Paraganglioma - Ronny Allan - Living with Neuroendocrine Cancer

20Nov 2023 by Ronny Allan No Comments

Neuroendocrine Cancer – Clinical Esoterica

Awareness

One of the key awareness messages for Neuroendocrine Cancer is the hormonal syndromes that can often accompany the diagnosis for many people. As it's a difficult disease to diagnose, many people struggle with these syndromes for some time before formal diagnosis of Neuroendocrine Cancer. Some continue to struggle after.Neuroendocrine Cancer can often be uncannily quiet, but the tumours can be 'functional' and over-secrete certain hormones to add or introduce symptoms that mimic many other diseases or conditions, such as Irritable Bowel Syndrome, Menopause, Heart disease and Asthma. In addition to common symptoms of flushing and diarrhea, others include generally feeling weak, fatigued, pain, agitated, anxious, dizzy, nauseous, acid reflux, skin irritation, anaemic, weight loss, weight gain, low blood sugar, high blood sugar, heart palpitations, headaches, sweating, high blood pressure.…

02Sep 2023 by Ronny Allan 5 Comments

Childhood Cancer Awareness Month – Neuroendocrine Cancer (featuring Elizabeth)

General

September is Childhood Cancer Awareness Month! The newspapers and social media will no doubt be featuring many childhood cancer articles. You may not see one about NET though. I personally cannot begin to imagine what it must be like to be a parent who has a child with cancer. I would just hope my child would be placed into the care and safe custody of experienced medical teams and would be able to get access to the best treatments available. I don't know that much about Childhood cancers, but I do know that over half of childhood cancers are either Acute Leukaemias or Brain Cancer, both of which are frequently in the news. It always breaks my heart when I read stories of children with cancer. I do know a…

28Aug 2023 by Ronny Allan 1 Comment

Neuroendocrine Cancer – normally slow but don’t be fooled!

Awareness

An awareness post from Ronny Allan. There are a lot of scary diseases in this world but some of them are particularly sneaky. One such sneaky disease is the lesser-known type of cancer that infiltrated my body - Neuroendocrine Cancer. Not only is it scary and sneaky, but it's also cunning, devious, misleading, and double-crossing. It likes nothing better than to play tricks on you. Don't be fooled, it's the great pretender. It will grow in your body without you knowing. It finds places to hide, mainly the small intestine, appendix, lungs, stomach, pancreas, rectum, and a host of other places. It can be fiendishly small to avoid being seen. Once it's established in the primary location (....or locations), it will try to break out via your blood and lymphatic…

26Aug 2023 by Ronny Allan 1 Comment

Phase 3 CABINET Clinical Trial – Cabozantinib for Advanced Neuroendocrine Tumours

Clinical Trials and Research

BREAKING NEWS - 24TH AUG 2023Exelixis Announces Remarkable Efficacy in CABINET Trial for Advanced Neuroendocrine TumorsOn August 24, 2023, Exelixis made an exciting announcement regarding the Phase 3 CABINET pivotal trial. This trial focused on evaluating the effectiveness of cabozantinib in treating advanced pancreatic and extra-pancreatic neuroendocrine tumors. The CABINET trial was a significant undertaking, involving multiple centers and enrolling a total of 290 patients who were divided into two groups.The trial progressed smoothly, but to everyone’s surprise, it was stopped early. The reason for this premature conclusion was the remarkable improvement in efficacy observed. The Alliance for Clinical Trials in Oncology independent Data and Safety Monitoring Board unanimously recommended ending the trial due to these unprecedented results."CABINET trial will be unblinded and stopped early due to a dramatic improvement…

07Jun 2023 by Ronny Allan No Comments

A spotlight on duodenal Neuroendocrine Neoplasms (dNENs)

