vasoactive-intestinal-peptide and Retroperitoneal-Neoplasms

A literature review was conducted in relation to a case of chronic diarrhea associated with a VIP (vasoactive intestinal polypeptide) producing ganglioneuroblastoma (GNB), in an 18-month old female baby. This is a rare entity characterized by premonitory, persisting diarrhea, causing fluid and electrolyte changes typical of the WDHA syndrome, associating watery diarrhea, hypokalemia, and achlorhydia. Elevated VIP plasma levels are an indication for an echographic and/or CT-scan search for the causal secreting tumor. Although the prognosis of this condition seems favorable, the recommended treatment is surgery. The VIP substance represents an excellent biological monitoring marker. Ganglioneuroblastomas are tumors of the sympathetic nervous system, which, according to Pearse's cell and embryologic theory (1966), have to be linked to the APUD system tumors (paraneuromas). VIP-producing forms are rare in children, and only 29 case studies have been compiled in the literature since 1970, when the VIP substance was discovered. The case reported in this study illustrates the diagnostic problems raised by such lesions, and allows us to confirm VIP's imputability for the occurrence of the chronic diarrhea condition in this child.

Topics: Chronic Disease; Diarrhea, Infantile; Female; Ganglioneuroma; Humans; Infant; Retroperitoneal Neoplasms; Vasoactive Intestinal Peptide

Neuroblastoma (NB) is the most common extracranial solid tumor in children. Diarrheal NB is quite rare and is not easy to diagnose in the early stage. Six cases of diarrheal NB in our hospital treated from 1996 to 2006 were retrospectively analyzed, including characteristics such as electrolyte imbalance, pathologic features, vasoactive intestinal peptide (VIP) immunohistochemical staining results, treatment, and prognosis. All patients were boys with 3-8 loose or watery stools each day and routine fecal tests were normal. Abdominal tumors were identified by B-ultrasound. Drugs were ineffective. Three patients underwent surgery, and the remaining three patients received surgery and chemotherapy. Diarrhea stopped after treatment in five patients. Two patients died due to intractable hypokalemia. The tumor was located in the adrenal gland in four patients, in the upper retroperitoneum in one patient, and in the presacral area in one patient. Pathologic findings were NB and ganglioneuroblastoma. Five patients were at clinical stage I-II, and one was at stage III. Four patients survived (followed-up for 6 mo to 4 years). Immunohistochemical staining for VIP was positive. Refractory diarrhea is a paraneoplastic syndrome of NB and is rare. Patients aged 1-3 years who present with chronic intractable diarrhea should be followed closely. Intractable diarrhea, hypokalemia, and dysplasia are the initial clinical manifestations. Increased VIP is characteristic of this disease. Potassium supplementation plays a vital role in the treatment procedure, especially preoperatively. The prognosis of diarrheal NB is good following appropriate treatment.

Topics: Adrenal Gland Neoplasms; Biomarkers, Tumor; Biopsy; Child, Preschool; Diarrhea; Humans; Hypokalemia; Immunohistochemistry; Infant; Male; Neoplasm Staging; Neuroblastoma; Paraneoplastic Syndromes; Retroperitoneal Neoplasms; Retrospective Studies; Treatment Outcome; Vasoactive Intestinal Peptide

It has been reported that vasoactive intestinal peptide (VIP)-producing tumors accompanied by watery diarrhea, hypokalemia, and achlorhydria (WDHA) syndrome often produce multiple hormones biochemically and immunohistochemically.. The authors examined the distribution of several peptides--VIP, peptide histidine methionine (PHM), neuropeptide Y (NPY), methionine-enkephalin (M-EK), somatostatin (SS), substance-P (Sub-P), corticotropin-releasing hormone, and tyrosine hydroxylase--with immunohistochemical studies and an in situ hybridization method in three ganglioneuroblastomas with WDHA syndrome. All patients had an extremely elevated plasma level of VIP.. Peptides examined immunohistochemically in the tumor were all detectable in ganglionic cells and some neurites. The coexistence of those peptides was observed with serial section staining. The presence of messenger RNA of VIP/PHM-27, NPY, and SS was detectable in the cytoplasm of the tumor cells with the in situ hybridization.. It was shown that multiple genes of peptides are expressed simultaneously and translated to proteins in those tumors.

