NET Cancer Day
The Unicorn Foundation has launched their first NET Cancer Nurse Specialist telephone and internet support for Australian (& New Zealand) patients. Given the large geographical impediments for patients outside large metropolitan centres to get to our support groups, it was decided that the most effective way to service the needs of all NET patients was to have a ‘hotline’ staffed by an experienced NET nurse. The response to this program has had overwhelming support from our patients and has improved the journey with NETs for many. Please take a look at our first in a series of Patient Videos that show the affect that support from a patient group has on a NET patients journey.
INCA member, the Association for Multiple Endocrine Neoplasia Disorders (AMEND) has sponsored the production of Medikidz™ comics on multiple endocrine neoplasia types 1 and 2 (MEN1 and 2). MEN disorders are inherited syndromes comprising more than one type of neuroendocrine tumour. Each child of an affected parent has a 1 in 2 chance of inheriting the condition. NETs occurring in MEN include those in the pancreas and phaoechromocytomas amongst others.
It is never easy trying to tell a child that they have or are at risk of having a genetic disorder. Children learn different things at different ages. In many instances however, this needs to be done, whether to ensure compliance with hospital tests or to discuss genetic testing. This is why AMEND worked with Medikidz™ to produce the exciting, vibrant and simply-written comic books. Comics, together with a leaflet on how to talk to children about MEN are available FREE to patients via AMEND.
To learn more about the comics which were produced as part of AMEND’s ‘Project Superhero!’ funded by the UK National Lottery, please visit the AMEND (HONCode certified) website: http://amend.org.uk/how-we-help/project-superhero.html
For more information about Medikidz™, visit: http://www.medikidz.com/
Coming soon will be corresponding children’s website animations on MEN1 and MEN2, so keep an eye on the AMEND website for further news!
“…the premier provider of quality information on Multiple Endocrine Neoplasia Disorders” Genetic Alliance (USA)
Ga68 PET/CT is widely recognised as the best imaging modality for detecting indolent neuroendocrine tumors in whole body scans. However there is a significant risk of false positives in the region of the ulcinate process, also known as ‘head of the pancreas’. Any Ga68 PET/CT which leads to suspicion of a tumor in the head of the pancreas should be compared with other imaging modalities such as MRI. If a tumor is still suspected, it should be corelated with an endoscopic ultrasound (EUS) and Fine Needle Aspiration (FNA) biopsy. These counter-checks should certainly be done before deciding on any invasive procedure such as a Whipple surgery. Article (400 words) The Ga 68 PET/CT (called ‘Gallium 68 scan’) is a combined functional and anatomical imaging technique for indolent neuroendocrine tumors. This technique offers much better resolution than other types of scans and has the added benefit of producing a measurable indication of the density of SST receptors. The SST receptor density (called ‘uptake’) is an accurate predictor of whether a particular patient is a suitable candidate for Peptide Receptor RadioTherapy (PRRT). Plus, receptor density can be used to monitor the success of any treatments the patient may be receiving, including PRRT. In centers which offer the Gallium 68 scan, it has replaced Octreoscan because of the better resolution. And it is more convenient for patients and nuclear medicine staff, since everything can be done during a single visit. For the above-mentioned reasons, the Gallium 68 scan has been described as the ‘gold standard’ of imaging neuroendocrine tumors. However there is reason to be cautious when interpreting the results of a Gallium 68 scan which shows uptake in the pancreas. There can be false positives due to physiological uptake in the head of the pancreas, as described in several medical studies (eg- http://www.ncbi.nlm.nih.gov/pubmed/21571796, http://www.ncbi.nlm.nih.gov/pubmed/22157030, http://www.ncbi.nlm.nih.gov/pubmed/21792572). This occurs because of the high concentration of SST receptors in the pancreatic islet cells, resulting in physiological uptake of the somatostatin analog (ie- DOTATOC or DOTANOC). One paper concludes: “Uptake in the head of the pancreas is a common finding in patients undergoing Ga68-DOTATOC PET/CT. However, this finding most likely represents a physiological condition, especially if the uptake in the pancreatic head is similar to uptake in the liver. Therefore, quantification is recommended to avoid false-positive diagnosis.” Another concludes: “Pancreatic DOTANOC uptake must be interpreted with caution, and further studies are required.” The possibility of Gallium scan false positives in the head of the pancreas has been recognised in Europe since about 2007. It is has been reported that up to 70% of such scans may display physiological uptake “sufficiently intense to be interpreted as pathologic and simulating a tumor” (http://www.ncbi.nlm.nih.gov/pubmed/22391705). But the potential for mis-diagnosis should be of particular concern in the USA, where a few medical centers have begun to offer this imaging modality after this technique was given orphan drug designation by the FDA (http://interactive.snm.org/index.cfm?PageID=13165). In Singapore there is a court trial underway brought by a patient who was suspected of having a pancreatic neuroendocrine tumor, and who underwent a Whipple resection, only to learn that no malignancy was found. The patient is Clement Hii, a businessman from Malaysia (see image below). The surgeon had proceeded with the Whipple operation on the basis of a positive Ga68 PET/CT even while the CT and MRI were negative and no blood markers were elevated. This took place in 2010, when the physiological uptake of Ga68 DOTANOC PET/CT in the pancreas was not well understood, and centers in Asia were just starting to use the scan. So patients should undergo a Ga68 PET/CT should not rely solely on apparent uptake in the head of the pancreas as evidence of disease there. They should get an endoscopic ultrasound and other diagnostics, including a biopsy if possible, to confirm a lesion before considering a radical surgery such as a Whipple resection.
