Neuroendocrine tumours (NETs) can present with a wide range of symptoms that impact patients’ quality of life, daily function, and emotional wellbeing. While tumour control is a central goal of treatment, managing symptoms is equally important.
Effective symptom management can reduce pain, control hormonal effects, prevent complications, and allow patients to live fuller, more independent lives.
Let’s explore the key strategies used to manage symptoms in NET patients, from somatostatin analogues and interferon therapy to targeted symptom control for carcinoid syndrome and acute crises.
NeuroEndocrine Cancer Australia (NECA), is dedicated to assisting individuals diagnosed with NETs, and their families. NECA offers a wealth of resources, educational programs, and advocacy efforts aimed at deepening the understanding of NETs, improving patient care, and encouraging research advancements. Patients diagnosed with neuroendocrine cancer can engage with NECA’s comprehensive support and information by calling the NET nurse line.
NETs are unique in that many of them secrete hormones or bioactive amines, which can lead to systemic symptoms beyond the effects of tumour growth. Common hormone-related symptoms include diarrhoea, flushing, wheezing, palpitations, and, in some cases, severe electrolyte disturbances or hypoglycaemia. Tumour-related symptoms often stem from mass effect, such as bowel obstruction, abdominal pain, or pressure on nearby structures.
Uncontrolled symptoms can lead to hospitalisations, malnutrition, anxiety, and social withdrawal. For example, persistent diarrhoea may cause dehydration and weight loss, while uncontrolled carcinoid syndrome can damage heart valves over time. Early recognition and treatment of symptoms can prevent these complications, improve physical comfort, and preserve organ function.
The primary goal of medical therapy in NET symptom management is to reduce or eliminate symptoms while minimising treatment-related side effects. This involves not only controlling hormone secretion but also managing tumour burden and its effects. Additional objectives include:
Symptom management should be individualised, reviewed regularly, and integrated into the overall treatment plan.
Somatostatin is a naturally occurring hormone that inhibits the release of many other hormones. Synthetic somatostatin analogues (SSAs), such as octreotide and lanreotide, are designed to mimic this effect and bind to somatostatin receptors on tumour cells. This action reduces the secretion of serotonin, gastrin, insulin, and other substances that drive hormone-related symptoms.
Octreotide and Lanreotide are the most widely used SSAs in Australia. These are generally given as monthly injections and have been shown to:
Side effects are usually mild and may include injection site discomfort, gallstone formation, or mild gastrointestinal upset. Dose escalation, reduction, or switching between SSAs may be needed if symptoms are not fully controlled.
Interferon-alpha has immunomodulatory and antiproliferative effects and can be used as a second-line therapy when SSAs are not sufficient. It is particularly helpful in controlling serotonin secretion and reducing flushing and diarrhoea.
While effective, interferon therapy can be associated with fatigue, flu-like symptoms, depression, and thyroid dysfunction. Its use is generally reserved for patients with refractory symptoms or those who are not candidates for other systemic treatments. Careful monitoring is required, and shared decision-making is essential to weigh benefits against potential impact on quality of life.
Carcinoid syndrome is one of the most recognisable manifestations of functional NETs and is caused by excessive serotonin release. Uncontrolled carcinoid syndrome can lead to diarrhoea, flushing, bronchospasm, and eventually carcinoid heart disease.
Supportive medications such as bile acid sequestrants may also be used if diarrhoea is related to short bowel syndrome after surgery.
Abdominal pain may result from mesenteric fibrosis, tumour pressure, or bowel obstruction. Pain management often includes:
Nutritional support is critical, as many patients experience malabsorption. A dietitian may recommend small, frequent meals, pancreatic enzyme replacement, or vitamin supplementation. Psychological support, physiotherapy, and fatigue management strategies are also key components of holistic symptom care.
Carcinoid crisis is a potentially life-threatening complication characterised by sudden flushing, hypotension, hypertension, bronchospasm, and arrhythmias. It can be triggered by surgery, anaesthesia, or invasive procedures. Preventive measures include:
In an acute crisis, rapid administration of intravenous octreotide, fluids, and vasopressors is critical. Management should be carried out in a setting where airway support and cardiovascular monitoring are available.
