Home » Treatments » Treatment for Pancreatic Neuroendocrine Tumours
Pancreatic Neuroendocrine Tumours (PNETs) are a subset of tumours arising from the pancreas, characterised by unique biological behaviours and clinical implications. Despite their relatively lower incidence compared to other conditions affecting the pancreas, PNETs require specialised attention due to their distinct features and treatment considerations.
PNETs arise from neuroendocrine cells in the pancreas responsible for regulating hormone production and secretion. These tumours can be functional, producing hormones that cause characteristic symptoms, or non-functional, leading to more subtle clinical presentations.
PNETs present histological characteristics, ranging from well-differentiated, slow-growing tumours to poorly differentiated, aggressive neoplasms.
Neuroendocrine Cancer Australia is here for the support of patients and families battling PNETs. For more information, or to talk to someone directly, you can call our free NET nurse service.
Early detection of PNETs is imperative for initiating timely intervention and improving patient outcomes. These tumours often exhibit slow growth and may remain asymptomatic until advanced stages, underscoring the critical role of screening protocols and advanced imaging techniques in facilitating early diagnosis.
Swift identification allows for faster implementation of appropriate treatment strategies, potentially limiting disease progression and enhancing prognosis.
Understanding the classifications and grades of PNETs is essential for guiding treatment decisions and optimising therapeutic outcomes. PNETs can be categorised based on histological features such as differentiation and proliferation rates, significantly influencing treatment approaches and prognosis.
PNETs are classified into grades based on their proliferative activity, as determined by histological markers such as Ki-67 index and mitotic rate.
Surgery plays a central role in the management of localised PNETs, offering curative potential and symptom relief. Various surgical types are employed based on tumour characteristics and patient factors, and can include:
Eligibility for surgery is determined through comprehensive evaluation and multidisciplinary collaboration, ensuring optimal treatment outcomes and postoperative care.
Pancreaticoduodenectomy, also known as the Whipple procedure, involves the surgical removal of the head of the pancreas, duodenum, gallbladder, and part of the bile duct and stomach. This procedure is commonly performed for tumours located in the head of the pancreas or periampullary region.
Distal pancreatectomy involves the removal of the distal portion of the pancreas and is typically performed for tumours located in the body or tail of the pancreas. Enucleation is a more conservative surgical approach that involves removing only the tumour while preserving the surrounding pancreatic tissue.
Medical treatments and targeted therapies play a crucial role in managing PNETs, offering symptomatic relief and disease control. There are several common ways that these can positively affect PNETs:
Somatostatin analogues work by binding to somatostatin receptors on tumour cells, acting to inhibit hormone secretion and reduce tumour growth. These agents are commonly used to control symptoms associated with functional PNETs, such as flushing, diarrhoea, and abdominal pain. Somatostatin analogues can also slow the growth of PNETs.
Everolimus and sunitinib are targeted therapies that inhibit specific signalling pathways involved in tumour growth and angiogenesis. These agents have demonstrated efficacy in controlling disease progression and improving progression-free survival in patients with advanced PNETs.
PRRT is an innovative treatment option for advanced PNETs, particularly those expressing somatostatin receptors. This targeted therapy delivers radiation directly to tumour cells, minimising systemic toxicity and preserving healthy tissues. Ongoing research aims to optimise PRRT protocols and expand their availability, offering greater therapeutic benefits for eligible patients.
PRRT involves the administration of radiolabeled somatostatin analogues, which selectively bind to somatostatin receptors on tumour cells. Once bound, the radiolabelled compound delivers radiation directly to the tumour, leading to localised tumour destruction while sparing surrounding healthy tissue.
PRRT has shown efficacy in controlling tumour growth, improving symptom control, and prolonging progression-free survival in patients with advanced PNETs. Ongoing clinical trials are evaluating the efficacy of PRRT in combination with other treatment modalities, including targeted therapies and chemotherapy, to further optimise treatment outcomes.
Chemotherapy is indicated for high-grade PNETs / NECs and cases resistant to conventional therapies. Common chemotherapeutic agents, including streptozocin and 5-fluorouracil, exert cytotoxic effects on tumour cells, albeit with potential side effects such as myelosuppression and gastrointestinal disturbances. Individualised chemotherapy regimens tailored to tumour histology and patient tolerance create better treatment outcomes.
