Vipoma

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Vipoma
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Overview

VIPomas are neuroendocrine tumors that secrete vasoactive intestinal peptide, originate in APUD (amine precursor uptake and decarboxylation) cells present in the gastroenteropancreatic system and in adrenal or extra-adrenal neurogenic sites. The symptoms of VIPoma were described for the first time in 1958, when Verner and Morrison described a syndrome of watery diarrhea, hypokalemia, and achlorhydria. In 90% of cases, VIPomas arise from the pancreas, but they may also be present in periganglionic tissue or at colon, bronchus, adrenal glands, and liver, especially in children. VIPomas are almost always solitary, with less than 5% being multicentric. At the time of diagnosis, VIPomas are usually greater than 3 cm in diameter and are found mainly in the body and tail of the pancreas.

Causes:

The underlying cause of VIPomas is not completely known. It is believed that many factors, such as genetic and environmental, may have a role in disease etiology.

Signs and symptoms:

Main signs and symptoms include hypokalemia, secretory diarrhea, anorexia (loss of appetite), chronic fatigue, dehydration, diabetes mellitus, erythema, abdominal pain, hepatomegaly (enlargement of liver), generalized muscle weakness, hypercalcemia, muscle cramps, malabsorption, nausea, vomiting, malaise and weight loss.

Other signs, symptoms, findings and clinical features may include pituitary adenoma, prolactin excess, liver neoplasm, excess growth hormone, follicular thyroid carcinoma, ascites, jaundice, intrahepatic cholestasis, elevated calcitonin, hyperparathyroidism, subcutaneous lipoma and respiratory insufficiency.

Complications:

VIPomas can lead to multi organ failure, coma and death.

Diagnosis:

VIPomas are usually diagnosed in young children and middle-aged adults. Apart from the customary medical history and clinical examination, specific tests used to diagnose a VIPoma may include blood tests (including VIP level), imaging studies (abdominal CT scan or MRI), and examination of a stool sample. PET scan can detect the spread of tumor and its exact extent. Differential diagnoses include malabsorption syndrome, Crohn disease, ulcerative colitis, microscopic colitis, irritable bowel syndrome, and gastrointestinal infections.

Management:

Initial treatment of VIPomas is aimed at correction of volume and electrolyte abnormalities. Octreotide acetate is clinically effective in control of diarrhea in up to 90% of patients. Glucocorticoids are also associated with reduction in symptoms in 50%. In case of unresectable or progressive disease, systemic chemotherapy may be needed in the form of streptozocin, doxorubicin, fluorouracil, or a combination of these. Treatment of choice is surgical resection of the tumor. In advanced disease, tumor debulking may relieve some of the symptoms, but it is not curative or completely effective in all cases. Transarterial chemoembolization with chemotherapy-loaded materials may help with palliation in patients with extensive disease. External radiation therapy may be given in advanced, unresectable tumors. Targeted therapy may be used in advanced cases. The median survival in VIPomas is 96 months, but eventual, long-term survival depends hugely on the tumor grade, staging, and resectability of the tumor. Pharmacological measures include octreotide and lanreotide to stop the diarrhea and inhibit the secretion of VIP. The majority of metastatic VIPomas are not be cured by surgery, but prolonged survival is often possible due to the slow-growing nature of these tumors.

Other names of the condition:

Vasoactive intestinal peptide (VIP) tumor

Pancreatic VIPoma

Vasoactive intestinal peptide-producing tumor

Diarrheogenic Islet Cell Tumor

VIP-secreting tumor