
Pancreatic neuroendocrine tumours (PNETs) are a rare group of tumours arising from the neuroendocrine cells of the pancreas that produce hormones, including insulin, glucagon, somatostatin, etc. These tumours may be benign or malignant, and they usually grow more slowly than other pancreatic tumours, most notably pancreatic adenocarcinoma.
Causes
Risk Factors
Pancreatic neuroendocrine tumors (PanNETs) in Malaysia are coming under treatments based on a blend of classical and new methods. Laparoscopic and robotic-assisted surgeries are becoming more common approaches. In distant metastasis or tumors not amenable to surgery, targeted therapies are employed for anti-tumour growth activity. Advanced imaging techniques help localise and assess the tumor accurately. Interdisciplinary treatment planning teams, consisting of the oncologist, surgeon, radiologist, and endocrinologist, actively work together to develop individual treatment plans. Thus, patients participating in international clinical trials will have early access to novel therapies to aid global understanding.
The medical treatment for Pancreatic Neuroendocrine Tumors requires evaluating the patient's severity and medical condition. The following are the treatment options:
Surgical Resection: Surgical tumour removal is the most effective treatment option for localised PNETs. Thus, surgery provides the best chance of cure or long-term control in the case of a tumour localised to the pancreas.
Targeted Therapy : Targeted therapies inhibit tumour growth in advanced-stage or metastatic PNET cases. These therapies help slow down disease progression and enhance survival.
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Chemotherapy: Chemotherapy with medications is used in aggressive or metastatic PNETs. Chemotherapy is most often used when surgery or targeted therapy proves ineffectual in curbing the disease.
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Whipple Procedure (Pancreaticoduodenectomy) : This procedure is performed for localised and resectable tumours. It is intended for functional or non-functional PNETs localised within the pancreas.
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Stereotactic Radiotherapy (SRT) is the treatment modality for localised, inoperable, or metastatic PNETs when surgery is not performed. It is also for patients who cannot undergo surgery or whose disease has already spread to other sites.
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CyberKnife : The CyberKnife system is typically beneficial in managing small to medium-sized tumours that have proven inoperable, are subject to difficult surgical excision, or have metastasised into surrounding organs or tissue planes.
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Proton Therapy : Proton therapy is for such out-of-sight or inoperable PNETs. Given its significance in tumours near critical structures such as blood vessels or the spine, it is also applicable in treatment for metastatic PNETs when the cancer has spread elsewhere.
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These are the standard diagnostic methods for diagnosing Pancreatic Neuroendocrine Tumors:
Imaging Studies
Biopsy
Blood Tests
Somatostatin Receptor Scintigraphy (SRS)
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Yes, surgeries in robotic and laparoscopic procedures are practised primarily for faster recovery.
The duration of treatment varies according to tumor type and stage but usually lasts several weeks to several months.
The prognosis of PNETs is based on the tumour's size, location, functional status, and the presence of metastases. Early detection and surgery are the best options for offering a good prognosis, whereas therapies must be combined with advanced or metastatic cases to manage PNETs.
Most PNETs are sporadic, although they can be combined with some genetic conditions, such as MEN1 syndrome and von Hippel-Lindau disease. Individuals with family histories of these conditions might require genetic counselling and regular screening.
Currently, there is no known preventive measure against PNETs. A healthy lifestyle that avoids smoking and genetic screening for higher-risk individuals can contribute to early detection and improved management of the disease.
Even after treatment, the PNETs can recur depending on whether the tumour was removed entirely or metastasised. Regular follow-up scans and monitoring are very crucial to detect recurrences at an early stage.
The treatment method used will determine the long-term effects. Surgical recovery can cause digestive problems, chemotherapy has intermediate side effects such as nausea and lethargy, and targeted therapies can cause skin rashes or fatigue. Regular follow-up care must ensure monitoring for recurrence or complications.
A nutritious diet, physical exercise, and abstinence from alcoholic beverages and smoking may enhance a patient's health. Hormone-secreting tumours may also require an individual to follow directed medical advice in managing portions of low blood sugar or digestive issues.
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