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.
Most functional phaeochromocytomas/paragangliomas produce noradrenaline and/or adrenaline. Those that produce dopamine are rare. We describe the distinguishing clinical features of dopamine-secreting phaeochromocytomas and paragangliomas from those that secrete noradrenaline/adrenaline and the impact on their management. We present a case of a dopamine-secreting paraganglioma from our institution and review 14 case reports of dopamine-secreting phaeochromocytomas/paragangliomas published between 1984 and 2008. As observed in the literature, 80% of the tumours were extra-adrenal. Most patients presented with non-specific symptoms or mass effect without the classical presentation of catecholamine excess. The majority were diagnosed with urinary or plasma dopamine. Five patients had malignant tumours and 12 patients underwent surgical resection of the primary tumours. Unlike noradrenaline/adrenaline-secreting phaeochromocytomas/paragangliomas, dopamine-secreting tumours lack a classical presentation, are extra-adrenal and have a higher malignant potential. A routine inclusion of urinary or plasma dopamine as part of catecholamine screening in all suspected phaeochromocytomas and paragangliomas is recommended.
INTRODUCTION: Osteoporosis and osteopenia are well-recognized complications of inflammatory bowel disease. Previous studies have suggested that vitamin D deficiency is an important risk factor for the development of osteoporosis. We hypothesized that low vitamin D levels is the main reason for reduced bone mineral density in patients with inflammatory bowel disease. We aimed to study its potential role in Malaysia, which is a tropical country with 3 large ethnic groups. We also sought to examine the relationship between fracture risk and bone mineral density in this group.
METHODOLOGY: Relevant history as well as 25-hydroxycholecalciferol (vitamin D) levels and bone mineral density were obtained. Normal, inadequate, and low vitamin D levels were defined as 61-160 nmol/L (24-64 ng/mL), 30-60 nmol/L (12-24 ng/mL), and less than 30 nmol/L (<12 ng/mL), respectively.
RESULTS: Seventy-two patients were recruited. The prevalence of osteopenia and osteoporosis, respectively, were 58% and 17% in the spine and 51% and 14% in the hip. Mean vitamin D level in the group was low at 45.12 ± 17.4 nmol/L (18.05 ± 6.96 ng/mL), but there was no significant association between bone mineral density and vitamin D level. Twelve patients (16.7%) had a fragility fracture after the diagnosis of inflammatory bowel disease. The cumulative fracture incidence was 10% at 5 years and 35% at 10 years. There was a statistically significant association between osteoporosis of hip and a history of fracture (odds ratio 5.889; 95% confidence interval 1.41-24.53, P = .009).
CONCLUSION: Osteoporosis is prevalent among Malaysian patients with inflammatory bowel disease and is associated with a 6-fold increased risk of fractures. Most inflammatory bowel disease patients had inadequate or low vitamin D levels, but there was no association between vitamin D levels and BMD.