Pancreatic endocrine tumours associated with WDHA syndrome |
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| Authors: | Akishi Ooi Toru Kameya Masaru Tsumuraya Ken Yamaguchi Kaoru Abe Yukio Shimosato Noboru Yanaihara |
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| Affiliation: | (1) Pathology Division, National Cancer Center Research Institute, Tokyo;(2) Department of Pathology, Kitasato University, School of Medicine, Kanagawa;(3) Endocrinology Division, National Cancer Center Research Institute, Tokyo;(4) Laboratory of Bioorganic Chemistry, Shizuoka College of Pharmacy, Shizuoka;(5) Department of Pathology, School of Medicine, Kanazawa University, Kanazawa |
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| Abstract: | Summary Nine pancreatic endocrine tumours of patients with watery diarrhoea hypokalaemia achlorhydria (WDHA) syndrome were examined by immunohistochemistry and electron microscopy. All cases revealed neoplastic proliferation of VIP (vasoactive intestinal peptide)-immunoreactive (IR) cells. Immunoreactivity to a novel peptide hormone PHM-27, which is processed from a common big precursor peptide of VIP (prepro VIP/PHM-27), was identified in VIP-IR cells of 8 tumours. VIP-PHM-IR cells had secretory granules measuring about 130 to 220 nm in diameter. Radioimmunoassay of tumour tissue extracts showed high VIP and PHM contents in proportional amounts in most cases. According to the results of immunostaining, the 8 tumours fell into two large groups; 5 with PP (pancreatic polypeptide)-IR cells and 3 with CT (calcitonin)-IR cells. The former group demonstrated VIP cells and PP cells intermingled in various proportions, including one tumour in which coexistence of PP-IR and VIP-IR in the same cells was demonstrated. Cell heterogeneity of the tumours and possible relationships of VIP, PP and CT cells were discussed.This work was supported in part by Grants-in-Aid for Cancer Research from the Ministry of Health and Welfare and from the Ministry of Education, Science and Culture, Japan |
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| Keywords: | Endocrine pancreatic tumours WDHA syndrome Peptide hormones Immunostaining |
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