No abstract available.
Diarrhea* ; Hypokalemia* ; Vipoma

Child, Preschool ; Female ; Humans ; Vasoactive Intestinal Peptide ; secretion ; Vipoma

The syndrome of watery diarrhea, hypokalemia, and achlorhydria (WDHA syndrome) is rare, and occasionally related with neurogenic tumors. A 20-month-old girl presenting symptoms of WDHA syndrome was transferred to our hospital. She had a ganglioneuroblastoma of adrenal gland. The serum level of VIP was elevated. After complete excision of tumor, all symptoms of WDHA syndrome were improved. The postoperative course was uneventful, and symptoms of WDHA syndrome disappeared. The serum level of VIP dropped to normal. The girl with postoperative chemotherapy and radiation therapy survived without evidence of disease for 33 months after complete resection.
Achlorhydria ; Adrenal Glands ; Diarrhea ; Drug Therapy ; Female ; Ganglioneuroblastoma* ; Humans ; Hypokalemia ; Infant ; Vipoma*

OBJECTIVE: To explore the diagnosis and surgical treatment for pancreatic vasoactive intestine polypeptide tumor (VIPoma). METHODS: Clinical data of 7 patients with VIPoma from Xiangya Hospital, Central South University between January 1990 and July 2011 were collected and analyzed retrospectively. RESULTS: The different operation modes were selected according to the location of VIPomas, and the postoperative symptoms of all 7 patients were gradually relieved and cured. The follow up showed that life spans of the above-mentioned patients were 3-6 years. CONCLUSION The incidence of pancreatic VIPoma is low but it is easy to misdiagnose. The excision for the tumor is the most effective therapy. Combining with somatostatin, intervention and other effective strategies, the life quality of patients can be improved and long-term survival may be achieved.
Humans ; Pancreatic Neoplasms ; diagnosis ; surgery ; Retrospective Studies ; Somatostatin ; Vipoma ; diagnosis ; surgery

Catheter Ablation ; adverse effects ; methods ; Humans ; Liver Neoplasms ; secondary ; surgery ; therapy ; Male ; Middle Aged ; Vipoma ; secondary ; surgery ; therapy

OBJECTIVETo discuss the experiences of diagnosis and treatment for vasoactive intestinal peptide-secreting-tumors (VIPoma) by summarizing clinical informations of 15 patients with VIPoma.

METHODSTo analyze Clinical manifestations, laboratory examinations, imaging features, operation, pathological findings and follow up survey of 15 patients, among them 1 case from our hospital and the other 14 cases were searched in chinese biological and medical literature database from Jan 1987 to Dec 2002.

RESULTSThe main clinical manifestation include periodical secretory watery diarrhea, hypokalemia, achlorhydria, in addition, periodical backache, skin rash, and polyps of colon were presented in the case in our hospital. The immunohistochemical expression of many kinds of digestive hormone including VIP presented positive; All clinical symptoms of which except polyps disappeared after operation, elevated VIP data in serum also markedly decreased. Part resection of superior mesenteric vein was performed in the same patient.

CONCLUSIONSVIPoma is rare. Typical symptoms and the serum value of VIP were keys to diagnosis, the operation is the most effective means for treatment. Resection of tumor, Radiofrequency tissue ablation, liver transplantation can be selected for metastatic VIPoma in the liver.

Adult ; Aged ; Female ; Humans ; Male ; Middle Aged ; Pancreatic Neoplasms ; diagnosis ; pathology ; therapy ; Vasoactive Intestinal Peptide ; secretion ; Vipoma ; diagnosis ; pathology ; therapy

Vasoactive intestinal polypeptide-secreting tumors (VIPomas) cause VIPoma syndrome, which is characterized by watery diarrhea, hypokalemia, and achlorhydria. The treatment options for metastatic VIPomas include somatostatin analogs, cytoreductive surgery, and chemotherapy. We report the case of a 54-year-old male who presented with a peripancreatic mass with multiple hepatic metastases on computed tomography. After resection, the peripancreatic mass was demonstrated pathologically to be a neuroendocrine tumor. Although the patient received systemic chemotherapy and somatostatin analogs for the hepatic metastatic masses, the tumor increased in size. The patient then experienced severe diarrhea, despite treatment with the somatostatin analogs. Elevated serum VIP levels (3,260 pg/mL) and typical symptoms confirmed the diagnosis of VIPoma. We performed hepatic artery embolization (HAE) to reduce the tumor volume and control his symptoms, which led to a very rapid symptomatic response. The patient has remained symptom-free for 18 months with repeated HAE.
Achlorhydria ; Diagnosis ; Diarrhea ; Drug Therapy ; Hepatic Artery* ; Humans ; Hypokalemia ; Liver* ; Male ; Middle Aged ; Neoplasm Metastasis* ; Neuroendocrine Tumors ; Somatostatin ; Tumor Burden ; Vipoma*

