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Last updated Aug. 9, 2018

Approved by: Maulik P. Purohit MD, MPH

Nesidioblastosis is a pancreatic condition caused by increased levels of insulin-like growth factor II (a vital growth protein), due to abnormal function of the pancreas. This increased level is caused by hyperplasia and dysplasia of beta cells of pancreas.

What are the other Names for this Condition? (Also known as/Synonyms)

  • Congenital Hyperinsulinism due to Nesidioblastosis
  • Congenital Islet Hyperplasia of Pancreas
  • Noninsulinoma Pancreatogenous Hypoglycemia Syndrome (NIPHS)

What is Nesidioblastosis? (Definition/Background Information)

  • Nesidioblastosis is a pancreatic condition caused by increased levels of insulin-like growth factor II (a vital growth protein), due to abnormal function of the pancreas. This increased level is caused by hyperplasia and dysplasia of beta cells of pancreas
  • The condition causes increased amounts of insulin in blood that result in decreased blood glucose (which is termed as hyperinsulinemic hypoglycemia). Nesidioblastosis is typically seen in children, though it may affect adults too
  • The risk factors for the condition include chronic pancreatitis, multiple endocrine neoplasia types 1 and 2, and individuals who have undergone gastric bypass surgery
  • Due to Nesidioblastosis, the individual may have signs and symptoms that include dizziness, blurred vision, abdominal pain, increased urination frequency, and even unconsciousness. It can also cause metabolic dysfunction and brain damage (if severe)
  • The treatment of Nesidioblastosis may involve medications and surgery (either a partial or total pancreatectomy). However, the benefits of a surgery are limited
  • The prognosis depends upon many factors, most importantly on the severity of the condition and the patient’s response to treatment. Although, in majority of the cases, the prognosis of Nesidioblastosis is good

The pancreas is an important organ of the digestive system.

  • Based on function of the pancreas, it can be functionally divided into 2 parts, namely:
    • Exocrine pancreas, which produces digestive enzymes, and
    • Endocrine pancreas that produces hormones such as insulin, glucagon, and somatostatin. Insulin and glucagon helps with controlling sugar levels in blood
  • 95% of the pancreas is exocrine portion and 5% is endocrine portion. Pancreatic tumors (both benign and malignant) can arise from both the exocrine and the endocrine part
  • Based upon anatomy of the pancreas, it can be divided into 3 main parts, namely the:
    • Head,
    • Body, and
    • Tail
  • Pancreatic tumors can affect the head, body, and tail region of the pancreas. Some tumors can affect one area of the pancreas more than the other areas.

Hence, localizing the tumor site can guide the healthcare provider to arrive at a probable diagnosis.

Who gets Nesidioblastosis? (Age and Sex Distribution)

  • Nesidioblastosis is a congenital condition that affects newborns and young children. In adults, it is an acquired condition and is very infrequent
  • It can affect both males and females
  • The condition can occur worldwide; individuals of all racial and ethnic background may be affected

What are the Risk Factors for Nesidioblastosis? (Predisposing Factors)

The risk factors for Nesidioblastosis include the following:

  • Children born to diabetic mothers
  • Chronic pancreatitis
  • Cystic fibrosis
  • Multiple endocrine neoplasia types 1 and 2 (MEN 1 and MEN 2)
  • Zollinger-Ellison syndrome
  • Beckwith-Wiedemann syndrome
  • Gastric bypass surgery

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Nesidioblastosis? (Etiology)

  • The exact cause of Nesidioblastosis development is unknown. It can be congenital or acquired
  • The condition occurs due to increased levels in blood of insulin-like growth factor II (IGF-II), which is a very important protein for growth and development. An IGF-II increase occurs due to hyperplasia and dysplasia of the pancreatic beta cells
  • Since it may be associated with various genetic syndromes, research scientists believe that the cause of the condition is mostly due to genetic mutations

What are the Signs and Symptoms of Nesidioblastosis?

The signs and symptoms of Nesidioblastosis may include the following:

  • Symptoms of low glucose level in blood, such as sweating, dizziness/confusion, blurring of vision, and even loss of consciousness
  • Nausea and vomiting
  • Abdominal pain, back pain
  • Loss of appetite, weight loss
  • Fatigue (easily getting tired)
  • Increased hunger and thirst
  • Increased urination
  • Headaches
  • Unexplainable weight loss
  • Pain or numbing of the feet and hands

How is Nesidioblastosis Diagnosed?

