MULTIPLE-HORMONE GENE-EXPRESSION IN GANGLIONEUROBLASTOMA WITH WATERY DIARRHEA, HYPOKALEMIA, AND ACHLORHYDRIA SYNDROME

Background. It has been reported that vasoactive intestinal peptide (V IP)-producing tumors accompanied by watery diarrhea, hypokalemia, and achlorhydria (WDHA) syndrome often produce multiple hormones biochemic ally and immunohistochemically. Methods. The authors examined the dist ribution 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 hy droxylase-with immunohistochemical studies and an in situ hybridizatio n method in three ganglioneuroblastomas with WDHA syndrome. All patien ts had an extremely elevated plasma level of VIP. Results. Peptides ex amined immunohistochemically in the tumor were all detectable in gangl ionic cells and some neurites. The coexistence of those peptides was o bserved 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. Conclusions. It was shown that m ultiple genes of peptides are expressed simultaneously and translated to proteins in those tumors.