A spotlight on NENs - Types

What are Duodenal Neuroendocrine Neoplasms (dNEN)? The duodenum is technically part of the small intestine, and you can find that in most anatomy-based descriptions. One of the research references I used was titled "Somewhere between the pancreas and the small intestine" and in the case of Duodenal NENs (dNENs), I can understand why they adopted that unconventional anatomy description. I suspect they contextually meant that they are not like either of these organs, but they share some similarities. They are not normally treated like small intestine NETs (SI NETs) or pancreatic NETs (pNETs) but they share some relationships, particularly with the latter. They are also closely aligned to the stomach (Gastric NENs) in terms of symptoms and syndromes that they sometimes present. Although they are a type of NEN (i.e. dNENs),…

18Feb 2023 by Ronny Allan No Comments

Belzutifan for the Treatment of Advanced Pheochromocytoma/Paraganglioma (PPGL), Pancreatic Neuroendocrine Tumor (pNET), or Von Hippel-Lindau (VHL) Disease-Associated Tumors

Clinical Trials and Research, Treatment

What is von Hippel-Lindau disease (VHL)?Von Hippel-Lindau disease (VHL) is an autosomal dominant disease that can predispose individuals to multiple neoplasms. Germline pathogenic variants in the VHL gene predispose individuals to specific types of benign tumors, malignant tumors, and cysts in many organ systems. These include central nervous system hemangioblastomas; retinal hemangioblastomas; clear cell renal cell carcinomas and renal cysts; pheochromocytomas, cysts, cystadenomas, and neuroendocrine tumors of the pancreas; endolymphatic sac tumors; and cystadenomas of the epididymis. What is Belzutifan (Welireg)?It is an oral hypoxia-inducible factor-2 alpha (HIF-2α) inhibitor. As an inhibitor of HIF-2α, belzutifan reduces transcription and expression of HIF-2α target genes associated with cellular proliferation, angiogenesis and tumor growth.On August 13 2021, FDA approved belzutifan (Welireg) to treat adults who have several tumors associated with VHL. Specifically, the drug is approved to treat VHL-associated renal cell carcinoma (a type of kidney…

24Nov 2022 by Ronny Allan No Comments

Neuroendocrine Neoplasms (NEN) – genetic related syndromes

Awareness, Patient Advocacy

Cancer is a genetic disorder. It happens when genes that manage cell activity mutate and create abnormal cells that divide and multiply, eventually disrupting how your body works. Medical researchers estimate 5% to 12% of all cancers are caused by inherited genetic mutations that you can’t control. More frequently, cancer happens as an acquired genetic mutation. Acquired genetic mutations happen over the course of your life. This is a very general description of the close relationships between genetics and cancer but I wanted to focus below on hereditary syndromes and genetic disorders related to Neuroendocrine Neoplasms. Definitions - the differences between Hereditary vs Familial vs Genetic Disorders I wanted to start with these definitions because people may unintentionally use these three terms interchangeably which is sometimes not the correct use of…

14Oct 2022 by Ronny Allan No Comments

The Hidden Pheochromocytoma

Awareness, Patient Advocacy

I've written a few times about Pheochromocytomas and Paragangliomas, allegedly rare types of Neuroendocrine Tumour (NET). I've also written about various hidden diagnoses of NET cases where they are eventually found in living patients having been 'hidden' within other diseases, i.e. misclassified in cancer registries or even not classified as cancer at all. If you read any medical site (including hospitals which treat Pheochromocytoma) you will note statements along the lines of "mostly benign" - so it's possible the incidence rate is vastly understated on this factor alone.The other interesting data I found is that many are not discovered until autopsy. Perhaps some people were asymptomatic or maybe they just stoically put up with their symptoms. Perhaps their symptoms were put down to routine illness such as hypertension, maybe imaging…

03Oct 2022 by Ronny Allan 2 Comments

Neuroendocrine Cancer: Glossary of Terms

Awareness, Clinical Trials and Research, Diet and Nutrition, Living with Neuroendocrine Cancer, Patient Advocacy, Survivorship, Treatment

