Pancreatic Pathology Online

Pathology of Pancreatic Polypeptide-Secreting Tumours  

Dr Sampurna Roy MD                    July 2016


Pancreatic polypeptide-producing tumours are rare and are not associated with a clinical syndrome, despite the fact that they are functional and secrete high levels of pancreatic polypeptide in the blood.

The tumours are usually single and benign, although a few have metastasized to the liver.

Pancreatic polypeptide is also secreted by other islet cell tumours in addition to their specific hormones, especially after stimulation by a meal, and it has been suggested that it may be a useful adjunct marker to detect islet cell tumours.


Pancreatic polypeptide-producing tumors are mostly located in the pancreas and may present as three pathologic lesions:

- pure Pancreatic polypeptide-omas,

- mixed tumors with minor Pancreatic polypeptide cell population, and

- Pancreatic polypeptide-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 Pancreatic polypeptide 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 Pancreatic polypeptide.

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 Pancreatic polypeptide-producing tumors.


Neoplasms of the Endocrine Tumours Normal Islets of Langerhans The Apud Concept Islet Cell Tumours Alpha Cell Tumours ;  Beta Cell Tumours (Insulinomas) ; Somatostatinoma VIPomasEnterochromaffin Cell (Carcinoid) Tumours Multiple Endocrine Neoplasia (MEN) Syndrome.


Further reading

Pancreatic polypeptide update: its roles in detection of the trait for multiple endocrine adenopathy syndrome, type I and pancreatic polypeptide-secreting tumors.

Pancreatic polypeptide-secreting islet cell tumor. A follow-up report.

Pancreatic vasoactive intestinal polypeptide-secreting tumor: report of a case

Islet amyloid polypeptide-producing pancreatic islet cell tumor. A clinical and biochemical characterization.

Malignant pancreatic polypeptide secreting tumour of islet cells: a case for aggressive surgical palliation.

Pancreatic polypeptide islet cell tumor: case report and review of the literature

Vasoactive intestinal polypeptide-secreting tumor (VIPoma) with liver metastases: dramatic and durable symptomatic benefit from hepatic artery embolization, a case report.

Symptomatic pancreatic polypeptide-secreting tumor of the distal pancreas (PPoma).

A case of dopamine agonists inhibiting pancreatic polypeptide secretion from an islet cell tumor.

Indium-111 pentetreotide uptake by pancreatic polypeptide cell hyperplasia: potential pitfall in somatostatin receptor scintigraphy.

A Pancreatic Polypeptide-Producing Pancreatic Tumor Causing WDHA Syndrome.

Sporadic pancreatic polypeptide secreting tumors (PPomas) of the pancreas.

Vasoactive intestinal polypeptide secreting pancreatic tumour with hepatic metastases: long term survival after orthotopic liver transplantation.

Pancreatic polypeptide-producing tumors. Silent lesions of the pancreas?

Pancreatic polypeptide-related tumors.

Pancreatic polypeptide hyperplasia causing watery diarrhea syndrome: a case report.

Occurrence of human pancreatic polypeptide in pancreatic endocrine tumors. Possible implication in the watery diarrhea syndrome.



Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)






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