivor, caregiver and business professional.
Shari is a former co-chair of the Dana Farber Adult Patient Family Advisory Council where she effectively led an effort to restructure the Council and strengthen its role within the institution. She also served on Dana Farber’s quality committees and is currently a member of the Quality and Patient Safety Committee of the Massachusetts Board of Registration in Medicine.
Shari has spoken on behalf of Dana Farber at many fund-raising events and presentations on survivorship and patient care. Her talks have focused on a variety of topics including her personal experience with cancer, survivorship issues, patient centered care and how to develop and sustain an effective Patient and Family Advisory Council. Shari has also been asked to consult on a variety of projects with organizations such as the National Academies of Medicine, (formally Institute of Medicine), American Society of Clinical Oncology (ASCO), The Journal of American Medical Association (JAMA) and Livestrong.
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.
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.
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).
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).
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.
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:
Please Share this post for Neuroendocrine Cancer awareness and to help another patient
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!
For the first few years after my diagnosis, I avoided using the word ‘survivor’ in relation to my incurable cancer. It just didn’t seem to sit right despite the fact I’m a ‘glass half full’ kind of guy.
However …….. I was studying the term ‘Survivorship’ and found it also applies to those living with incurable and long term cancer. This piece of research totally changed my thinking. The slides above were provided by National Cancer Survivors Day (which seems to have turned rather international) – well done NCSD.Org – you should check out the site and sign up for their newsletter
What is ‘Survivorship’?
The definition differs slightly between national cancer advocate organisations but it would appear it also means “Living with, through, and beyond cancer“ which is a UK term. According to these definitions, cancer survivorship begins at diagnosis and includes people who continue to have treatment over the long-term, to either reduce the risk of recurrence or to manage chronic disease. It follows that those with incurable Cancers such as my own (Neuroendocrine) should be included under the term ‘Survivorship’.
Times are changing
I think it’s useful to look at overall statistics for survivorship to contextualise why the word ‘survivor’ might actually be more apt than it was 20 years ago. For example, in the UK, more than one in three people (35%) of those people who die having had a cancer diagnosis will now die from other causes. This is up from one in five (21%) 20 years ago. By 2020 this will improve further to almost four in 10 people (38%). This means the number of people who get cancer but die from another cause will have doubled over the past 20 years. I’m seeing similar reports from USA too where, for example, the number of cancer survivors is predicted to rise by a third by 2026 (15 million to 20 million). Almost half of the current survivors are aged 70+. In another example, the US National Cancer Institute (NCI) is predicting:
‘Silver Tsunami’ of Cancer Survivors in the next quarter century (read here).
There is also a very recent article which confirms this thinking, let me quote a bit of it: The cancer death rate has dropped by 23 percent since 1991, with some even larger gains in types of cancer that used to be extremely lethal. This means there are more and more patients like Thornton who are neither dying from cancer nor defeating it entirely. Instead, they’re learning to live with it. Full article here.
Of course, it must not be forgotten that many people will need support to maintain a decent quality of life and be supported with ongoing and long-term treatment. These are both challenges global health systems need to face with rising cancer prevalence. And both of these challenges will greatly affect my own type of cancer – Neuroendocrine. This is why I’m a very strong advocate for more focus on some of these support type issues and unmet needs, and the resources to deliver them.
So does that mean I’m a Survivor?
My research indicates this can be a very individual thing. I guess ‘survivor’ does not appeal to all people who simply have a ‘history of cancer’; and most likely for different reasons. For those with incurable or long-term cancers, some people might not think of themselves as a survivor, but more as someone who is “living with cancer.” Some may feel like they’re living but not surviving. I get that and it potentially resonates with my reluctance to use the ‘S’ word for a short period after my diagnosis. Thinking this topic through has made me compare where I was at diagnosis and where I am now. Also, I’ve considered what I’ve been able to do and what I have plans to do, despite my condition. I’ve done so much, been to so many places since I was diagnosed, and got plans to do much more, I must be surviving! Perhaps the words “I got this” was my subconscious thought in the picture I’ve used below?
And this picture which always gets a tremendous response every time I post it:
Here’s a great quote I found in relation to the term ‘survivor’:
“You may not like the word, or you may feel that it does not apply to you, but the word “survivor” helps many people think about embracing their lives beyond their illness”.
You may sometimes feel like you’re not surviving but if you’re reading this then you most definitely must be?