Ron Hollander, Executive Director of the Caring for Carcinoid Foundation’s (CFCF) is pleased to announce the Foundation’s sponsorship of its first clinical trial. Using a new approach for delivering radiation to neuroendocrine tumors (NET), the project is designed to study a potentially safer and more effective form of Peptide Receptor Radionuclide Therapy (PRRT) for NET patients. The project will be carried out at Memorial Sloan-Kettering Cancer Center (MSKCC) in New York and will use this new approach for both treatment with the radionuclide, Lutetium-177 and imaging with the radionuclide, Gallium-68. Thanks to the generous support of our donors, we are thrilled to have this opportunity to test this approach in the hopes that it can lead to an improved treatment option for patients living with neuroendocrine cancers in the United States and beyond. To find out more, please visit the blog of CFCF’s Director of Research, Lauren Erb. Lauren’s blog.
This article was submitted by the Caring for Carcinoid Foundation.
CNETS Canada is dedicated to bringing information to NET patients across the country. To meet this goal, CNETS Canada has organized one day NET Patient Education seminars in four different cities across the country and plans to do more in 2015. The sessions cover topics such as the basics of NETS, diagnosis and treatment options, surgery and “whats new in NETs”. The sessions are being webcast recorded and made available to all.
With such a vast country and two official languages one of CNETS Canada’s challenges is to reach our NET patients. The easiest solution to reach every corner of Canada is to have a comprehensive and user friendly website. In 2013 we launched an easier to navigate English website and the French version is expected to be released this month. The French version of our NET Patient Handbook will be available from the French site. Visit us at www.cnetscanada.org
CNETS Canada is delighted to announce our first comprehensive Canadian NET cancer patient and caregiver education handbook in interactive soft copy format. This handbook is the result of many months of work and is a valuable source of information not only for patients but also for caregivers, health care providers, politicians, civil servants, funders and the general public to inform them on this uncommon, often undiagnosed or misdiagnosed form of cancer.
A huge thanks to the entire team that pulled this together; Sage Medica, our KOL’s, Dr. Shereen Ezzat, Dr. Simron Singh, Dr. Jean-Mathieu Beauregard and Karey McCann, RN as well as Silvia Steinhilber, CNETS Canada board member and Linda Dean, former CNETS Canada board member. A huge thanks to our funders as well, Novartis, Pfizer and Covidien, without whom this project would not have been possible.
We invite all patients and their caregivers and also health care providers to download a copy.
Link to handbook here http://www.cnetscanada.org/pdf/CNET-patient-booklet-13-11-27-interactive.pdf
The French version will be online shortly.
This article was submitted by CNETS Canada
Instigated in September 2009, the Pheo Para Troopers formed to provide support and advocacy for the small and geographically disparate metastatic pheochromocytoma and paraganglioma community. Pheochromocytoma and paraganglioma are very rare NET’s that manifest as adrenaline-producing tumours throughout the body. The disease occurs in its metastatic form in less than three in a million people per annum in the developed world and is managed via modern medical techniques rather than cured.
Located in the U.S., 60 – 70 % of the Pheo Para Trooper membership originates in North America. We wish to extend our reach and support throughout the world, by providing authoritative and up-to-date information to patients, and actively promote the disease to governments and doctors whilst networking and co-operating with as many other similarly minded NET patient groups as possible. Our motto is, “For the patients, by the patients” and we believe there is strength in bringing our globally disparate patients together.
We launched our re-designed web site (pheoparatroopers.org) in January 2014 and publish our own well-regarded newsletter called the Drop Zone, which is also translated into Japanese, Dutch and German. In addition, we have constructed the largest global directory of physicians who are conversant to the best practice, diagnosis, and treatment of our disease., which went on-line in February of this year.
The Troopers are also very active in developing our own patient and physician conferences and webcasts, the first of which will take place on Saturday 28/6/14, in Washington DC. During 2015, we hope to organise our first European patient conference and will also be attending the International Symposium on Pheochromocytoma and Paraganglioma, to be held in Kyoto, Japan during 17th-20th September. This event occurs every 3 years and is the focus of the publication of significant developments in research or treatment relevant to our disease.
