Neuroendocrine tumours (NETs) are rare cancers that develop from neuroendocrine cells, which regulate hormone production throughout the body. While NETs are more commonly diagnosed in adults, they can also occur in children and young adults, presenting unique challenges in diagnosis, treatment, and long-term care.
Due to their rarity in younger patients, awareness and research efforts are essential to improving early detection and treatment options. This article explores how NETs affect children and young adults, their causes, symptoms, and treatment options, as well as the psychological and social impacts of living with this condition.
Neuroendocrine Cancer Australia (NECA), is dedicated to assisting young people diagnosed with NETs and their loved ones. 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 can engage with NECA’s comprehensive support and information by calling the NET nurse line.
While NETs share many similarities across age groups, certain differences make their diagnosis and treatment more complex in younger patients.
NETs in children and young adults tend to have distinct genetic and biological characteristics, often associated with inherited syndromes.
Additionally, the long-term effects of treatment on growth, development, and fertility are significant concerns in younger individuals, requiring specialised care.
NETs are exceedingly rare in children and adolescents, making up a small fraction of all paediatric cancers. In Australia, childhood cancer accounts for less than 1% of all cancers, and within that, NETs represent an even smaller percentage.
Due to their low prevalence, many healthcare professionals are unfamiliar with paediatric NETs, leading to potential delays in diagnosis and treatment.
Like NETs in older people, there are a range of risk factors that contribute to the prevalence of NETs in young people. Similarly, the underlying causes of NETs still remain a mystery.
In children and young adults, NETs are more likely to be linked to genetic conditions, including:
While some NETs in younger patients are hereditary, others arise sporadically with no clear genetic cause. These cases may be triggered by random mutations in neuroendocrine cells that lead to uncontrolled tumour growth.
Although research is ongoing, potential environmental and developmental risk factors for paediatric NETs include:
However, in most cases, the exact cause of NETs in young people remains unknown.
Some types of NETs are more prevalent in young people than others.
Paediatric pancreatic neuroendocrine tumours (pNETs) can be functional or non-functional:
NETs can develop in the small intestine, appendix, or colon, often causing digestive symptoms. Appendiceal NETs are one of the more common NETs in younger patients and are sometimes discovered incidentally after an appendectomy.
Lung NETs in children and young adults are rare but can develop as typical or atypical carcinoid tumours, which may cause breathing difficulties or recurrent infections.
There are several other rare forms of NETs in young people that make up a fraction of the total case numbers of the condition in Australia.
Symptoms of NETs vary widely based on the tumour’s location and whether it is functional (producing hormones) or non-functional.
Common symptoms of NETs include:
Because NETs are rare in children, they are often misdiagnosed as other conditions, such as irritable bowel syndrome, asthma, or anxiety-related disorders. It’s important that people with changing symptoms contact their GP immediately.
Diagnosis usually involves:
Fortunately, there is a range of treatment options available to younger patients diagnosed with NETs. The treatment options, or mix thereof, to be pursued will be dictated by the patient, their care team, and the patient’s family.
Surgery is often the first-line treatment, especially for localised tumours that can be removed completely.
Somatostatin analogues (SSAs), such as octreotide and lanreotide, help control hormone-related symptoms and may slow tumour growth. Targeted therapies like everolimus and sunitinib are sometimes used in cases where the tumour is inoperable.
Chemotherapy is rarely used in low-grade NETs but may be considered for high-grade or aggressive cases. Radiotherapy, including peptide receptor radionuclide therapy (PRRT), is emerging as a potential option for paediatric NETs.
Treatment must be carefully managed in younger patients to minimise effects on growth, puberty, and long-term fertility. Hormone replacement therapy may be needed if endocrine function is affected.
Diagnosis of NETs in younger patients can be serious. However, patients face the best chance of a positive long term outlook if the tumours are diagnosed and treated as quickly as possible.
Prognosis depends on tumour type, grade, and stage at diagnosis. Many paediatric NETs are slow-growing and have good survival rates, but high-grade tumours require aggressive treatment.
Even after treatment, NETs often require long-term management. Dietary modifications, hormone therapy, and routine monitoring are essential for maintaining a good quality of life.
Regular check-ups with oncology and endocrinology teams are crucial to monitor recurrence and manage any late effects of treatment.
A NET diagnosis can be emotionally challenging for children and young adults, leading to anxiety, depression, or social isolation.
Organisations like Neuroendocrine Cancer Australia (NECA) provide support groups, resources, and counselling services. Schools and workplaces can also offer accommodations to help young patients manage their condition.
Advocacy efforts aim to increase awareness and funding for NET research, improving care options for young patients.
At present, researchers are exploring genetic markers, immunotherapy, and next-generation targeted therapies to improve treatment outcomes.
Advancements in liquid biopsy, AI-driven diagnostics, and molecular profiling hold promise for earlier detection and personalised treatment plans.
Though NETs are rare in children and young adults, early detection and access to expert care can significantly improve outcomes. Continued research, advocacy, and support are essential for improving the lives of young Australians diagnosed with NETs.
Further information and support for people diagnosed with NETs is available by calling the NECA NET nurse line.
