Intraductal tubulopapillary neoplasm of pancreas

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Synonyms: ITPN

A rare epithelial tumor of pancreas characterized by a solid nodular mass growing within dilated pancreatic ducts histologically composed of nodules of back-to-back tubular glands forming large cribriform structures with high-grade dysplasia and ductal differentiation. There is no overt production of mucin. About half of the tumors occur in the head of the pancreas one third involve the gland diffusely. Patients present with nonspecific symptoms including abdominal pain vomiting weight loss steatorrhea and diabetes mellitus while obstructive jaundice is uncommon. This tumor type accounts for less than 1% of exocrine neoplasms and 3% of intraductal neoplasms of the pancreas.

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 Data version June 2024

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