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Pancreatic polypeptide-related tumors.Peptides.
2002 Feb;23(2):339-48.
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.
Pancreatic polypeptide hyperplasia causing watery diarrhea syndrome: a
case report.Can
J Surg. 1999 Feb;42(1):55-8.
Neuroendocrine
tumours of the pancreas can secrete numerous peptides, leading to
various recognizable clinical syndromes. The secretion of pancreatic
polypeptide has been used as a marker for neuroendocrine tumours but
is considered to be a biologically inert peptide. A 37-year-old woman
had watery diarrhea syndrome from pancreatic polypeptide hyperplasia.
Only 2 other reported cases in the literature have described
pancreatic polypeptide hyperplasia; however, this is the first
reported case in which the patient was successfully treated by
surgical resection, with a 2-year follow-up. This report and review of
the literature illustrate that pancreatic polypeptide hypersecretion
may present as a clinical endocrinopathy.
Pancreatic
polypeptide-producing tumors. Silent lesions of the pancreas? Arch of
Surg. Vol. 119 No. 5, May 1984.
Eight patients
with pancreatic polypeptide (PP)-producing islet cell tumors
and one patient with pseudo-PP-producing tumors were examined.
Their age range was 20 to 74 years. Clinical features
included abdominal pain in four patients, weight loss in
four patients, diarrhea in two patients, gastrointestinal
bleeding in two patients, and jaundice in one patient. The
range of the basal serum level of PP was 394 to 35,100 pg/mL. In two
patients the PP-producing tumors were associated with multiple
endocrine neoplasia. Two patients had diffuse hepatic
metastases at the time of diagnosis and four patients had
disease limited to the pancreas. Pancreaticoduodenectomy
and 80% pancreatectomy were performed in four and two
patients, respectively. Immunohistochemical staining was positive for
PP and neuron-specific enolase in all cases and was negative for
other peptides except in one specimen with
microadenomatosis. Patients who underwent curative
resection are asymptomatic with normal serum levels of PP.
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