Welcome to my Neuroendocrine Cancer Glossary of Terms list providing a source of meanings for acronyms and medical terms, all sourced from top Neuroendocrine Cancer and general cancer sites. How to use this list:1. If your term begins with an A, click on A to find all terms beginning with A. Select your term from the list.2. For numerical terms, please click on the hashtag (#) symbol in the A-to-Z strip.3. The term definition including acronym or abbreviation will be given in full along with any of my published articles containing that term as long as I have tagged it on my website to display in the list. Please note I'm constantly working on the repository to clean up all definitions, adding and removing links where necessary, and ensuring all definitions are…

10Sep 2022 by Ronny Allan 2 Comments

Awareness Post – Neuroendocrine Cancer in children and adolescents

Awareness, Inspiration

September is Childhood Cancer Awareness Month! The newspapers and social media will no doubt be featuring many children's cancer articles.I personally cannot begin to imagine what it must be like to be a parent who has a child with cancer. I would just hope my child would be placed into the care and safe custody of experienced medical teams and would be able to get access to the best treatments available. In 2020. this case came up in my google alerts and was related to a family who live not far from me in the south of England. An 8-year-old with Paraganglioma had lost an eye due to the location of the tumour. It is absolutely heartbreaking to read but it's amazing how resilient children can be. The Facebook followers were…

13Jul 2021 by Ronny Allan No Comments

Every picture tells a story (point, click, read)

Awareness, Clinical Trials and Research, Diet and Nutrition, Inspiration, Living with Neuroendocrine Cancer, Newsletters, Patient Advocacy, Survivorship, Treatment

I always try to use graphics for a number of pictures, I admit mainly to catch people's attention but also because sometimes a picture on its own tells a story or at least provides a great introduction to one. If the picture catches your eye, clicking on will take you to the text. This post will auto update as new blogs are published. thanks for reading and sharing! Scroll, point, click, read, share! Click here and answer all questions to join my private Facebook group Share on facebook Facebook Share on twitter Twitter Share on pinterest Pinterest Share on whatsapp WhatsApp Share on email Email Thanks for reading. Ronny I’m also active on Facebook. Like my page for even more news. Help me build up my new site here –…

24Jun 2020 by Ronny Allan No Comments

Targeted Therapy for Neuroendocrine Cancer – Sunitinib (Sutent)

Clinical Trials and Research, Treatment

Click here to see the A to Z of Neuroendocrine Cancer What is Sunitinib (Sutent)? Manufactured by Pfizer, this is a targeted biological therapy or more accurately, a multitargeted tyrosine kinase inhibitor (TKI). You may also see it described as an anti-angiogenic agent on the basis that these tumor types are highly vascularized and show high expression of something called vascular endothelial growth factor (VEGF), a key driver of angiogenesis in neuroendocrine tumors. Because NETs are generally hypervascularized tumors, treatment with antiangiogenic drugs seems a rational approach. A complex process but in the simplest of terms, sunitinib blocks a particular enzyme and keeps tumors from making their own blood vessels, which are needed to deliver oxygen and other nutrients to help them survive and grow. In clinical trials, SUTENT was…

25Feb 2020 by Ronny Allan 1 Comment

Clinical Trial: Lu-177 DOTATOC (PRRT) in adult subjects with Somatostatin receptor (SSTR) positive Pulmonary, Pheochromocytoma, Paraganglioma, Unknown primary, and Thymic Neuroendocrine Tumors

Clinical Trials and Research

Excellent news for those who don't have Gastroenteropancreatic NETs (GEP-NETs). A trial of PRRT is being set-up for the less common types of NET including those with an unknown primary. Using the DOTATOC radionuclide rather than the Lutathera model of using DOTATATE, this trial will cater for Pulmonary, Pheochromoctyoma, Paraganlioma, Unknown Primary and Thymic Neuroendocrine Tumours.Excel Diagnostics and Nuclear Oncology Center (EDNOC) announced today that its physician sponsored Investigational New Drug (IND) application to evaluate safety and efficacy of Lu-177 DOTATOC in adult subjects with Somatostatin receptor (SSTR) expressing Pulmonary, Pheochromocytoma, Paraganglioma, Unknown primary, and Thymic Neuroendocrine Tumors (PUT-NET), has received approval to proceed by USA Food and Drug Administration (FDA) and also charge authorization has been granted (IND#143631, NCT04276597). This IND is anticipated to start enrolling patients by early…

