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VIPoma syndrome.Am
J Med. 1987 May 29;82(5B):37-48.
Since the
description of the watery diarrhea syndrome by Verner and Morrison 29
years ago, clinical and experimental observations have elucidated the
pathophysiology of this disease. Vasoactive intestinal polypeptide
(VIP) is produced and released by a tumor of the pancreatic islets or
by a tumor of neural crest origin such as a ganglioneuroma. Under
normal conditions, current evidence suggests that VIP is a
neurotransmitter in the central and peripheral nervous systems and
particularly in the peptidergic nervous system. The low VIP plasma
concentration observed in healthy subjects is viewed as a neuronal
overflow since it has been impossible to ascertain any endocrine role
for circulating VIP. Markedly elevated VIP plasma levels in the VIPoma
syndrome lead to intestinal secretion with severe secretory diarrhea,
resulting in hypovolemia, hypokalemia, and acidosis. These symptoms
subside after successful tumor removal. Approximately 50 percent of
patients have metastatic spread at the time of diagnosis. For these
patients, a new and promising therapeutic modality is available in the
form of a subcutaneously administered somatostatin analogue that
relieves symptoms through potent inhibition of VIP release from tumor
tissue.
The morphology and
neuroendocrine profile of pancreatic epithelial VIPomas and
extrapancreatic, VIP-producing, neurogenic tumors.Ann
N Y Acad Sci. 1988;527:508-17.
The histology,
histochemistry, and ultrastructure of 43 VIP-producing tumors (34 from
the pancreas, one jejunal, six retroperitoneal and two mediastinic),
37 of which were associated with the WDHA syndrome, have been
investigated on paraffin sections of primary or metastatic tumor
tissue. The pancreatic and jejunal tumors showed all structural and
secretory patterns of epithelial endocrine tumors, including
expression of cytokeratin, neuroendocrine markers like neuron-specific
enolase, chromogranins and synaptophysin, peptides like VIP, PHM, GRH,
PP, insulin, neurotensin, glucagon, somatostatin and enkephalin,
secretory granules, small clear vesicles, peculiar osmiophilic bodies,
and occasional formation of tubules or microacini with specialized
luminal surfaces. All the remaining tumors were neurogenic, showing
either neurons and nerve fibers together with Schwann cells (ganglioneuromas
and ganglioneuroblastomas) or endocrine cells (pheochromocytomas)
reacting with VIP, PHM, NPY, enkephalin, somatostatin, neuron-specific
enolase, synaptophysin, and MAP2 (but not cytokeratin, PP, or GRH)
antibodies. A possible origin of pancreatic VIPomas from transformed
pancreatic PP cells or ductular stem cells partially committed to
differentiation along the PP cell line is suggested.
Radiofrequency
ablation has a valuable therapeutic role in metastatic VIPoma.Pancreatology.
2006;6(1-2):155-9.
BACKGROUND:
Vasoactive intestinal peptide-secreting tumours (VIPomas) are rare
islet cell tumours of the pancreas that can result in life-threatening
biochemical abnormalities. The optimal intervention for metastatic
VIPoma remains undecided. This case history documents the clinical
role of radiofrequency (RF) ablation in the treatment of metastatic
VIPoma. CASE HISTORY: A primary pancreatic VIPoma was diagnosed in a
61-year-old female in 1998 and a distal pancreatectomy and splenectomy
were performed. She remained disease-free for 44 months when she
presented as an emergency with watery diarrhoea, hypokalaemia, renal
failure and an elevated serum VIP level. CT scanning showed a liver
metastasis and open RF ablation was performed with complete resolution
of symptoms and biochemistry within 48 h. Post-ablation imaging
confirmed complete ablation of the metastasis. She remained
disease-free until 22 months later when watery diarrhoea resumed and a
new hepatic metastasis was seen on CT. Percutaneous RF ablation was
performed and follow-up CT scan showed complete ablation of the
metastasis. The patient remains disease- and symptom-free 10 months
after the second RF ablation. CONCLUSION: This case illustrates that
the pronounced clinical and biochemical upset caused by metastatic
VIPoma can be resolved safely, quickly and repeatedly by RF ablation.
Large cell
carcinoma with calcitonin and vasoactive intestinal
polypeptide-associated Verner-Morrison syndrome.Mayo
Clin Proc. 2005 Jan;80(1):116-20.
Verner-Morrison
syndrome, characterized by diarrhea, hypokalemia, and hypochlorhydria,
is caused most commonly by vasoactive intestinal polypeptide-secreting
islet cell tumors of the pancreas. Verner-Morrison syndrome has not
been described as a paraneoplastic syndrome in non-small cell lung
cancer. We describe a 38-year-old man with metastatic non-small cell
lung cancer of large cell carcinoma with neuroendocrine
differentiation who presented with bone metastasis and intractable
secretory diarrhea that was unresponsive to pharmacological treatment,
including octreotide. Laboratory evaluation indicated elevated serum
calcitonin and vasoactive intestinal polypeptide levels. Chemotherapy
resulted in a transient response in the patient's diarrhea and
neuroendocrine markers. The patient did not respond to further therapy
and died 5 months after onset of back pain. To our knowledge, this is
the first published case of large cell carcinoma with neuroendocrine
differentiation associated with treatment-responsive paraneoplastic
Verner-Morrison syndrome.
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