Topics: Child, Preschool; Corticotropin-Releasing Hormone; Enkephalin, Methionine; Female; Ganglioneuroma; Gene Expression Regulation, Neoplastic; Humans; In Situ Hybridization; Infant; Male; Neuropeptide Y; Peptide PHI; Retroperitoneal Neoplasms; Somatostatin; Substance P; Tyrosine 3-Monooxygenase; Vasoactive Intestinal Peptide; Vipoma

A 18-month-old boy with stage 4 neuroblastoma needed intensive care because of prerenal acute renal failure related to an intractable watery diarrhoea syndrome occurring 10 months after the diagnosis of the primary tumor. This diarrhoea was in relation with a late hyperproduction of vasoactive intestinal peptide by the relapsing neuroblastoma itself and stopped with intravenous somatostatin administration.

Topics: Diarrhea, Infantile; Humans; Infant; Male; Neoplasm Invasiveness; Neoplasm Recurrence, Local; Neuroblastoma; Retroperitoneal Neoplasms; Vasoactive Intestinal Peptide

Topics: Adenoma, Islet Cell; Adult; Female; Ganglioneuroma; Humans; Neoplasms, Multiple Primary; Pancreatic Neoplasms; Retroperitoneal Neoplasms; Vasoactive Intestinal Peptide; Vipoma

A ganglioneuroblastoma was excised at surgery from a 1-yr-old girl with severe watery diarrhea. The tumor, weighing 1 g, was extracted in trifluoracetic acid and contained 8.3 nmol immunoreactive vasoactive intestinal peptide. The peptide was isolated by affinity chromatography and high pressure liquid chromatography and was found to be identical to porcine vasoactive intestinal peptide by amino acid analysis and microsequence analysis.

Topics: Amino Acid Sequence; Amino Acids; Chemical Phenomena; Chemistry; Female; Ganglioneuroma; Humans; Infant; Radioimmunoassay; Retroperitoneal Neoplasms; Vasoactive Intestinal Peptide; Vipoma

Topics: Adenoma, Islet Cell; Adrenal Gland Neoplasms; Child; Child, Preschool; Chronic Disease; Female; Ganglioneuroma; Hormones, Ectopic; Humans; Male; Retroperitoneal Neoplasms; Vasoactive Intestinal Peptide; Vipoma

Topics: Adrenal Gland Neoplasms; Child, Preschool; Female; Ganglioneuroma; Hormones, Ectopic; Humans; Infant; Male; Retroperitoneal Neoplasms; Vasoactive Intestinal Peptide

A 23-month-old girl with intractable diarrhea that had persisted for the past 6 months showed typical evidence of water-diarrhea-hypokalemia-achlorhydria (WDHA) syndrome. Serum vasoactive intestinal peptide (VIP)-like immunoactivity was very high, and urine homovanillic acid and noradrenaline were also abnormally increased. A calcified tumor in the right paravertebral region was noted radiologically. Soon after resection of the tumor, the diarrhea dramatically improved. A ganglioneuroblastoma was histologically confirmed. Serum VIP decreased to normal level at 1 h after the removal of the tumor. VIP activity in the tumor was extraordinarily high, and the cells were stained by the indirect immunofluorescence technique with anti-VIP serum. This is to report the rapid turnover of serum level of VIP after resection of ganglioneuroblastoma with WDHA syndrome in a child.

Topics: Achlorhydria; Child; Child, Preschool; Diarrhea, Infantile; Female; Ganglioneuroma; Gastrointestinal Hormones; Humans; Hypokalemia; Infant; Male; Retroperitoneal Neoplasms; Syndrome; Time Factors; Vasoactive Intestinal Peptide

Topics: Adult; Catecholamines; Child; Child, Preschool; Diarrhea; Female; Ganglioneuroma; Gastrointestinal Hormones; Humans; Infant; Retroperitoneal Neoplasms; Vasoactive Intestinal Peptide

A case of adult ganglioneuroma-pheochromocytoma with an associated watery diarrhea syndrome is reported. High levels of vasoactive intestinal peptide (VIP) were found in preoperative serum and in tumor tissue. The serum VIP levels fell to normal, and the watery diarrhae syndrome completely ceased following removal of the tumor. In addition to containing VIP, the tumor was rich in catecholamines, and calcitonin. Peptide hormone-containing extracts and catecholamine extracts from the tumor both activated the adenyl cyclase system and increased lipolytic activity in a preparation of isolated rat fat cells. The findings in this patient further link VIP with neural crest tissues, and suggest the importance of determining catecholamine levels in patients with the watery diarrhea syndrome.

Topics: Acidosis; Adenylyl Cyclases; Adipose Tissue; Adult; Calcitonin; Catecholamines; Diarrhea; Female; Ganglioneuroma; Gastrointestinal Hormones; Humans; Hypokalemia; In Vitro Techniques; Pheochromocytoma; Retroperitoneal Neoplasms; Syndrome; Vasoactive Intestinal Peptide