Pancreatic neuroendocrine tumors (PNETs), a group of endocrine tumors arising in the pancreas, are among the most common neuroendocrine tumors. The genetic causes of familial and sporadic PNETs are somewhat understood, but their molecular pathogenesis remains unknown. Most PNETs are indolent but have malignant potential. The biological behavior of an individual PNET is unpredictable; higher tumor grade, lymph node and liver metastasis, and larger tumor size generally indicate a less favorable prognosis. Endocrine testing, imaging, and histological evidence are necessary to accurately diagnose PNETs. A 4-pronged aggressive treatment approach consisting of surgery, locoregional therapy, systemic therapy, and complication control has become popular in academic centers around the world. The optimal application of the multiple systemic therapeutic modalities is under development; efficacy, safety, availability, and cost should be considered when treating a specific patient. The clinical presentation, diagnosis, and treatment of specific types of PNETs and familial PNET syndromes, including the novel Mahvash disease, are summarized.
Combined Modality Therapy ; Gastrinoma ; diagnosis ; therapy ; Glucagonoma ; diagnosis ; therapy ; Humans ; Insulinoma ; diagnosis ; therapy ; Multiple Endocrine Neoplasia Type 1 ; diagnosis ; therapy ; Neuroendocrine Tumors ; classification ; diagnosis ; therapy ; Pancreatic Neoplasms ; classification ; diagnosis ; therapy ; Vipoma ; diagnosis ; therapy ; von Hippel-Lindau Disease ; complications

MEN1 is characterized by the combined occurrence of tumors of the parathyroids, pancreatic islet cells and the anterior pituitary. Pancreatic islet cell tumors occur in 40% of MEN1 patients. Pancreatic polypeptidomas occur most commonly but they are asymptomatic. Gastrinomas are the second most common type. VIPomas are rare and there has been no report of a case in Korea so far. We encountered a case of pancreatic VIPoma in MEN Type I. A 49 year old man was referred from his local hospital presenting with a sudden onset of an explosive watery diarrhea of 3 months duration. Abnormal findings in his blood chemistry were hypercalcemia and hypokalemia. The 99mTc-sestamibi sintigraphy showed an increased uptake in right lower parathyroid gland. Abdominal CT demonstrated a mass of 6x4 cm in tail of the pancreas and multiple lesions in both hepatic lobes. Serum levels of VIP hormones were elevated. Subtotal parathyroidectomy and subtotal pancreatectomy were done. Postoperatively his symptoms were improved transiently, however the patient showed repetitive attacks of watery diarrhea. So in order to palliate his symptoms, an RF ablation of the metastatic liver masses was performed. After that therapy his clinical symptoms were reduced dramatically. Unfortunately, the patients condition worsened again. Despite of continuous octreotide therapy, interferon and two courses of combination chemotherapy, the hepatic metastases failed to regress and the patient died 10 months after the diagnosis of a metastatic VIPoma. This is the first report of pancreatic VIPoma in MEN type I in Korea.
Chemistry ; Diagnosis ; Diarrhea* ; Drug Therapy, Combination ; Gastrinoma ; Humans ; Hypercalcemia ; Hypokalemia ; Interferons ; Islets of Langerhans ; Korea ; Liver ; Male ; Middle Aged ; Multiple Endocrine Neoplasia Type 1* ; Multiple Endocrine Neoplasia* ; Neoplasm Metastasis ; Octreotide ; Pancreas ; Pancreatectomy ; Parathyroid Glands ; Parathyroidectomy ; Technetium Tc 99m Sestamibi ; Tomography, X-Ray Computed ; Vipoma

Multiple endocrine neoplasia I(MEN I) is a genetic disorder that consists of neoplasia of neuroendocrine type in the parathyroid glands, in the islets of Langerhans in the pancreas, and in the anterior pituitary gland. Primary hyperparathyroidism is the most common feature and occurs in approximately 95% of MEN I patients. Pancreatic islet cell tumors occur in 40% of MEN I patients. Most of these tumors produce excessive amounts of hormones, such as gastrin, insulin, glucagon and vasoactive intestinal polypeptide(VIP). VIP-producing pancreatic tumors(VIPoma) associated with MEN I are rare and so far only one has been reported in Korea. Recently, we came across a case of MEN I, associated VIPoma presented persistent hypercalcemia after a parathyroidectomy. A 70 year old man had suffered from large amount of watery diarrhea, severe general weakness and paralysis of lower limbs for 3 months which suggests symptoms of hypercalcemia. Before the patient visited our hospital, he underwent subtotal parathyroidectomy due to hyperparathyroidism. Even though he was operation, there was no subsidization of the symptoms and abnormal findings of blood chemistry such as hypercalcemia, hypocalemia were remained unchanged. However, the parathyroid hormone level was still within normal limits. Abdominal computerized tomography scan demonstrated a mass of 2.5cm diameter in tail of the pancreas. As serum level of VIP hormone was also elevated, distal pancreatectomy was carried out to performed. There was improvement in the symptoms towards the normal condition and the level of biochemical parameters such as serum potassium, calcium and VIP, were also within the normal limits. In a direct sequence analysis, GAC-->CAT(Asp-->His) point mutation, at codon 383 of exon 9 of the MEN I gene was identified in both the patient and his son. The authors report a rare case of VIPoma associated with MEN I with review of literature on MEN I.
Aged ; Calcium ; Chemistry ; Codon ; Diarrhea ; Exons ; Gastrins ; Germ-Line Mutation ; Glucagon ; Humans ; Hypercalcemia ; Hyperparathyroidism ; Hyperparathyroidism, Primary ; Insulin ; Islets of Langerhans ; Korea ; Lower Extremity ; Multiple Endocrine Neoplasia Type 1 ; Multiple Endocrine Neoplasia* ; Pancreas ; Pancreatectomy ; Paralysis ; Parathyroid Glands ; Parathyroid Hormone ; Parathyroidectomy ; Pituitary Gland, Anterior ; Point Mutation ; Potassium ; Sequence Analysis ; Vipoma*