A spotlight on Appendiceal Neuroendocrine Neoplasms

A spotlight on Appendiceal Neuroendocrine Neoplasms

A spotlight on NENs - Types, Awareness, Patient Advocacy, Spotlight on NENs - Types Series
What are Appendiceal NENsAppendiceal Neuroendocrine Neoplasms (NENs) account for approximately 60% of all primary cancers in the appendix.  The other main cancer types found in the appendix are regular adenocarcinomas (including signet ring cell and goblet cell) and mucinous neoplasms such as Pseudomyxoma Peritonei (PMP) or Low-Grade Mucinous Neoplasms (LAMD).  Appendiceal neuroendocrine tumours (aNET) (i.e. well differentiated) account for 75% of all aNENs, are mostly indolent tumours treated effectively with simple appendectomy. In fact, most are incidentally found upon presentation of right lower quadrant (RLQ) pain in keeping with appendicitis. They are typically (but not exclusively) diagnosed in younger patients. Poorly differentiated appendiceal Neuroendocrine Carcinomas (aNEC) resemble small-cell or large-cell neuroendocrine carcinomas of the lung, have aggressive behaviour, and usually present with metastatic disease at diagnosis.Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) are possible, particularly…
Read More
A spotlight on Gastric Neuroendocrine Neoplasms

A spotlight on Gastric Neuroendocrine Neoplasms

A spotlight on NENs - Types, Awareness, Patient Advocacy, Spotlight on NENs - Types Series, Treatment
What are Gastric NENsUnlike other anatomical types, the word Gastric is used to indicate a tumour (NET) or carcinoma (NEC) in the stomach.  The stomach lies at the bottom of your oesophagus and connects to the first part of the small intestine (the duodenum).  Clearly a key part of the gastrointestinal system, it processes food on its journey downwards.The stomach produces strong acid. This kills many microorganisms that might have been swallowed along with the food. It also contains special chemicals called enzymes. These are important for breaking down the food so it can be absorbed by the body. After it leaves the stomach, the partially digested food passed into the intestines where it begins to be absorbed.The main parts of the stomach are shown below (often this can give…
Read More
A Spotlight on Lung Neuroendocrine Neoplasms

A Spotlight on Lung Neuroendocrine Neoplasms

A spotlight on NENs - Types, Awareness, Patient Advocacy, Spotlight on NENs - Types Series
WHO Classification of Tumours, 5th Edition, Volume 5: Thoracic TumoursThe aim of this spotlight is to provide a summary of the latest information on Lung Neuroendocrine Neoplasms (NEN) including the latest terminology, epidemiology data, and guidelines.   This follows the publication of the Thoracic WHO classification 5th edition (2021): terminology and criteria for neuroendocrine neoplasms (Blue Book).  This book has been anticipated to see if the panel compiling this follows the lead of the Gastroenteropancreatic Neuroendocrine Neoplasms (GEPNEN) editions issued in 2017 (Endocrine) and 2019 (Digestive systems) by removing the antiquated misnomer term ''Carcinoid" in line with the recommendations made by senior NEN doctors in 2020 to have commonality across all blue books. Neuroendocrine neoplasm update: toward universal nomenclature © 2020 Society for Endocrinology 2020, Guido Rindi and Frediano Inzani.I can tell…
Read More
Neuroendocrine Tumours: a spotlight on Pheochromocytoma and Paraganglioma

Neuroendocrine Tumours: a spotlight on Pheochromocytoma and Paraganglioma

A spotlight on NENs - Types, Awareness, Patient Advocacy, Spotlight on NENs - Types Series
Updated 19th November 2023I spend a lot of time talking about the most common forms of Neuroendocrine Tumours (NETs), but what about the less well-known types?  As part of my commitment to all types of NETs, I'd like to shine a light on two less common tumour types known as Pheochromocytomas and Paragangliomas - with an incidence rate of approximately 8 per million per year. They are normally grouped together, and the definitions below will confirm why.  If you think it's difficult to diagnose a mainstream NET, this particular sub-type is a real challenge.So, let's get definitions out of the way:Pheochromocytomas (Pheo for short)Pheochromocytomas are tumours of the adrenal gland that produce excess adrenaline. They arise from the central portion of the adrenal gland, which is called the adrenal medulla…
Read More