vasoactive-intestinal-peptide and Soft-Tissue-Neoplasms

A 3 year old Chinese girl with watery diarrhoea, abdominal distension and hypokalaemia due to a thoracic paraspinal vasoactive intestinal peptide (VIP) secreting ganglioneuroma is reported. The pre-operative serum VIP was 314 pmol/l (normal less than 30). Her diarrhoea stopped after the removal of the tumour. The VIP was 14 pmol/l 6 months post-operatively. Review of the 19 reported cases in children with documented elevated serum VIP showed that many of the cases presented with watery diarrhoea for prolonged duration before the diagnosis was made. Earliest age of onset was 2 weeks of age. The male to female ratio was 9:10. Ganglioneuroma and ganglioneuroblastoma were the commonest tumours. Pancreatic non-beta cell hyperplasia and neurofibroma were also reported. Location of the tumour was variable: neck, chest or abdomen. Increased urinary catecholamine excretion was reported in 50% of the cases. Abdominal distension, flushing, episodic hypertension and failure to thrive were the other associated features.

Topics: Adenoma, Islet Cell; Child; Child, Preschool; Female; Ganglioneuroma; Humans; Soft Tissue Neoplasms; Vasoactive Intestinal Peptide; Vipoma

Intramuscular hemangiomas are idiopathic lesions which are either tumoral or developmental in origin. A close association of abnormal blood vessels with nerve fibers is found and may suggest that nerves have a primary inciting role in the development of these lesions. In the current study, the number of nerve fibers in different zones around the tumors, as well as the type of neuropeptides present in these fibers, was quantitatively assessed by computer-assisted image analysis of immunohistochemical staining of histological slides. The number of nerve fibers as determined by positive staining by anti-protein S-100 antibodies was found to be elevated in the immediate vicinity of the abnormal blood vessels. The density of the nerve fibers rapidly declined with increasing distance from the hemangiomas, reaching normal values at distances of over 2 mm. Furthermore, hemangiomas contain a significantly higher number of calcitonin gene-related peptide (CGRP), substance P, and Met-enkephalin-positive fibers. The most significant rise in number is that of CGRP-positive fibers. This neuropeptide is a known mitogen, which could be responsible for the growth of the hemangiomatous blood vessels. Substance P is a nociceptive neurotransmitter and its presence can explain the pain which often accompanies even tiny intramuscular hemangiomas.

Topics: Adolescent; Adult; Calcitonin Gene-Related Peptide; Child; Enkephalin, Leucine; Enkephalin, Methionine; Hemangioma; Humans; Immunohistochemistry; Muscles; Muscular Diseases; Nerve Fibers; Neuropeptides; S100 Proteins; Soft Tissue Neoplasms; Substance P; Vasoactive Intestinal Peptide