Chemotherapy may be used as first-line therapy for patients with advanced or metastatic PNETs/ NECs who are not candidates for surgery or other local treatments. Combination chemotherapy regimens, including oxaliplatin, irinotecan plus 5-fluorouracil, have been shown to improve response rates and prolong progression-free survival in patients with advanced PNETs.
Transarterial Chemoembolisation (TACE) and Selective Internal Radiation Therapy (SIRT) are specialised interventions targeting liver metastases in PNET patients. These locoregional therapies deliver chemotherapy or radiation directly to tumour-feeding vessels, facilitating tumour regression and symptom control while preserving liver function.
TACE involves the injection of chemotherapy drugs directly into the blood vessels supplying the tumour, followed by the placement of embolic agents to block blood flow to the tumour. This results in localised delivery of chemotherapy and ischemic necrosis of the tumour tissue.
SIRT involves the intra-arterial delivery of radioactive microspheres directly to the tumour site, resulting in targeted radiation therapy. This approach minimises systemic radiation exposure and spares healthy liver tissue, making it an effective treatment option for patients with unresectable liver metastases from PNETs.
Radiotherapy plays a minimal role in the treatment of Pancreatic Neuroendocrine Tumours (PNETs), particularly in cases where surgery is not possible or when residual tumour remains after surgery. It involves the use of high-energy radiation to target and destroy cancer cells, either as a standalone treatment or in combination with surgery and other therapies.
External beam radiation therapy is a localised treatment option occassionally used for PNETs. It delivers radiation from outside the body directly to the tumour site, allowing for precise targeting while minimising damage to surrounding healthy tissues. The treatment duration and intensity vary depending on factors such as
Potential side effects of external beam radiation therapy may include fatigue, skin irritation, nausea, and temporary changes in bowel habits. These side effects are typically manageable and tend to resolve after treatment completion. However, patients should be closely monitored throughout their radiation therapy course to ensure optimal symptom management and treatment adherence.
Combining different treatment modalities, such as surgery, chemotherapy, targeted therapy, and radiation, forms the cornerstone of multimodal approaches to PNET treatment. This comprehensive strategy aims to maximise treatment efficacy while minimising adverse effects, offering patients the best chance of disease control and improved outcomes.
Participation in clinical trials is of paramount importance in advancing our understanding of PNETs and exploring novel treatment approaches. Clinical trials provide access to cutting-edge therapies and allow patients to contribute to the development of new treatment paradigms. In Australia, ongoing clinical trials for PNETs focus on evaluating the efficacy and safety of emerging therapies, including targeted agents, immunotherapy, and combination treatment regimens.
In cases of advanced PNETs where treatment options are limited, palliative care plays a critical role in enhancing patients’ quality of life and providing symptom relief. Palliative care ensures holistic support for patients and their families, focusing on addressing
Managing symptoms such as pain and hormonal imbalances is central to palliative care in PNETs. Pain management strategies may include medication, nerve blocks, and complementary therapies to alleviate discomfort and improve overall well-being. Hormonal imbalances associated with functional PNETs can be addressed through medication and hormonal therapy, aimed at controlling hormone secretion and symptom burden.
Emphasising a holistic approach to patient care underscores the importance of addressing not only physical symptoms but also psychosocial and spiritual needs. Integrating supportive services, such as counselling, nutrition support, and symptom management, promotes comprehensive care and enhances patients’ overall quality of life.
Long-term follow-up care for PNETs allows patients and their care teams to:
Regular follow-up appointments, including imaging studies (such as PET scans, CT scans or MRIs) and blood and urine tests, enable healthcare providers to assess disease status and intervene promptly if necessary.
The monitoring process involves close collaboration between patients, oncologists, and multidisciplinary care teams to ensure continuity of care and optimal treatment outcomes. Emphasising the importance of ongoing surveillance empowers patients to take an active role in their healthcare journey and facilitates early detection of disease progression or treatment-related complications.
The management of pancreatic neuroendocrine tumours requires a multifaceted approach encompassing radiotherapy, multimodal treatment strategies, palliative care, and vigilant follow-up care. By prioritising holistic patient care and proactive monitoring, healthcare providers can optimise outcomes and improve the quality of life for individuals affected by PNETs.
Neuroendocrine Cancer Australia advocates for the treatment and support of all people affected by neuroendocrine tumours. If you’d like to speak to someone about your situation and receive advice specific to your condition, get in touch with our NET nurse service.