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Pancreatic neuroendocrine cancer (PNET) is a unique and rare form of cancer. Nevertheless, this disease exhibits distinctive characteristics and has a huge impact on the lives of patients around Australia. In this comprehensive exploration, we delve into the fundamentals of PNET, from its introduction to its association with the intricate endocrine system in the pancreas.
NeuroEndocrine Cancer Australia advocates and supports all patients affected by PNETs and their families. The content covered on this page is purely informational. For specific information related to your diagnosis, contact your healthcare team, or call our NET cancer nurse.
Pancreatic neuroendocrine cancer is a subset of pancreatic tumours that originate in the neuroendocrine cells of the pancreas. Unlike more common pancreatic cancers, PNETs exhibit different biological behaviours, and can produce hormones. This sets them apart from other cancers, and underscores the significance of the need to understand how they form, function, and affect patients.
The rarity of PNETs, accounting for only a small percentage of all neuroendocrine cancers, makes them a focus of particular interest within the medical community. Despite their lower prevalence, PNETs can pose challenges in terms of diagnosis, prognosis, management, and treatment decisions.
To comprehend pancreatic neuroendocrine cancer, it is crucial to understand the role of the endocrine system within the pancreas. The pancreas, a multifunctional organ, serves both exocrine and endocrine functions.
Within the islet cells, the endocrine system regulates the production and secretion of hormones, including insulin and glucagon, which play pivotal roles in maintaining blood sugar levels. In the context of PNETs, it is these islet cells that undergo abnormal changes, leading to the development of neuroendocrine tumours.
This interplay between the endocrine and exocrine systems means that pancreatic neuroendocrine tumours gain the capacity to produce hormones independently. This phenomenon can result in a variety of symptoms, ranging from hormonal imbalances to nausea, and other less distinct clinical syndromes.
Pancreatic neuroendocrine cancer represents a convergence of abnormal cellular behaviour and the disruption of the finely tuned endocrine system in the pancreas. This understanding is instrumental not only in the diagnosis, risk factors, and causes of the disease, but also in the development of targeted treatments that address the unique characteristics of PNETs.
While the specific causes of pancreatic neuroendocrine cancer are unknown, research suggests that these tumours present a unique set of factors, with a notable role played by genetic predisposition and environmental influences.
Hereditary syndromes such as Multiple Endocrine Neoplasia Type 1 (MEN1) and Von Hippel-Lindau (VHL) syndrome significantly increase the risk of developing PNET. This underscores the importance of genetic testing for individuals with a family history of these syndromes.
Beyond genetic factors, environmental influences play a significant role in the genesis of PNETs. For example, exposure to substances like tobacco smoke and certain chemicals has been linked to an elevated risk of pancreatic neuroendocrine tumours.
Early detection of pancreatic neuroendocrine cancer relies on recognising and understanding the diverse array of symptoms associated with the disease. Here, we’ll cover common symptoms that patients may experience.
When discussing neuroendocrine cancers, it’s important to note that the presence and severity of symptoms can vary among individuals. Some of the common symptoms associated with pancreatic neuroendocrine cancer include:
PNETs can cause localised pain in the abdomen. The location and intensity of the pain may depend on the size and location of the tumour within the pancreas.
Individuals with PNETs may experience changes in their bowel habits, including diarrhoea or constipation. These changes can be attributed to the disruption of normal digestive processes by the tumour.
Unexplained weight loss is a common symptom of PNETs. The tumour’s impact on metabolism and nutrient absorption can lead to weight loss even without changes in diet or physical activity.
As discussed earlier, PNETs often can produce hormones, leading to hormonal imbalances. This can result in various symptoms such as flushing of the skin, persistent redness, particularly on the face and neck, and diarrhoea.
In cases where the tumour obstructs the bile ducts, jaundice may occur. Jaundice is characterised by yellowing of the skin and eyes due to the buildup of bilirubin, a yellow pigment.
Tumours affecting the digestive process can lead to nausea and vomiting. These symptoms may be exacerbated by the disruption of normal pancreatic function, impacting the digestion of food.
PNETs can interfere with the normal production and regulation of insulin, leading to changes in blood sugar levels. This can result in symptoms such as increased thirst, frequent urination, and fatigue.
