Pancreatic Pathology Online

Pathology of Glucagonomas (Alpha-Cell Tumours)

Dr Sampurna Roy MD                 July 2016

 

Glucagon-secreting tumours of the pancreatic islets (glucagonomas) are rare and have been encountered mainly in perimenopausal and postmenopausal women.

These tumours are associated with a syndrome consisting of mild diabetes, a necrotizing, migratory, erythematous rash of the lower body, and anemia.

Since mild diabetes, dermatitis, and anemia are not infrequent in older adults, the syndrome is often missed until its persistence and severity suggest that it may be associated with an unsuspected underlying disease.

Late diagnosis, together with a possibly increased propensity for malignant behavior of alpha cell tumours, may serve to explain why more than half of the more than 40 cases reported have been malignant and have metastasized to regional lymph nodes and liver.

The diagnosis is firmly established by the finding of elevated levels of glucagons in the serum and the localization of glucagons in the tumour.

However, not all cases of alpha cell tumour stain for glucagons.

Some are positive for glicentin peptides, and those that are not stained by antibodies to glucagons and the glicentins may contain abnormal molecular variants that do not possess the antigenic determinants recognized by antibodies to the normal molecules.

The secretory granules of alpha cell tumours sometimes differ ultrastructurally from their normal counterparts, but they are often indistinguishable from normal.

Recently, it has been reported that in addition to glucagons some alpha cell tumours contain other hormones, such as insulin, somatostatin, and pancreatic polypeptide.

In patients with alpha cell tumours, plasma glucagons levels are elevated, up to 30 times above normal.

In addition to the characteristic hyperglycemia, fasting plasma amino acid levels are decreased to levels as low as 20% of normal. 

Surgical removal of benign, functional alpha cell tumours is followed by a rapid and complete remission of clinical symptoms.

In patients with the malignant variant and metastases, surgical removal of the bulk of the tumour leads to a marked amelioration of symptoms, which return as the metastases grow to sufficient size to raise the serum level of glucagons.

Glucagonoma syndrome is a paraneoplastic phenomenon characterized by an islet alpha-cell pancreatic tumour, necrolytic migratory erythema, diabetes mellitus, weight loss, anemia, stomatitis, thromboembolism, and gastrointestinal and neuropsychiatric disturbances.

These clinical findings in association with hyperglucagonemia and demonstrable pancreatic tumour establish the diagnosis.

 

Neoplasms of the Endocrine Tumours ; Normal Islets of Langerhans ; The Apud Concept ; Islet Cell Tumours ;   Somatostatinoma  ; VIPomas ; Multiple Endocrine Neoplasia (MEN) Syndrome ; Pancreatic Polypeptide-Secreting Tumours ; Enterochromaffin Cell (Carcinoid) Tumours .

 

Further reading:

Plasma glucagon levels suppressed by a glucose load in a man with incidental pancreatic glucagonoma.

Diagnostic challenge of glucagonoma: case report and literature review.

Rare presentation of endocrine pancreatic tumor: a case of diffuse glucagonoma without metastasis and necrolytic migratory erythema.

Pancreatic glucagonoma metastasising to the right ovary five years after initial surgery: a case report.

Glucagonoma syndrome: case report and literature review.

Glucagonoma.

Immunologic characterization of plasma glucagon components in a patient with malignant glucagonoma.

[Glucagonoma. A tumor disease with multiple clinical manifestations].

Gastrointestinal endocrine tumours. Glucagonomas.

Malignant glucagonoma of the pancreas diagnoses through anemia and diabetes mellitus.

[Glucagon-secreting malignant neuroendocrine tumor of the pancreas].

Rare presentation of endocrine pancreatic tumor: a case of diffuse glucagonoma without metastasis and necrolytic migratory erythema.

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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