The following are the diagnostic methods that may be used to detect Nesidioblastosis:

  • A thorough physical examination and a complete medical history is very important
  • Blood test to check for insulin and glucose levels
  • Blood test to check for serum amylase levels
  • Complete blood count with differential
  • Blood tests that may involve tumors markers, such as:
    • Carcinoembryonic antigen (CEA)
    • CA 19.9
    • CA 15.3
    • Alpha fetoprotein
  • IGF-II level testing using selective transhepatic portal venous sampling. In this test, the insulin-like growth factor II levels from various parts of the portal system is measured to localize the tumor
  • Radiological studies that may include:
    • Abdominal ultrasound: A procedure where high-frequency sound waves are used to produce real-time images
    • Endoscopic ultrasound: It is a minimally-invasive procedure that uses high-frequency sound waves to obtain detailed images of the pancreas
    • CT scan of abdomen: It may be helpful in detecting recurrences, or if metastasis to other organs has occurred
    • MRI scan of abdomen: It helps produce high-quality pictures of certain body parts including the tissues, muscles, nerves, and bones
  • Additionally, the following magnetic resonance imaging procedures may be used particularly in individuals suspected of having pancreatic tumor/cancer:
    • MR cholangio-pancreatography (MRCP)
    • MR angiography (MRA)
  • Positron emission tomography (PET) scan: It is particularly helpful in visualizing the spread of the cancer to other body parts (metastasis) and/or recurrence
  • Percutaneous transhepatic cholangiography (PTC): A contrast material is injected into the bile ducts to visualize the structure
  • Endoscopic retrograde cholangio-pancreatography (ERCP): This technique is used when symptoms of pancreatic cancer are present in the individual. A special equipment and dye injections are used to obtain a series of images. If required, a biopsy sample may also be collected during the procedure
  • Invasive diagnostic procedures such as:
    • Laparoscopy: A special device is inserted through a small hole into the abdomen, to visually examine it. If necessary, a tissue sample is obtained for further analysis. Exploration of the abdomen using a laparoscope is called ‘exploratory laparoscopy’
    • Laparotomy: The abdomen is opened through an incision for examination, and if required, a biopsy sample obtained. Exploration of the abdomen using laparotomy procedure is called ‘exploratory laparotomy’
  • Tissue biopsy: A tissue biopsy is performed and sent to a laboratory for a pathological examination to the pathologist, who examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

The tissue for diagnosis can be procured in multiple different ways, and they include:

  • Fine needle aspiration (FNA) biopsy of the pancreatic tumor - however, because the areas of hyperplasia are small, it is very hard to perform a FNA
  • Core biopsy of the pancreatic tumor
  • Open biopsy of the pancreatic tumor

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Nesidioblastosis?

The complications due to Nesidioblastosis may include the following:

  • It can cause severe metabolic dysfunction
  • Severe hypoglycemia can result in brain damage

How is Nesidioblastosis Treated?

Nesidioblastosis is considered to be normal in infants. In case treatment is required, then the following measures may be undertaken:

  • Administration of medications
  • The hyperplasia can be focal or diffused
    • In focal cases, partial pancreas is removed
    • In diffused cases, total pancreas removal is attempted
  • However, surgical treatment methods are generally found to bring only limited benefits to many patients
  • Follow-up care with regular screening and checkups are important

How can Nesidioblastosis be Prevented?

Currently, there are no specific methods or guidelines to prevent Nesidioblastosis. However, the condition may be avoided through the following measures:

  • Genetic testing in individuals with a family history of pancreatic tumors or other underlying genetic conditions associated with pancreatic tumors
  • Regular medical screening at periodic intervals with blood tests, scans, and physical examinations for those who have already endured the tumor

What is the Prognosis of Nesidioblastosis? (Outcomes/Resolutions)

  • The prognosis of Nesidioblastosis depends upon the severity of the signs and symptoms. It is excellent, if the individuals respond to appropriate treatment
  • However, regular follow up visits with the healthcare provider are important

Additional and Relevant Useful Information for Nesidioblastosis:

It is estimated that the average lifetime risk of developing pancreatic cancer is about 1.5%.

What are some Useful Resources for Additional Information?

References and Information Sources used for the Article:

Helpful Peer-Reviewed Medical Articles:

Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: Nov. 27, 2015
Last updated: Aug. 9, 2018