24Sep 2019 by Ronny Allan 1 Comment

Clinical Trials – PEN-221 for Neuroendocrine Cancer

Clinical Trials and Research

What is PEN-221?Tarveda Therapeutics is discovering and developing a new class of potent and selective precision oncology medicines for the treatment of patients with various solid tumor malignancies. Their strategy includes developing their own proprietary Pentarin miniature conjugates to enhance the effectiveness of promising anti-cancer payloads that have struggled without their selective targeting to solid tumors. These medicines are known as 'Pentarins'. PEN-221 is the lead candidate 'Pentarin' aimed at Neuroendocrine Cancer - PEN-221.Somatostatin receptor 2 (SSTR2) is frequently overexpressed on several types of solid tumors, including neuroendocrine tumors and small-cell lung cancer. Peptide agonists of SSTR2 are rapidly internalized upon binding to the receptor and linking a toxic payload to an SSTR2 agonist is a potential method to kill SSTR2-expressing tumor cells. PEN-221 is a conjugate consisting of microtubule-targeting…

09Sep 2019 by Ronny Allan No Comments

Clinical Trials – ONC201 for Neuroendocrine Cancer (including Pheo/Para)

Clinical Trials and Research

What is ONC201?A company called Oncoceutics is developing a novel class of safe and effective cancer therapies called imipridones. Imipridones have a unique three-ring core structure and selectively target G protein-coupled receptors (GPCRs), the largest class of membrane receptors and a common target of approved drugs that are underexploited in oncology. Despite being historically uncommon as targets in oncology, GPCRs control an array of critical prosurvival and stress signaling pathways that are often dysregulated in human cancer to favor cancer cell survival and propagation.The ability of imipridones to target GPCRs with a high degree of selectivity represents a novel opportunity in oncology that generates remarkably safe and effective therapeutics. ONC201, the founding member of this novel class of therapies, is an orally active, safe, and selective antagonist of the GPCR…

30Oct 2018 by Ronny Allan 9 Comments

Neuroendocrine Cancer Hormonal Syndromes – a witch’s brew

Awareness

PLEASE SHARE One of the key awareness messages for Neuroendocrine Cancer is the hormonal syndromes that can often accompany the diagnosis for many people. As it's a difficult disease to diagnose, many people struggle with these syndromes for some time before formal diagnosis of Neuroendocrine Cancer. Some continue to struggle after.Neuroendocrine Cancer can often be uncannily quiet, but the tumours can be 'functional' and over-secrete certain hormones to add or introduce symptoms that mimic many other diseases or conditions, such as Irritable Bowel Syndrome, Menopause, Heart disease and Asthma. In addition to common symptoms of flushing and diarrhea, others include generally feeling weak, fatigued, pain, agitated, anxious, dizzy, nauseous, acid reflux, skin irritation, anaemic, weight loss, weight gain, low blood sugar, high blood sugar, heart palpitations, headaches, sweating, high…

02Oct 2017 by Ronny Allan 10 Comments

Genetics and Neuroendocrine Tumors

Awareness, Living with Neuroendocrine Cancer, Survivorship

Hereditary genetics.....where to focus In recent years, it has become increasingly apparent that a number of Neuroendocrine tumours arise as a result of germline genetic mutations and are inherited in an autosomal dominant pattern. The number of genes implicated is increasing and I cannot guarantee this post will contain all of them. Apparently, 5-10% of NETs are estimated to have a hereditary background. Hereditary syndromes associated with these include Multiple Endocrine Neoplasia (MEN), Von Hippel Lindau (VHL), Neurofibromatosis Type 1 (NF1), Tuberous Sclerosis (TS) and others. People who have a genetic condition may present with the tumors (perhaps along with an associated functional hormone syndrome) and so the genetic condition if there is one, may not be known at this point. [caption id="attachment_14317" align="aligncenter" width="1280"] Overview of genes with recurrent…

