Page 65 - Read Online

Topic: Neuroendocrine Tumors

Pancreatic neuroendocrine tumor with hypoglycemia and elevated
insulin-like growth factor II: a case report

Roberta Modica , Antonella Di Sarno , Annamaria Colao , Antongiulio Faggiano 3
2
1
1
1 Department of Clinical Medicine and Surgery, Federico II University, 80131 Naples, Italy.
2 UOC of Oncology, A.O. Dei Colli, Monaldi Unit, 80131 Naples, Italy.
3 Thyroid and Parathyroid Surgery Unit, Istituto Nazionale per lo studio e la cura dei tumori “Fondazione G. Pascale” - IRCCS, 80131 Naples, Italy.
Corresponding Author: Dr. Antongiulio Faggiano, Thyroid and Parathyroid Surgery Unit, Istituto Nazionale per lo studio e la cura dei tumori
“Fondazione G. Pascale” - IRCCS, 80131 Naples, Italy. E-mail: afaggian@unina.it
A B S T R AC T
Pancreatic neuroendocrine tumors (pNETs) can be associated with different clinical syndromes. Insulinoma is the most common
functioning pNET characterized by hypoglycemia and hyperinsulinemia. The authors report a case of a man presenting with
hypoglycemia and biochemical features of insulinoma. A pancreatic lesion was found and growth hormone (GH) deficiency
was also diagnosed associated with an empty sella present on the pituitary magnetic resonance imaging. The disappearance of
hypoglycemia and normalization of GH secretion after surgical resection of the pancreatic lesion, revealed a rare pNET secreting
insulin-like growth factor II.

Key words: Pancreatic neuroendocrine tumor; insulinoma; hypoglycemia; insulin-like growth factor II

INTRODUCTION and the hormone hypersecreted: insulinoma, gastrinoma
(Zollinger-Ellison syndrome), glucagonoma, VIPoma,
Pancreatic neuroendocrine tumors (pNETs) represent GHRFoma (growth hormone releasing factor secreting),
1-2% of all pancreatic tumors and 7% of NETs in general, ACTHoma, and somatostatinoma. Among F-pNETs,
with an incidence of 0.43 per 100,000. Epidemiological insulinoma is the most common with an estimated annual
data show that pNET incidence is increasing, perhaps due incidence of 1-4 per million patients, representing 35-
to more widespread use of diagnostic imaging techniques, 40% of all F-pNETs. [1-3] Although rare, insulinoma
especially computed tomography (CT) scans, and increased represents the most common cause of hypoglycemia
physician awareness of this tumor type. Moreover, a high related to endogenous hyperinsulinemia, characterized by
prevalence of pNETs is reported in autopsy studies (from inappropriately high insulin and/or proinsulin and high
0.8% to 10%), thus suggesting that they are frequently C-peptide concentrations. The presence of hypoglycemia
clinically silent. A slight male predominance (55% together with a pancreatic lesion is usually the clinical
male vs. 45% female) is reported and the median age at picture of insulinoma. Nevertheless hypoglycemia
presentation is around 50 years. [1] represents a relatively common biochemical finding,
which may be due to many causes, thus a careful clinical
pNETs may be sporadic or part of a genetic syndrome, most history, together with biochemical and radiological tests, is
commonly multiple endocrine neoplasia type 1 (MEN1), essential to identify the underlying cause. Other subtypes
von Hippel-Lindau disease (VHL), neurofibromatosis type I of F-pNETs have been reported, although rarely. Diagnosis
(NF), or tuberous sclerosis complex (TSC). Clinically pNETs of F-pNET can be challenging, as clinical presentation
can be distinguished into two groups: functional (F-pNET) may simulate other more common diseases, thus causing
and nonfunctional (NF-pNET). The majority of pNETs are delay in diagnosis. Of note, the hormone-excess state in
non-functional (90%) and present with symptoms due to F-pNET requires both acute and long term control, since it
mass effect or as incidental findings, whereas F-pNETs represents a potential life threatening condition along with
(10%) are characterized by hormone hypersecretion
with different clinical signs and symptoms. F-pNET are This is an open access article distributed under the terms of the Creative
distinguished according to the clinical hormonal syndrome Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows
others to remix, tweak, and build upon the work non-commercially, as long as
the author is credited and the new creations are licensed under the identical
Access this article online terms.
Quick Response Code: For reprints contact: service@oaepublish.com
Website:
www.jcmtjournal.com
How to cite this article: Modica R, Di Sarno A, Colao A, Faggiano
A. Pancreatic neuroendocrine tumor with hypoglycemia and elevated
insulin-like growth factor II: a case report. J Cancer Metasta Treat
DOI: 2016;2:345-7.
10.20517/2394-4722.2016.44
Received: 11-07-2016; Accepted: 10-08-2016

©2016 Journal of Cancer Metastasis and Treatment ¦ Published by OAE Publishing Inc. 345