Neuroendocrine Tumor
Rare tumors arising from hormone-producing cells of the pancreas with variable behavior.
Overview
Pancreatic neuroendocrine tumors (PNETs) arise from islet cells and are much less aggressive than pancreatic adenocarcinoma. They can be functional (producing hormones) or non-functional. Even metastatic PNETs have relatively good prognosis with multimodal treatment.
Causes
- Mostly sporadic
- MEN1 syndrome (Multiple Endocrine Neoplasia type 1)
- Von Hippel-Lindau disease
- Tuberous sclerosis
- Neurofibromatosis type 1
Risk Factors
- MEN1 syndrome
- Family history of PNET
- Von Hippel-Lindau disease
- No known modifiable risk factors
Symptoms
Diagnosis
- CT/MRI (hypervascular on arterial phase)
- EUS with FNA
- Chromogranin A (tumor marker)
- Specific hormone levels (insulin, gastrin, etc.)
- Ga-68 DOTATATE PET (somatostatin receptor imaging)
- Ki-67 index for grading (G1/G2/G3)
Treatment Options
Surgical Treatment
Surgery is the only curative treatment for localized PNETs. Small (<2cm), low-grade tumors: enucleation (tumor removal preserving pancreatic tissue). Larger tumors: formal pancreatic resection (distal pancreatectomy or Whipple). Even liver metastases may be resectable. Dr. Bhagat performs parenchyma-sparing approaches when oncologically safe.
Recovery
Enucleation: 4-5 days hospital stay. Formal resection: 5-10 days depending on extent. Long-term follow-up with imaging and chromogranin A. Somatostatin analogues for unresectable disease control symptoms for years.
Prevention
- No known prevention for sporadic cases
- Genetic screening for MEN1 families
- Regular surveillance for known MEN1/VHL patients
Frequently Asked Questions
Are pancreatic NETs cancer?
What is the difference between PNET and pancreatic cancer?
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