05Jul 2017 by Ronny Allan No Comments

NETwork with Ronny © – Community Newsletter JUNE 2017

Newsletters

Hi NETworkers! Welcome to my monthly 'Community' newsletter. This is June 2017's monthly summary of Ronny Allan's Community news, views and ICYMI (in case you missed it!). NET News The following news items may be of interest: NETs in the UK National News. Great publicity. Featuring NET Patient Foundation. Click here. Personalised PRRT is highlighted. Click here. Everolimus and Sunitinib. In England, NICE approves Everolimus (Afinitor) and Sunitinib (Sutent). Read more by clicking here. Videos from LACNETS. I've not watched them all yet due to holiday but they are always great! Click here. PRRT. News of a PRRT trial being set up for Inoperable Pheochromocytoma/ Paraganglioma. Not yet recruiting but read more here. Immunotherapy. Merkel Cell Carcinoma is already benefiting from an FDA approved drug with another pending. Check out…

17Jun 2017 by Ronny Allan 8 Comments

Immunotherapy: Studies with Neuroendocrine Neoplasms

Clinical Trials and Research, Treatment

IntroductionThere's a lot of Immunotherapy stuff out there! However, I also wanted to break it down and perhaps see if I can pick up the what, when, why, where and how in regard to Neuroendocrine Cancer. It's difficult, not least because the picture is not clear and there is no general roadmap printed, let alone one for Neuroendocrine disease. Immunotherapy for NETs was discussed at ENETS 2017 in Barcelona. The presentation that sticks out was one given by Dr Matthew Kulke, a well-known NET Specialist in Boston. My reaction to the presentation was one of 'expectation management' and caution i.e. it's too soon to know if we will get any success and when we will get it. He also hinted that it's more likely that any success will first be…

08May 2017 by Ronny Allan 45 Comments

All you need to know about Peptide Receptor Radionuclide Therapy (PRRT)

Clinical Trials and Research, Treatment

Short PRRT PrimerWhat is Peptide Receptor Radionuclide Therapy (PRRT)?What is PRRT? PRRT stands for Peptide Receptor Radionuclide Therapy, an FDA-approved therapy used for systemic treatment of neuroendocrine tumors. Peptide refers to the small molecule for this therapy. The Peptide used (DOTATATE or DOTATOC or DOTANOC) is very similar to Somatostatin, a hormone which binds to receptors found on neuroendocrine tumors. Receptor refers to a specific target on neuroendocrine tumor cells that the peptide attaches to. After the peptide joins with a Receptor, it becomes attached and enters the targeted tumor cell. Radionuclide refers to radioactive atom that is attached to the peptide. As the peptide enters the cell, it brings the Radionuclide into the tumor cell. The radionuclide (e.g. lutetium-177), then decays and emits radiation within the tumor cell.Therapy refers to the idea that we are using this combination of Peptides, Receptors, and Radionuclides…

18Jan 2017 by Ronny Allan 5 Comments

Theranostics for Neuroendocrine Cancer – A Find and Destroy Mission

Awareness, Clinical Trials and Research, Living with Neuroendocrine Cancer, Treatment

[caption id="attachment_14426" width="1200"] Courtesy of Pashtoon Kasi MD on Twitter https://twitter.com/pashtoonkasi/status/1078675398601396224[/caption] Theranostics is a joining of the words therapeutics and diagnostics. You may also see it conveyed as 'Theragnostics' and these terms are interchangeable. The basic aim of theranotistics is to find and then destroy the 'bad guys'. With Neuroendocrine Cancer, finding the tumours (the bad guys) can often be a challenge - they can be small and/or difficult to find - they are sometimes expert at camouflage. Moreover, once found, they can then be difficult to treat (destroy), as they can often prove resistant to conventional cancer drugs and many are inoperable due to sheer quantity, spread and positioning. When they are found and identified, it's also really helpful to know from the intelligence gathered, how successful the destroy…

05Nov 2016 by Ronny Allan 8 Comments

Neuroendocrine Cancer – If you can see it, you can detect it!

