Multiple-hormone gene expression in ganglioneuroblastoma with watery diarrhea, hypokalemia, and achlorhydria syndrome.
BACKGROUND
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.


METHODS
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.


RESULTS
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.


CONCLUSIONS
It was shown that multiple genes of peptides are expressed simultaneously and translated to proteins in those tumors.
Kimura
N
N
Department of Pathology, Tohoku University, School of Medicine, Sendai, Japan.
Yamamoto
H
H
Okamoto
H
H
Wakasa
H
H
Nagura
H
H
D002675:Child, Preschool; D003346:Corticotropin-Releasing Hormone / Q000032:analysis; D004744:Enkephalin, Methionine / Q000032:analysis; D005260:Female; D005729:Ganglioneuroma / Q000235:genetics*; D015972:Gene Expression Regulation, Neoplastic / Q000235:genetics; D006801:Humans; D017403:In Situ Hybridization; D007223:Infant; D008297:Male; D009478:Neuropeptide Y / Q000032:analysis; D010451:Peptide PHI / Q000032:analysis; D012186:Retroperitoneal Neoplasms / Q000235:genetics*; D013004:Somatostatin / Q000032:analysis; D013373:Substance P / Q000032:analysis; D014446:Tyrosine 3-Monooxygenase / Q000032:analysis; D014660:Vasoactive Intestinal Peptide / Q000032:analysis; D003969:Vipoma / Q000235:genetics*
D009478:Neuropeptide Y; D010451:Peptide PHI; D013373:Substance P; D014660:Vasoactive Intestinal Peptide; D013004:Somatostatin; D004744:Enkephalin, Methionine; D003346:Corticotropin-Releasing Hormone; D014446:Tyrosine 3-Monooxygenase