Pancreas
Congenital anomalies
Nesidioblastosis
Author: Deepali Jain, M.D.
Last author update: 1 August 2012
Last staff update: 22 October 2020
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: Nesidioblastosis[TI] pancreas[TIAB]
Table of Contents
Definition / general | Clinical features | Case reports | Treatment | Microscopic (histologic) description | Microscopic (histologic) imagesCite this page: Jain D. Nesidioblastosis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/pancreasnesidio.html. Accessed April 18th, 2024.
Definition / general
- Islets in intimate association with ducts, with formation of ductuloinsular complexes
- Also called congenital islet hyperplasia
- Indicates active formation of endocrine cells by multipotential cells in basal layer of ducts
Clinical features
- Normal in infants, exaggerated in neonatal hyperglycemia (infants of diabetic mothers)
- In adults, is rare cause of persistent hyperinsulinemic hypoglycemia
- Associated with Beckwith-Weidemann syndrome, chronic pancreatitis, cystic fibrosis, endocrine neoplasms, gastric bypass patients (Mod Pathol 2009;22:239), Zollinger-Ellison syndrome
- May be due to increase in expression of growth factors IGF2, IGF1Ra and TGFBR3 in islets (Mod Pathol 2009;22:239)
- Must rule out mutation in HADH gene (J Clin Endocrinol Metab 2011;96:E498)
- Either focal or diffuse (Am J Surg Pathol 1989;13:766):
- Focal: nodular hyperplasia of islet-like cell clusters, including ductuloinsular complexes and hypertrophied insulin cells with giant nuclei
- Diffuse: involves entire pancreas; irregularly sized islets and ductuloinsular complexes present, both contain hypertrophied insulin cells, peliosis type vascular ectasia
Case reports
- 44 year old man with symptomatic hypoglycemia and localized nesidioblastosis (Hum Pathol 2010;41:447)
Treatment
- Focal nesidioblastosis: partial pancreatectomy with excision of diseased areas; diffuse: near total pancreatectomy
Microscopic (histologic) description
- Islets in intimate association with ducts, with formation of ductuloinsular complexes
- Beta cell hypertrophy (Am J Surg Pathol 2005;29:524)
Microscopic (histologic) images
Images hosted on other servers:
20 day old girl with persistent hypoglycemia and seizures
23 year old man with hypoglycemia mimicking an insulinoma
