open access

Vol 66, No 4 (2015)
Case report
Published online: 2015-09-01
Submitted: 2014-08-01
Accepted: 2014-12-01
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Nesidioblastosis coexisting with non-functioning islet cell tumour in an adult

Valeria Valli, Stella Blandamura, Davide Pastorelli, Stefano Merigliano, Cosimo Sperti
DOI: 10.5603/EP.2015.0045
·
Endokrynologia Polska 2015;66(4):356-360.

open access

Vol 66, No 4 (2015)
Case report
Published online: 2015-09-01
Submitted: 2014-08-01
Accepted: 2014-12-01

Abstract

The most common cause of hyperinsulinaemic hypoglycaemia in adult is insulinoma. Although nesidioblastosis is a rare but well-recognised disorder of persistent hypoglycaemia in infants, it is extremely rare in adults.

We present a case of a 59-year-old woman with small neuroendocrine tumour of the tail of the pancreas, diagnosed by CT scans and MRI, and hypoglycaemic syndrome. Laparoscopic distal pancreatectomy was performed, and pathologic examination showed a well-differentiated, non-functioning endocrine tumour of the pancreas and diffuse nesidioblastosis in the remnant gland. In the early postoperative period, recurrent hypoglycaemia occurred in spite of oral diazoxide therapy. Plasma proinsulin levels were extremely high. 18F-DOPA positron emission tomography showed a pathologic uptake of tracer in the head and the uncinate process of the pancreas. Subtotal pancreatectomy was suggested but the patient refused operation: she is taking diazoxide 100 mg three times daily.

Coexistence of nesidioblastosis with a neuroendocrine tumour makes preoperative diagnosis and management of severe hypoglycaemia more difficult. Nesidioblastosis should be considered in differential diagnosis of hypoglycaemic syndrome, but histological examination is necessary for a definitive tissue diagnosis. (Endokrynol Pol 2015; 66 (4): 356–360)

Abstract

The most common cause of hyperinsulinaemic hypoglycaemia in adult is insulinoma. Although nesidioblastosis is a rare but well-recognised disorder of persistent hypoglycaemia in infants, it is extremely rare in adults.

We present a case of a 59-year-old woman with small neuroendocrine tumour of the tail of the pancreas, diagnosed by CT scans and MRI, and hypoglycaemic syndrome. Laparoscopic distal pancreatectomy was performed, and pathologic examination showed a well-differentiated, non-functioning endocrine tumour of the pancreas and diffuse nesidioblastosis in the remnant gland. In the early postoperative period, recurrent hypoglycaemia occurred in spite of oral diazoxide therapy. Plasma proinsulin levels were extremely high. 18F-DOPA positron emission tomography showed a pathologic uptake of tracer in the head and the uncinate process of the pancreas. Subtotal pancreatectomy was suggested but the patient refused operation: she is taking diazoxide 100 mg three times daily.

Coexistence of nesidioblastosis with a neuroendocrine tumour makes preoperative diagnosis and management of severe hypoglycaemia more difficult. Nesidioblastosis should be considered in differential diagnosis of hypoglycaemic syndrome, but histological examination is necessary for a definitive tissue diagnosis. (Endokrynol Pol 2015; 66 (4): 356–360)

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Keywords

diazoxide; hypoglycaemia; nesidioblastosis; neuroendocrine tumour; pancreas; pancreatectomy

About this article
Title

Nesidioblastosis coexisting with non-functioning islet cell tumour in an adult

Journal

Endokrynologia Polska

Issue

Vol 66, No 4 (2015)

Pages

356-360

Published online

2015-09-01

DOI

10.5603/EP.2015.0045

Bibliographic record

Endokrynologia Polska 2015;66(4):356-360.

Keywords

diazoxide
hypoglycaemia
nesidioblastosis
neuroendocrine tumour
pancreas
pancreatectomy

Authors

Valeria Valli
Stella Blandamura
Davide Pastorelli
Stefano Merigliano
Cosimo Sperti

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