Acinar cell carcinoma of pancreas
Synonyms: Pancreatic acinar cell carcinoma
A very rare malignant epithelial tumor of the pancreas characterized macroscopically by a usually large well-circumscribed fully or partially encapsulated solid mass often with hemorrhage necrosis and cystic changes in any portion of the pancreas and histologically by neoplastic cells with variable degrees of differentiation and morphology ranging from acinar structures similar to normal pancreatic acini to large sheets of poorly differentiated neoplastic cells. Presenting symptoms are typically non-specific and include abdominal pain weight loss vomiting nausea and/or less commonly jaundice. Immunohistochemical evidence of acinar-specific products is observed. Association with Lynch syndrome familial adenomatous polyposis and pancreatic panniculitis has been reported.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.
Reference: Access aggregated data from Orphanet at Orphadata.
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version April 2024
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