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Pancreatic endocrine tumours associated with WDHA syndrome

An Immunohistochemical and electron microscopic study


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.

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Author information

Correspondence to Toru Kameya.

Additional information

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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Ooi, A., Kameya, T., Tsumuraya, M. et al. Pancreatic endocrine tumours associated with WDHA syndrome. Vichows Archiv A Pathol Anat 405, 311–323 (1985). https://doi.org/10.1007/BF00710067

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Key words

  • Endocrine pancreatic tumours
  • WDHA syndrome
  • Peptide hormones
  • Immunostaining