Microcystic pancreatic adenoma is an extremely rare tumor, arising in exocrine pancreatic tissue, which may lead to severe abdominal pain. To date malignant transformation has not been reported. There is a conspicuous, statistically significant, association of microcystic pancreatic adenoma with various other diseases. This report describes a 57 year-old woman with associated clinical signs of scleroderma. Therapeutic modalities are primarily aimed at complete removal of the tumor, which may--depending on the localisation--require major pancreatic resections.

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