Pancreatic Pathology Online

Pathology of Nesidioblastosis

 Dr Sampurna Roy MD                July 2016

 

Laidlaw coined the term Nesidioblastosis in 1938 to characterize the neodifferentiation of the islet cells of Langerhans from pancreatic duct epithelium.

Nesidioblastosis is a term that describes multifocal hyperplasia of all pancreatic cell components and is characterized primarily by their disorganization and proliferation throughout the entire pancreas.

The islets are present in intimate association with ducts, with formation of so-called ductulo-insular complexes.

Focal and diffuse forms of nesidioblastosis have been reported.

Focal lesions may be small (2 - 4 mm) and can easily be overlooked.

Nesidioblastosis is commoner in the body and tail of the pancreas.

It is advocated that the specimen is cut as thinly as possible, and then serial step-wise sections examined microscopically.

Nesidioblastosis showing clusters of endocrine and acinar cell delineated by delicate fibrous band.

Chromogranin A highlights the endocrine cell clusters in nesidioblastosis.

Microscopy shows endocrine cell clusters with peripheral rims of acinar cells.

A thin band of fibrous tissue separates each cluster.

Duct formation can sometimes be seen within the endocrine cell clusters.

Endocrine cells may also line these ducts.

The association of endocrine cells clusters with ductular structures is called a ductulo-insular (endocrine) complex.

The endocrine cells have a typical appearance with with finely granular, eosinophilic cytoplasm.

The resident endocrine cells within the islets display mild nuclear pleomorphism, with occasional cells having enlarged slightly bizarre nuclei.

This corresponds to islet cell hypertrophy.

Immunohistochemistry shows a normal spatial arrangement of the four endocrine cells that normally reside in the islets: glucagon, somatostatin, insulin and pancreatic polypeptide in both focal and diffuse types.

However, there is an increase in the number and size of the beta-cells resulting in a so-called 'maldistribution'.

No corresponding reduction in the other three cell types is apparent.

The endocrine cells within the clusters and ducts are highlighted by immunohistochemistry for chromogranin.

 

Nesidioblastosis is well recognized in the pediatric population as a  frequent cause of persistent neonatal hypoglycemia.

Some cases have been reported in adults with persistent hyperinsulinemic hypoglycemia.

 

Further reading:

A case of alpha-cell nesidioblastosis and hyperplasia with multiple glucagon-producing endocrine cell tumor of the pancreas.

Nesidioblastosis in adults.

Nesidioblastosis and Pancreatic Non-functioning Islet Cell Tumor in an Adult with Type 2 Diabetes Mellitus.

Nesidioblastosis. A case of hyperplasia of the islets of Langerhans in the adult.

Case report: focal nesidioblastosis ("nesidioblastoma") in an adult.

A 20-year-old man with recurrent abdominal pain and vomiting since the age of 5 years. Familial chronic pancreatitis with nesidioblastosis and hyperinsulinaemic hypoglycaemia.

Nesidioblastosis as a cause of focal pancreatic 111In-pentetreotide uptake in a patient with putative VIPoma: another differential diagnosis.

Treatment of hyperinsulinemic hypoglycemia due to diffuse nesidioblastosis in adults: a case report.

A case of nesidioblastosis causing hypoglycaemia after delivery.

Hyperinsulinemic hypoglycemia with nesidioblastosis: histologic features and growth factor expression.

An unusual case of concurrent insulinoma and nesidioblastosis.

Adult nesidioblastosis. Clinicopathologic correlation between pre-operative selective arterial calcium stimulation studies and post-operative pathologic findings

Diffuse nesidioblastosis causing hyperinsulinemic hypoglycemia: the importance of pancreatic sampling on EUS.

Nesidioblastosis and hyperplasia of alpha cells, microglucagonoma, and nonfunctioning islet cell tumor of the pancreas: review of the literature.

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

Histopathology-India.net

 

Pathopedia-India.com

 

Surgical Pathology.com

 

Pathology-India.com

 

Dermpath-India

 

Infectious Disease Online

 

Pathology Quiz Online 

 

Paediatric Pathology Online

 

Pancreatic Pathology Online

 

Paraganglioma-Online

 

Endocrine Pathology Online

 

Eye Pathology Online

 

Ear Pathology Online

 

Cardiac Path Online

 

Pulmonary Pathology Online

 

Lung Tumour Online

 

Mesothelioma-Online

 

Nutritional Pathology Online

 

Environmental Pathology Online

 

Soft Tissue Tumour Online

 

GI Path Online-India

 

Gallbladder Pathology Online

 

E-book - History of Medicine  

 

Microscope - Seeing the Unseen

 

 

 

Disclaimer

Privacy Policy  

Advertising Policy

Copyright 2016  surgical-pathology.com