Living with Neuroendocrine Cancer, Survivorship

Content in my review queue (line). Background 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…

25May 2016 by Ronny Allan 5 Comments

The 5 E’s (of Carcinoid Syndrome)

Awareness, Diet and Nutrition, Living with Neuroendocrine Cancer, Patient Advocacy, Survivorship, Treatment

Since my diagnosis, I seem to have been in a perpetual learning phase! What not to do, what not to eat, what not to read! However, early on in my experience, I came across a list of 'E' words (5 of them) which is a handy reminder for Carcinoid Syndrome patients, particularly those whose symptoms are not under control. When I say "carcinoid syndrome" in this article, I only mean the syndrome that is caused by what was once called "Carcinoid Tumors", i.e. predominately serotonin secreting types which mainly metastatic cases where the well differentiated tumours are found in the small intestine, but much less commonly in appendiceal, very rarely in rectal (rarely even at stage IV), lung (more atypical and around 10%), and one or two other significantly less…

09Mar 2016 by Ronny Allan 4 Comments

A spotlight on Multiple Endocrine Neoplasia (MEN)

Awareness, Living with Neuroendocrine Cancer

This is an overview, if you require detail, try my more comprehensive post by clicking here or on the picture[caption id="attachment_38157" align="aligncenter" width="640"] Click on the picture to read more[/caption]OverviewWe all know that Neuroendocrine Tumours (NETs) and their syndromes are complex but there is even more complexity to be found in a group of related disorders known as Multiple Endocrine Neoplasia (MEN). I recommend all NET patients should try to understand the basics of MEN and vice versa, particularly as both conditions seem to come with a plethora of endocrine-related effects.MEN patients will normally have a tumour in at least two endocrine glands - thus the terms 'Multiple' and 'Endocrine' (tumours can also develop in other organs and tissues). Neoplasia is just another name for tumour and these can be non-cancerous (benign) or…

10Aug 2015 by Ronny Allan 16 Comments

The Syndromes of Neuroendocrine Cancer – Early Signs of a Late Diagnosis

Awareness

The Syndromes of Neuroendocrine Cancer - Early Signs of a Late DiagnosisOne of the curious things about Neuroendocrine Cancer (NETs elsewhere in the text) is that it can very often exhibit one or more vague symptoms collectively known as a 'syndrome'. Syndrome is an apt word to describe these complications as the most general meaning in medical terms is a group of symptoms that together are characteristic of a specific disorder or disease". Having a syndrome can often be the difference between having a 'functional' condition or a non-functional' condition - see more below.This frequently makes Neuroendocrine Cancer very difficult to diagnose quickly. It's a very devious disease.Functional / Non-FunctionalThese tumours and associated syndromes are treatable for most but the difficult part can be arriving at a diagnosis. Moreover, without a syndrome, some of these…

16Oct 2014 by Ronny Allan 14 Comments

Neuroendocrine Cancer – Horrible Hormones

Patient Advocacy

Hormonal imbalances are quite common in many conditions including day to day stuff. With Neuroendocrine Cancer, it can be a real challenge both at diagnostic and maintenance phases. In addition to the cancer angle, there's some strange stuff going on, inexplicable, frightening for the patient, an unwanted ingredient causing chaos!Until I was diagnosed with metastatic Neuroendocrine Cancer, I didn't have a clue about hormones - it's one of those things you just take for granted. However, hormones are vital to human health (male and female) and it's only when things go wrong you suddenly appreciate how important they are. Hormones are involved in many conditions, not just an issue with Neuroendocrine Tumours (NETs) but the presence of over-secreting hormones (often called peptides throughout) is useful to aid a diagnosis, albeit…

Tag: Paraganglioma