Pancreatic Pathology Online

Pathology of Pancreatic Gastrinoma (Ulcerogenic Islet Cell Tumour)

Dr Sampurna Roy MD          July 2016

 

The 1955 landmark publication by Zollinger and Ellison characterized the gastrinoma syndrome and presented a hypothesis for the pancreatic endocrine origin of the gastric hypersecretion and dramatic ulcer diathesis.

Pancreatic gastrinoma is an islet cell tumor consisting of so-called G-cells that synthesize and secrete gastrin, a potent hormonal stimulus for the secretion of gastric acid.

Histopathology image of Gastrinoma

The location of this tumor in the pancreas is curious, because gastrin-containing cells have not been demonstrated in normal islets.

The tumour is thought to arise from multipotent endocrine stem cells that have undergone inappropriate differentiation to form gastrin-secreting islet cells (G-cells) in the endocrine pancreas.

By electron microscopy G-cells bear a strong resemblance to the gastrin-secreting cells that normally reside in the duodenal mucosa.

This tumour was incriminated some 30 years ago as the cause of intractable gastric hypersecretion and severe peptic ulceration of the duodenum and jejunum, the so-called Zollinger-Ellison syndrome, named after the two surgeons who first described it.

The reason for the "ulcerogenic potential" of this peculiar islet cell tumour was eventually established when the development of highly specific antisera to gastrin made possible the demonstration of high levels of gastrin in the blood and in the tumour.

Among islet cell tumors, pancreatic gastrinomas are second in frequency only to insulinomas.

Fifteen percent of cases of Zollinger-Ellison syndrome are due to gastrinomas outside the pancreas, particularly in the duodenum.

Malignant gastrinomas are more common than the benign variant, and multiple adenomas are more frequent than single ones.

Metastases to the regional lymph nodes and liver are often functional.

In cases of this syndrome due to multiple functional adenomas of G-cells, the symptoms are disturbances of physiology that are so chronic and debilitating, with such a poor quality of life, that the term benign is a relative one.

Visit: Neoplasms of the Endocrine Tumours ; Normal Islets of Langerhans ; The Apud Concept ; Islet Cell Tumours Alpha Cell Tumours ; Beta Cell Tumours (Insulinomas) ; Somatostatinoma ; VIPomasCorticotropinoma ;Parathyrinoma; Multiple Endocrine Neoplasia (MEN) Syndrome ; Pancreatic Polypeptide-Secreting Tumours

 

Further reading:

Sporadic versus hereditary gastrinomas of the duodenum and pancreas: distinct clinico-pathological and epidemiological features.

Increased islet beta cell replication adjacent to intrapancreatic gastrinomas in humans.

Insulinoma and gastrinoma syndromes from a single intrapancreatic neuroendocrine tumor.

Gastrinoma before Zollinger and Ellison.

Natural history of gastrinoma: lessons from the past.

Primary hepatic gastrinoma as an unusual manifestation of zollinger-ellison syndrome.

Gastrinomas: a historical perspective.

Gastrinomas: advances in diagnosis and management.

Expression of the calcium-sensing receptor in gastrinomas

Pathologic aspects of gastrinomas in patients with Zollinger-Ellison syndrome with and without multiple endocrine neoplasia type I.

Surgical pathology of gastrinoma. Site, size, multicentricity, association with multiple endocrine neoplasia type 1, and malignancy.

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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