Symptom control should not occur in isolation. Ideally, it is integrated with the overall treatment strategy. For example:
Symptom management is an ongoing process. Regular review of hormone levels, imaging, and patient-reported outcomes helps clinicians adjust therapy as needed. Some patients may need higher SSA doses over time, while others may benefit from switching to a different agent or adding new treatments. Education empowers patients to recognise changes in symptoms early and seek help before complications develop.
Symptom management is a cornerstone of care for people living with neuroendocrine tumours. By controlling hormone secretion, reducing tumour burden, preventing complications, and supporting overall wellbeing, these strategies help patients maintain independence and quality of life. The best outcomes occur when symptom management is delivered as part of a coordinated, multidisciplinary approach that adapts over time to meet the patient’s changing needs.
As research continues, new treatments are emerging that offer even greater precision in controlling hormone-related symptoms, promising a future where living with NETs is more manageable and less disruptive.
Further information and support for people diagnosed with NETs is available by calling the NECA NET nurse line.
Kulke MH, O’Dorisio TM, Phan AT, et al.
TELEPRO: Patient‐reported carcinoid syndrome symptom improvement among patients initiating telotristat ethyl.
The Oncologist, 2019.
https://academic.oup.com/oncolo/article/24/11/1446/6439827 Oxford Academic
ClinicalTrials.gov.
STOPNET: Cessation of Somatostatin Analogues after PRRT in Mid, Hind-Gut and Pancreatic Neuroendocrine Tumours.
ClinicalTrials.gov registry.
https://clinicaltrials.gov/study/NCT06345079 ClinicalTrials.gov
Lexicon Pharmaceuticals / ICH GCP.
TELESTAR: Phase 3 study of telotristat etiprate in carcinoid syndrome not adequately controlled by SSA therapy.
Clinical Trials Registry (NCT01677910).
https://ichgcp.net/clinical-trials-registry/NCT01677910 ICHGCP
Chest Journal.
Somatostatin analogues improve respiratory symptoms in patients with NETs.
Chest, study on pulmonary function and symptom response to SSAs.
https://journal.chestnet.org/article/S0012-3692%2820%2930258-0/fulltext Chest Journal
Sciencedirect article.
Telotristat: A Novel Agent for Somatostatin Refractory Diarrhoea from Carcinoid Syndrome.
Long-term data and consensus guidelines supporting telotristat use with SSA.
https://www.sciencedirect.com/science/article/pii/S0885392424003336 ScienceDirect
Karger / NEN (Neuroendocrine Neoplasms).
Long-Term Treatment with Telotristat Ethyl in Patients with Carcinoid Syndrome.
TELEPATH Trial, safety and quality of life outcomes over ~100 weeks.
https://karger.com/nen/article/112/3/298/825357/Long-Term-Treatment-with-Telotristat-Ethyl-in Karger
BMJ Open.
SAUNA trial: Somatostatin analogue continuation upon progression in patients with gastroenteropancreatic neuroendocrine tumours.
Protocol comparing continuing or withdrawing SSAs when disease progresses.
https://bmjopen.bmj.com/content/15/7/e099996 BMJ Open
Annals of Oncology.
Efficacy and safety of telotristat ethyl in patients with carcinoid syndrome: TELESTAR study.
Phase III trial showing reductions in diarrhoea and improvements in symptom control.
https://www.annalsofoncology.org/article/S0923-7534%2820%2937856-X/fulltext Annals of Oncology
Consensus report (multiple authors).
Consensus report on the use of somatostatin analogues for the management of neuroendocrine tumours.
Review of symptom control, hormone excess, and long-term SSA use.
https://www.sciencedirect.com/science/article/pii/S0923753419618387 ScienceDirect
ENETS / real-world study.
Real-world effectiveness and serotonin reduction in patients with progressive NETs and carcinoid syndrome receiving telotristat ethyl.
TELEACE study (ENETS presentation) reporting biomarker and symptom changes over 6 months.
https://www.enets.org/abstract/real-world-effectiveness-and-serotonin-reduction-in-patients-with-progressive-neuroendocrine-tumors-and-carcinoid-syndrome-receiving-telotristat-ethyl.html
