Pancreatic Pathology Online

Pathology of Somatostatinoma (Delta Cell Tumour)

Dr Sampurna Roy MD            July 2016

 

Somatostatinoma a rare endocrine tumour is worthy of mention because the clinical findings are so nonspecific that the diagnosis is almost always missed preoperatively.

Somatostatinoma of the periampullary region, including duodenum and pancreas are rare.

Somatostatinomas may be sporadic or associated with neurofibromatosis type 1, Multiple Endocrine Neoplasia type 1, and Von Hippel-Lindau syndromes.

The tumour is usually solitary and slow growing.

The microscopic appearance of somatostinoma is similar in both locations.

The lesion is characterised by glandular structures lined by uniform, eosinophilic granular cells.

Solid, nested foci may be seen in focal areas.

Psammomatous calcification, which is of diagnostic importance and is characteristic, is seen in periampullary tumours and is uncommon in pancreatic somatostinoma.

There may be occasional mitoses and mild cytological atypic is frequently present.

The tumour has a characteristic infiltrative growth pattern and can be seen extending between muscle bundles of the muscularis propria.

Immunostaining for somatostatin is often brilliantly positive.

 

The tumour is characterised by a glandular pattern. Individual tumour cells are relatively uniform. Mild nuclear hyperchromasia and pleomorphism is present.

The cells are immunoreactive for somatostatin

 

Functional somatostatinomas release excessive amounts of somatostatin suppressing gallbladder motility and inhibiting the secretory activity of various endocrine and exocrine cell types. 

The syndrome consists of mild diabetes mellitus, gallstones, steatorrhea, indigestion, and hypochlorhydria.

These conditions result from the inhibitory actions of somatostatin on other cells of the pancreatic islets and APUD cells of the gastrointestinal tract that secrete insulin, cholecystokinin, glucagons, and gastrin.

Thus the levels of insulin and glucagons in blood are decreased.

In addition, to producing somatostatin some delta cell tumours also secrete calcitonin or ACTH.

Non-functional somatostatinomas tend either to be asymptomatic or to present with obstructive symptoms. These tumors are often malignant. 

25% of cases metastasise to regional lymph nodes, specially those tumours more than 2 cm in diameter. Liver metastases are rare.

Because of a relatively large size at the time of presentation (average diameter of 5 cm) and common location within the head of the pancreas, a Whipple procedure (pancreaticoduodenectomy) is the most appropriate treatment

Note: Somatostatinomas of the periampullary region is more common, often asscociated with Type1 Neurofibromatosis (von Recklinghausen's disease) and the multiple endocrine neoplasia (MEN) syndromes.

Reporting of Pancreaticoduodenectomy (Whipple's operation) specimen

 

Further reading:

Somatostatinoma: clinico-pathological features of three cases and literature reviewed.

Somatostatinoma of the Vater's papilla in a patient with von Recklinghausen's disease].

A case of somatostatinoma of the pancreas.

Spectrum of malignant somatostatin-producing neuroendocrine tumors.

Malignant somatostatinoma (brief overview and a case review).

Somatostatin-producing endocrine tumour of the duodenum associated with type 1 neurofibromatosis.

Pancreatic somatostatinoma manifested as severe hypoglycemia.

Somatostatin-producing pancreatic endocrine carcinoma presented as relapsing cholangitis -- a case report.

Periampullary somatostatinoma and multiple gastrointestinal stromal tumors associated with von Recklinghausen's disease.

Pancreatic and peripancreatic somatostatinomas.

Periampullary pancreatic somatostatinoma.

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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