Given NETs are less common and complex, medical studies are being conducted to better understand their underlying causes and to improve prevention, early detection, and treatment strategies.
If you have concerns about your risk of developing NETs or are experiencing symptoms, it is crucial to consult with a healthcare professional for proper evaluation and guidance.
While there are no apparent causes of neuroendocrine cancer, several risk factors have been identified. Risk factors are specific characteristics or exposures that increase an individual’s likelihood of developing neuroendocrine cancer.
In general, risk factors can be related to:
It’s important to note that having one or more risk factors does not guarantee that a person will develop cancer. However, risk factors can increase the chances. Conversely, many people diagnosed with cancer may not have any identifiable risk factors at all.
Certain rare, inherited diseases can elevate the risk of developing NETs. These conditions include multiple endocrine neoplasia (MEN 1, MEN 2), Von Hippel-Lindau (VHL) syndrome, tuberous sclerosis complex, and neurofibromatosis.
Multiple Endocrine Neoplasia Type 1 (MEN1): This rare genetic syndrome increases the risk of developing tumours in multiple endocrine glands, including the pancreas, parathyroid glands, and pituitary glands. Pancreatic NETs are a common feature of MEN1.
Multiple Endocrine Neoplasia Type 2 (MEN2): This inherited syndrome is associated with the development of tumours in the thyroid gland and adrenal glands. MEN2 can be further divided into MEN2A and MEN2B, with MEN2A often linked to the development of medullary thyroid cancer and adrenal medullary NETs.
Von Hippel-Lindau (VHL) Syndrome: VHL is a hereditary condition that predisposes individuals to the development of tumours in various organs, including the pancreas, adrenal glands, and kidneys. Pancreatic NETs are a significant concern in VHL syndrome.
Neurofibromatosis Type 1 (NF1): This genetic disorder causes tumours to form on nerves, and in some cases, it may lead to the development of NETs in various organs.
In some instances, a family history of NETs or related genetic conditions may increase the risk of developing these tumours.
Some types of NETs have a predilection for specific age groups and genders. For instance, neuroblastoma primarily affects young children, while pancreatic NETs and carcinoid tumours are more common in adults.
Neuroblastoma: This tumour is predominantly seen in young children, with the majority of cases diagnosed before the age of 5.
Pancreatic NETs and carcinoid tumours: These tumours are more commonly observed in adults, with the average age of diagnosis usually being in the 50s or 60s. Carcinoid tumours are slightly more common in women.
Environmental factors, such as exposure to certain carcinogens, may play a role in the development of NETs in some cases. However, the specific carcinogens that might be linked to NETs are not yet clearly identified, and more research is needed to establish direct links.
In some cases of Merkel cell carcinoma, infection with the Merkel cell polyomavirus (MCV) has been implicated as a contributing factor. MCV is thought to integrate into the genome of Merkel cells, potentially leading to cancerous changes.
Merkel cell carcinoma is also linked to excessive exposure to the sun and ultraviolet light. Additionally, it could also be triggered by the Merkel cell polyomavirus (MCV). Being aware of these risk factors can help individuals and healthcare professionals in the early detection and proactive management of NETs and Merkel cell carcinoma.
Hyperplasia is a condition in which there is an abnormal increase in the number of neuroendocrine cells in an organ or tissue. This hyperplasia may progress to the formation of NETs.
Disruptions in the immune system or immune suppression may contribute to the development of some NETs. For example, immunosuppression following organ transplantation can increase the risk of certain NETs.
Other factors: Some NETs may be associated with pre-existing conditions, such as:
It is important to note that while these factors have been associated with an increased risk of developing NETs, not everyone with these risk factors will develop the tumours, and many cases of NETs occur without an identifiable cause.
