As a star-studded crowd in Detroit wished a final farewell to Aretha Franklin the Queen of Soul, a relatively unheard-of group of patients around the world reflected on the suffering they had shared with her: neuroendocrine cancer. For the last seven years of her life, Aretha Franklin was being treated for a type of ‘pancreatic cancer’. Since her death on August 16th, hundreds of media commentators have been asking the question: How did she survive for so long when pancreatic cancer is usually very aggressive?’
The most common cancer of the pancreas we hear about is a pancreatic adenocarcinoma, which indeed does carry a poor prognosis, particularly when diagnosed at an advanced stage. Aretha Franklin, however, had a pancreatic neuroendocrine tumour (NET), and patients with this diagnosis can survive for many years even when the cancer has spread (metastasised) to other parts of the body.
Aretha Franklin, however, had a pancreatic neuroendocrine tumour (NET), and patients with this diagnosis can survive for many years even when the cancer has spread (metastasised) to other parts of the body.
Pancreatic NETs have relatively little in common with pancreatic adenocarcinoma, and share many more features with NETs of the small intestine, lung, appendix and stomach.
NETs have an incidence of approximately 7-9 per 100,000 people, compared to 16 per 100,000 for pancreatic adenocarcinoma. NETs can vary in their aggressiveness, but are mostly slow-growing, with a median overall survival of 9 years (2), and some patients surviving up to 20 years resulting in a prevalence of around 60 per 100,000. Apple co-founder Steve Jobs, BBC correspondent Nick Robinson, and recently Bollywood actor Irrfan Khan are all believed to have had NETs.
Groups such as the NET Patient Foundation (NPF) and UK & Ireland NET Society (UKINETS) are working hard to educate the UK medical community, supporting patients, as well as raising general awareness. A terminology problem exists within the international community – I talk here about neuroendocrine tumours or neuroendocrine cancers, others would use neuroendocrine neoplasm, neuroendocrine carcinoma, endocrine tumours, or the older term carcinoid. (Not to mention that it’s ‘tumor’ not ‘tumour’ if you’re from America. Or Ancient Rome.) The ‘neuro’ part refers to what the cells can resemble under the microscope (confusingly, it’s not actually anything to do with the brain) and the ‘endocrine’ part refers to the fact that some tumours are able to secrete chemicals (even though this is a minority of cases). Although a very complex issue, with the advent of ENETS guidelines in the last decade, experts are slowly moving towards using the same language internationally, which will ultimately greatly help patient understanding. Research into what is a very heterogeneous tumour – there are various groups of tumour, with subgroups which look and behave very differently (clinically, and under the microscope) – is also picking up pace.
Due to the insidious nature of this cancer, patients frequently present late, often with gastrointestinal symptoms or vague non-specific symptoms, leaving some patients being treated for an incorrect diagnosis. NETs are frequently diagnosed incidentally on imaging, and do also present as emergencies. Patients often go through a series of diagnostic/staging investigations and different therapies at various periods of their condition when their tumour is ‘stable’ or ‘progressive’. Input is required from numerous specialist teams, including gastroenterology, oncology, endocrinology, surgery and specialist nurses. None of the treatments generally ‘cure’ the condition (unless a full surgical resection can take place at an early stage) but will aim to slow the progression of the tumour, and thus enable a good quality of life.
As mentioned, some tumours can produce hormones, which cause specific symptoms. For example, carcinoid syndrome is a result of overproduction of chemicals such as serotonin, which causes flushing and diarrhoea and can result in fibrosis (scarring) of the cardiac valves and within the abdomen. The symptoms do, of course, impact on patients’ quality of life and emotional well-being.
With recent NICE-approved advances such as peptide receptor radionuclide therapy (PRRT), alongside established treatments such as somatostatin analogue injections, focus has turned towards managing the clinical, nutritional and psychological consequences of the treatments and tumour, so as to improve quality of life. This has been the specific goal of a new service for the benefit of patients with NETs throughout Wales. Commissioned by Welsh Government through WHSSC (Wales Health Specialised Services Committee), hosted by Cardiff and Vale University Health Board, and designed together with patients, this has already resulted in improved patient-reported outcomes and has further highlighted the complex needs of patients with NETs.
So, technically, yes, Aretha Franklin had a ‘pancreatic cancer’ but more correctly, she had a pancreatic neuroendocrine tumour. With increased awareness, we hope there will be an increase in research, funding and specialist services for patients with this hidden cancer.
1 Genus et al Incidence and Prevalence of Neuroendocrine Tumors in England. Neuroendocrinology 2018 https://www.enets.org/incidence-and-prevalence-of-neuroendocrine-tumors-in-england.html
2 Dasari et al. Trends in the Incidence, Prevalence, and Survival Outcomes in Patients With Neuroendocrine Tumors in the United States. JAMA Oncology 2017 Oct 1;3(10):1335-1342 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5824320/