Pancreatic Neuroendocrine Tumours

Pancreatic neuroendocrine tumours (PNETs), once called islet cell tumours, grow from the hormone-producing cells of the pancreas. They make up only a few percent of pancreatic tumours and generally grow far more slowly than ordinary pancreatic cancer, though their behaviour varies from indolent to aggressive.

Most do not release hormones and are found by chance or because of their size. A minority are functioning tumours, such as insulinoma and gastrinoma, which produce striking symptoms from the excess hormone. Treatment is tailored to the grade, size, spread and hormone activity, and ranges from observation to surgery and specialised drug or radionuclide therapy.

Symptome

• No symptoms, with a nodule found on a scan done for another reason
• Episodes of sweating, shakiness, confusion or fainting relieved by eating (insulinoma)
• Recurrent stomach or duodenal ulcers with diarrhoea (gastrinoma)
• Profuse watery diarrhoea with low potassium (VIPoma)
• A distinctive migrating red rash with weight loss and new diabetes (glucagonoma)
• Abdominal or back pain, jaundice or weight loss from a larger tumour
• Flushing and diarrhoea when there is spread to the liver

Ursachen und Risikofaktoren

Most PNETs occur sporadically without a known cause. About one in ten is part of an inherited syndrome, most importantly multiple endocrine neoplasia type 1 (MEN1), which also causes parathyroid and pituitary tumours, and less often von Hippel-Lindau disease, neurofibromatosis type 1 or tuberous sclerosis. Inherited cases tend to be multiple and appear at a younger age. Pathologists grade these tumours from 1 to 3 by how quickly the cells divide (the Ki-67 index), and grade is the strongest guide to behaviour.

Wie es diagnostiziert wird

• Contrast CT or MRI: These tumours take up contrast avidly in the arterial phase, which helps identify them and any liver deposits.
• Endoscopic ultrasound with biopsy: Finds small tumours, particularly insulinomas, and provides tissue for grading.
• Gallium-68 DOTATATE PET-CT: A scan that targets somatostatin receptors on the tumour cells. It stages the disease and shows whether radionuclide therapy could work.
• Hormone blood tests: Chromogranin A, plus insulin and C-peptide during a supervised fast, gastrin or other hormones depending on symptoms.
• Genetic testing: Advised for young patients, multiple tumours or a relevant family history, to look for MEN1.

Behandlungsmöglichkeiten

• Surveillance: Small, low-grade, non-functioning tumours under about 2 centimetres can often be monitored with periodic scans.
• Surgery: Enucleation of the tumour alone, distal pancreatectomy or a Whipple operation, depending on site and size. Functioning tumours are removed when possible, which usually stops the symptoms.
• Somatostatin analogues: Monthly octreotide or lanreotide injections slow growth and control hormone symptoms.
• Peptide receptor radionuclide therapy (PRRT): Lutetium-177 DOTATATE infusions, typically 4 sessions about 8 weeks apart, for receptor-positive advanced tumours.
• Targeted drugs and chemotherapy: Everolimus, sunitinib, or capecitabine with temozolomide for progressive disease. Grade 3 carcinomas receive platinum-based chemotherapy.
• Liver-directed treatment: Resection, ablation or embolisation of liver deposits to reduce tumour load and hormone output.

Wenn es dringend ist

Low blood sugar from an insulinoma can cause confusion, seizures or loss of consciousness: give sugar if the person can swallow and call emergency services if they do not recover promptly. Severe watery diarrhoea with weakness or palpitations, vomiting blood or black stools, and jaundice with fever all need urgent local hospital care.

Reisen nach Türkiye zur Behandlung

These tumours usually grow slowly, which leaves time for a considered opinion. Travel can be worthwhile for DOTATATE PET-CT, which is not available everywhere, for review by a neuroendocrine tumour team, for planned surgery with about 2 to 3 weeks' stay, or for PRRT, where each session needs only a few days but the sessions are 8 weeks apart and blood counts must be checked in between at home. Monthly injections and scan surveillance over many years should be run by your local team.

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