It is essential to note that symptoms are not exclusive to PNETs and may also be indicative of other medical conditions. Due to the often nonspecific nature of these symptoms, a comprehensive medical evaluation is necessary for an accurate diagnosis.
Diagnosing pancreatic neuroendocrine tumours combines medical history, physical examination, imaging studies, and laboratory tests. The following outlines the key components of the diagnostic approach for PNETs:
Once the presence of a PNET is confirmed, staging is performed to determine the extent of cancer spread. Staging involves evaluating factors such as tumour size, involvement of nearby tissues, and the presence of metastasis.
Cancers, including pNETs, are commonly staged using the TNM staging system:
This TNM system is often complemented by a numerical classification ranging from stage 0 to IV:
Understanding the specific stage and grade of the pNET enables healthcare professionals to formulate a targeted and personalised treatment strategy for optimal patient care. Consultation and early detection are always the best methods of finding cancers at the earliest possible stage.
Fortunately, there are treatment options available to patients at all stages of pancreatic neuroendocrine cancer. These will vary from patient to patient and from stage to stage. The combination of therapies delivered to a patient will require transparent discussions between the patient and their healthcare team.
For localised tumours, surgery remains a primary treatment option. Various surgical procedures can be employed in managing PNETs, ranging from tumour removal, partial pancreatectomy and Whipples procedure.
When surgery alone is insufficient, chemotherapy may be required dependent on the grade of the PNET (predominantly grade 3). Chemotherapy drugs are utilised to target and impede the growth of cancer cells, forming a valuable part of an overall treatment approach for PNETs.
Emerging as a promising avenue, targeted therapies offer a more precise and targeted approach to treating PNETs. Targeted therapies focus on specific molecules crucial for cancer cell growth and survival.
The prognosis for pancreatic neuroendocrine tumours (pNETs) varies widely and depends on several factors, including the tumour’s stage, grade, size, location, and how well it responds to treatment.
Localised pNETs, especially those discovered at an early stage, often have a better prognosis. Surgical removal is a common and effective treatment for localised tumours. However, some pNETs may be more aggressive, and their prognosis can be less favourable, especially if they have spread to other organs.
There are many factors that can influence the outcome of prognosis and survival of pancreatic neuroendocrine cancer. These considerations include:
Providing a comprehensive understanding of these factors allows patients and their families to actively participate in discussions about treatment options and make informed decisions that align with their unique circumstances.
Survival rates for PNET can also vary significantly. Unlike the more common pancreatic adenocarcinoma, PNET tends to have a more favourable prognosis. The five-year survival rate for localised PNET is higher, reaching around 65-70%.
The landscape of pancreatic neuroendocrine cancer research is dynamic, with ongoing efforts aimed at uncovering new insights into the disease. There are many new and exciting developments in the space, including:
Targeted therapies and precision medicine are very promising in the realm of pNET treatment. Molecular profiling contributes to the identification of specific tumour characteristics and paves the way for more effective, individualised interventions.
PRRT can have a significant part in the treatment of metastatic pNETs. Targeted radiation to combat cancer cells has been shown to offer a promising avenue for improved survival rates and control of tumours.
Emerging technologies like liquid biopsy hold promise for non-invasive monitoring of disease progression and response to treatment.
As our understanding of the disease deepens and innovative therapies emerge, the prospects for patients diagnosed with pancreatic neuroendocrine cancer are becoming increasingly optimistic.
Receiving a diagnosis of pancreatic neuroendocrine cancer can be emotionally challenging for both patients and their families. Support groups, both online and in-person, are highlighted as valuable platforms for connecting with others facing similar challenges.
Additionally, NeuroEndocrine Cancer Australia is dedicated to raising awareness, providing educational resources, and advocating for PNET research. We aim to empower patients and their families with a sense of community and shared purpose.
For support and information, you can contact our dedicated NET cancer nurse – a free and anonymous call service that can answer questions you might have about pancreatic neuroendocrine cancer.
A holistic approach to pancreatic neuroendocrine cancer involves not only understanding the prognosis, survival rates, and ongoing research but also tapping into the valuable support resources available. NeuroEndocrine Cancer Australia aims to empower individuals affected by PNETs with knowledge, resilience, and a sense of community as they navigate the complexities of diagnosis, treatment, and beyond.
