Pancreatic polypeptide-related tumors.
PP-producing tumors are mostly located in the pancreas and may present as three pathologic lesions: pure PP-omas, mixed tumors with minor PP cell population, and PP-cell hyperplasia. These tumors are among the most common multiple adenomas frequently found in patients with multiple endocrine neoplasia type 1. Hypersecretion and high circulating levels of PP are frequently found. They are symptomless but may be useful for the identification of the pancreatic tumors. Numerous types of extrapancreatic endocrine tumors are able to synthesize and secrete PP. They occur mostly but not exclusively in the gastrointestinal tract, particularly in the rectum. The inactivation of the MEN 1 gene at 11q13 appears to be involved in the development of pancreatic but not of rectal PP-producing tumors.
Bordi
Cesare
C
From the Department of Pathology and Laboratory Medicine, Section of Anatomic Pathology, University of Parma, Parma, Italy. cesare.bordi@unipr.it
Azzoni
Cinzia
C
D'Adda
Tiziana
T
Pizzi
Silvia
S
D002880:Chromosomes, Human, Pair 11 / Q000235:genetics; D006801:Humans; D006965:Hyperplasia / Q000378:metabolism; D018761:Multiple Endocrine Neoplasia Type 1 / Q000235:genetics / Q000378:metabolism; D010190:Pancreatic Neoplasms / Q000235:genetics / Q000378:metabolism*; D010191:Pancreatic Polypeptide / Q000235:genetics / Q000378:metabolism*; D012004:Rectal Neoplasms / Q000235:genetics / Q000378:metabolism
D010191:Pancreatic Polypeptide