Ganglioneuroblastoma containing several kinds of neuronal peptides with watery diarrhea syndrome.
This report presents an adrenal ganglioneuroblastoma containing several kinds of neuronal peptides. The tumour was found in the autopsy case of a 3-year-old girl with clinical manifestation of intractable diarrhea, hypokalemia, achlorhydria, and with elevated levels of plasma vasoactive intestinal peptide (VIP). Immunoperoxidase staining showed many immunoreactive VIP- containing cells, some somatostatin-and substance P-containing cells on the tumour sections. Ultrastructurally, the tumour cells contained numerous secretory granules that could be divided mainly two types; one is a small cored vesicle (50-150 nm in diameter) and the other large electron dense secretory granule (200-500 nm in diameter). It was suggested that the cells in ganglioneuroblastoma derived from neural crest are closely related to the cells that could differentiate into gut-hormone-producing cells.
D000310:Adrenal Gland Neoplasms / Q000032:analysis* / Q000150:complications; D002675:Child, Preschool; D003967:Diarrhea / Q000209:etiology*; D005260:Female; D005729:Ganglioneuroma / Q000032:analysis* / Q000150:complications; D006651:Histocytochemistry; D006801:Humans; D007124:Immunoenzyme Techniques; D008854:Microscopy, Electron; D010455:Peptides / Q000032:analysis*; D013577:Syndrome; D014660:Vasoactive Intestinal Peptide / Q000032:analysis; D003969:Vipoma / Q000473:pathology
D010455:Peptides; D014660:Vasoactive Intestinal Peptide