Other ongoing projects include the formation of a de-identified Pheochromocytoma and Paraganglioma patient registry. This registry has a number of research institutions actively involved in its formation and is intended to allow researchers better access to larger sample groups, which will allow much better trend analysis within the disease. The registry intends to facilitate greater collaboration and integration between research centers.
Currently, we have appointed active patient liaisons within the UK and Ireland and are linked with patient groups in Spain and the Netherlands. We intend to achieve a global network by appointing Trooper representatives within each nation, or by affiliating to established patient groups already active there. We welcome contact with any organization or individuals who might assist with this or have a similar wish to promote our patients interests within the world of NETs and beyond.
This article was submitted by the Pheo Para Troopers.
Daniel Parsons of Colorado Springs USA has pancreatic neuroendocrine cancer with extensive liver metastases. He and his caregiver wife Susan have encountered many setbacks in their medical journey, but they recently have new hope after discovering that one of the top physicians treating neuroendocrine patients in the US works only 50 miles from their home.
Their new physician is Dr. Charles Nutting, an interventional radiologist specializing in performing minimally invasive image-guided procedures to treat liver cancer including neuroendocrine tumor metastases. Dr Nutting practices at Radiology Imaging Associates (RIA) in Denver Colorada, USA. How the Parsons’ were referred to Dr Nutting by a support group in their area, how they got in touch with Nutting via social media, and how they worked with him to accomodate their financial challenges is a very inspiring story.
Daniel was first diagnosed as having a pancreatic neuroendocrine primary tumor (pNET) in November of 2010. Initially he was given a stent to open his bile duct, but after two failed stents, surgeons proposed a pancreatic resection. Daniel underwent a Whipple procedure in June of 2011, an expensive and invasive procedure which requires several weeks of recovery.
It was planned that Daniel would undergo a liver resection after recovering from the Whipple. However, by the time follow up imaging was done in early 2012, it was determined that the metastatic liver lesions has progressed and involved both lobes. Surgery was ruled out and Daniel was referred to an oncologist near home in Colorado Springs. The oncologist recommended a wait-and-see approach, to be followed up with biologic treatments in the event of progression.
Daniel was started on Sunitinib (Sutent) in summer 2013, but there was continued disease progression in the liver. So the oncologist switched regimens and started him on a course of Everolimus (Afinitor), again without success. There seemed to be no benefit from these biologic therapies.
In April 2014, Daniel was referred to a local interventional radiologist (IR) for liver-directed therapy. The IR specialist suggested trans-arterial radio-embolization using Yttrium 90 microspheres, also known as ‘Y90 embolization’. Although not a specialist in pNETs, this physician had performed a few Y90 ebolization treatments, and he offered to try to get it authorized for coverage by the Parsons’ insurance.
It was while waiting for this authorization that Daniel’s wife Susan was contacted by Chris Nashville who is a member of the Facebook group Carcinoid Cancer Cafe, which Susan had been contacting to seek guidance from the patient community. Chris noted that this emobolisation requires very specialised angio-graphic skills to assure the radioisotope is deposited in the right place, and recommended consulting Dr Nutting, describing him as one of the country’s best NET specialists performing inverventional radiology. And he was literally in the Parson’s own backyard, practicing in nearby Denver.
Susan reached out to Dr. Nutting via Facebook and he replied right away, scheduling a consult to review Daniel’s case. Dr Nutting said that he would be happy to perform a Y90 embolization and had done many such procedures for NETs cancer patients like Daniel. This was good news indeed!
But Daniel’s insurance is Medicaid (which is the government insurance for Colorado). Medicaid will cover the pre-treatment diagnostics, the hepatic embolisation and the hospitalisation costs, but not the Y90 beads which have an estimated cost of USD $16,000. The material is expensive because it is specially engineered for biological use and contains Yttrium radioisotope. The Parsons’ informed Dr Nutting that they could not afford this.
After a few exchanges, Dr Nutting informed Susan and Daniel that the manufacturer of the Y90 beads and the hospital had agreed to a reduced rate which they could pay via installments over the next two years. This savings and the ability to pay make reasonable monthly payments was all the result of Dr Nutting’s lobbying on behalf of the Parsons, and his commitment to get them this potentially life-saving therapy.
In mid-May, Susan wrote back to Chris and the rest of the Facebook group to share the good news. She said: “[thanks to Dr Nutting] Daniel will be able to move forward and have the Y-90 procedure as planned! I cried for two hours at the amazing blessing that we have been given.” This story demonstrates again how fortunate the NETs community is to have committed specialists like Dr Charles Nutting. He’s a NET hero!
This article was submitted by CNETs Singapore.