VIPoma syndrome: challenges in management.
Vasoactive intestinal peptide-producing tumour (VIPoma) or Verner-Morrison syndrome is a very rare neuroendocrine tumour. It occurs in less than ten percent of all pancreatic islet cell tumours, and about 70 percent to 80 percent of these tumours originate from the pancreas. Diagnosis is characteristically delayed. The first-line treatment is surgical. It may be curative in forty percent of patients with benign and non-metastatic disease. Palliative surgery is indicated in extensive disease, followed by conventional somatostatin analogue (octreotide) therapy. Somatostatin analogues improve hormone-mediated symptoms, reduce tumour bulk and prevent local and systemic effects. We present a female patient with VIPoma syndrome, which had metastasised to the liver at diagnosis. The patient underwent palliative Whipple procedure and subsequent cytoreductive radiofrequency ablations to her liver metastases. Unfortunately, after symptomatic improvement for three years, her disease progressed. Currently, she is on daily octreotide, achieving partial control of her symptoms.
Adam
N
N
Endocrine Unit, Department of Medicine, University of Malaya, Lembah Pantai, Kuala Lumpur 50603, Malaysia.
Lim
S S
SS
Ananda
V
V
Chan
S P
SP
D017115:Catheter Ablation / Q000379:methods; D003131:Combined Modality Therapy; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008113:Liver Neoplasms / Q000556:secondary* / Q000601:surgery; D008875:Middle Aged; D009361:Neoplasm Invasiveness / Q000473:pathology; D009367:Neoplasm Staging; D015282:Octreotide / Q000627:therapeutic use; D010166:Palliative Care / Q000379:methods; D010190:Pancreatic Neoplasms / Q000473:pathology* / Q000628:therapy; D013997:Time Factors; D016896:Treatment Outcome; D003969:Vipoma / Q000473:pathology / Q000556:secondary* / Q000628:therapy
D015282:Octreotide