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Nesidioblastosis coexisting with non-functioning islet cell tumour in an adult
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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)
Keywords
diazoxide; hypoglycaemia; nesidioblastosis; neuroendocrine tumour; pancreas; pancreatectomy
About this articleTitle
Nesidioblastosis coexisting with non-functioning islet cell tumour in an adult
Journal
Issue
Article type
Case report
Pages
356-360
Published online
2015-09-01
Page views
1790
Article views/downloads
2614
DOI
10.5603/EP.2015.0045
Pubmed
Bibliographic record
Endokrynol Pol 2015;66(4):356-360.
Keywords
diazoxide
hypoglycaemia
nesidioblastosis
neuroendocrine tumour
pancreas
pancreatectomy
Authors
Valeria Valli
Stella Blandamura
Davide Pastorelli
Stefano Merigliano
Cosimo Sperti
