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Multiple Insulinoma of Pancreas in MEN 1 Syndrome - A Rare Case

  • Writer: ivyhpbgigensurg
    ivyhpbgigensurg
  • Jan 15
  • 4 min read

Updated: Feb 5

A young lady presented with increased appetite, weight gain and episodes of hypoglycemia, i.e. ghabrahat, sweating, etc. Multiple consults from various doctors, Cect and MRI diagnosed a very rare disease for which the patient visited us. After a thorough evaluation of disease localisation, the patient was planned for surgery.

The patient underwent a successful surgery with enucleation of multiple pancreatic insulinomas with intraoperative ultrasonography, intraoperative frozen section and pathological examination to confirm cure during surgery.

The patient was cured of the disease and is on follow-up.


MEN 1 Syndrome and Insulinoma

What is MEN 1 Syndrome?

MEN 1 syndrome (Multiple Endocrine Neoplasia type 1) is a rare inherited condition in which a person develops tumors or overgrowth in certain hormone-producing glands of the body.

These tumors are usually not cancer, but they can cause problems because they make too many hormones.

Which parts of the body are affected?

MEN 1 mainly affects three glands (doctors often remember it as the “3 P’s”):

1. Parathyroid glands (neck)

  • These glands control calcium levels in the blood

  • In MEN 1, they become overactive

  • This causes high calcium levels

Common symptoms:

  • Kidney stones

  • Bone pain or fractures

  • Tiredness

  • Abdominal pain

  • Depression or mood changes


2. Pancreas (digestive hormone tumors)

  • Small tumors can form in the pancreas

  • They may produce excess hormones like insulin or gastrin

Problems that can happen:

  • Low blood sugar → sweating, shakiness, fainting

  • Excess stomach acid → ulcers, stomach pain, vomiting

3. Pituitary gland (brain)

  • The pituitary controls many body hormones

  • Tumors here may increase hormone production

Symptoms may include:

  • Headache

  • Vision problems

  • Milk discharge from breast (even in men)

  • Irregular periods in women

  • Sexual problems or infertility

Why does MEN 1 happen?

  • MEN 1 is caused by a gene defect

  • It is passed from parents to children

  • If one parent has MEN 1, there is a 50% chance the child may have it

How is MEN 1 diagnosed?

Doctors may suspect MEN 1 when:

  • A person has tumors in more than one endocrine gland

  • There is a family history

Tests include:

  • Blood tests (hormone levels, calcium)

  • Imaging scans (CT, MRI)

  • Genetic testing

How is MEN 1 treated?

There is no permanent cure, but the condition can be well controlled.

Treatment includes:

  • Surgery to remove problematic glands or tumors

  • Medicines to control hormone levels

  • Regular follow-up and screening for life

Important points to remember

  • MEN 1 is long-term, but manageable

  • Early diagnosis prevents serious complications

  • Family members may also need testing

  • Regular medical check-ups are very important



What is an insulinoma?

An insulinoma is a small tumor of the pancreas that makes too much insulin.

Insulin is the hormone that lowers blood sugar.When too much insulin is produced, the blood sugar falls dangerously low.

How is insulinoma related to MEN 1?

  • MEN 1 syndrome causes multiple hormone-producing tumors

  • In MEN 1, insulinomas:

    • Occur at a younger age

    • Are often multiple (more than one tumor)

    • May come back even after treatment

What problems does insulinoma cause?

Because insulin is high, blood sugar becomes low (hypoglycemia).

Common symptoms include:

  • Sweating

  • Shakiness or tremors

  • Hunger

  • Palpitations

  • Headache

  • Confusion or strange behavior

  • Blurred vision

  • Fainting or seizures (in severe cases)

Symptoms often:

  • Occur during fasting

  • Improve after eating sugar or food

Typical warning sign (important clue)

Doctors use something called Whipple’s triad:

  1. Symptoms of low blood sugar

  2. Low blood sugar measured in blood test

  3. Relief of symptoms after eating or giving glucose

How is insulinoma diagnosed?

Tests may include:

  • Blood tests showing:

    • Low blood sugar

    • High insulin levels at the same time

  • Fasting test done under medical supervision

  • Scans like CT, MRI, or special scans to locate the tumor

How is insulinoma treated?

1. Surgery (main treatment)

  • Removal of the tumor(s) from the pancreas

  • In MEN 1, multiple tumors may need careful surgical planning

2. Medicines (if surgery is delayed or not possible)

  • Drugs to prevent low blood sugar

  • Medications that reduce insulin release

Why is insulinoma in MEN 1 important?

  • Low blood sugar can be life-threatening

  • Early diagnosis prevents brain damage and seizures

  • Lifelong follow-up is needed because tumors can recur

Key points in simple words

  • Insulinoma is a pancreatic tumor that causes low blood sugar

  • In MEN 1, insulinomas are often multiple and early in onset

  • Main symptoms are related to hypoglycemia

  • Surgery is the best treatment

  • Regular monitoring is essential

Intraoperative photograph of of C- loop of duodenum showing an encapsulated tumor in Head of Pancreas.

WHY SURGERY FOR MEN 1 SYNDROME AND INSULINOMA

Surgery in MEN 1 Syndrome

1. Parathyroid Surgery

(Most common and usually first surgery)

  • Almost all MEN 1 patients develop overactive parathyroid glands

  • Surgery involves removing most or all parathyroid glands

Types of surgery:

  • Removal of 3½ glands (leaving a small part)

  • Or removal of all glands with re-implantation of tissue elsewhere

Outcome:

  • Calcium levels usually return to normal

  • Bone pain and kidney stones improve

2. Pancreatic Surgery (for Insulinoma)

What is done?

  • The surgeon removes the tumor(s) from the pancreas

  • In MEN 1, insulinomas are often multiple, so surgery may include:

    • Removal of part of the pancreas

    • Removal of visible tumors

Goal of surgery:

  • Stop excess insulin production

  • Prevent low blood sugar attacks

Outcomes after Insulinoma Surgery

Short-term results:

  • Blood sugar levels usually return to normal

  • Symptoms like sweating, shaking, and fainting disappear

  • Quality of life improves significantly

Long-term results:

  • Most patients do very well

  • In MEN 1, insulinomas may come back, so repeat treatment may be needed

Possible Risks of Surgery (Simple Explanation)

  • Bleeding or infection

  • Leakage of pancreatic fluid

  • Temporary or permanent diabetes (rare but possible)

  • Low calcium after parathyroid surgery

Doctors take steps to minimize these risks.

Follow-Up After Surgery (Very Important)

MEN 1 is a lifelong condition, so regular follow-up is essential, even after successful surgery.

Follow-up includes:

  • Regular blood tests:

    • Calcium levels

    • Blood sugar

    • Hormone levels

  • Periodic scans to detect new tumors

  • Monitoring symptoms like:

    • Weakness

    • Low sugar episodes

    • Bone pain

Family screening:

  • Close family members may need testing

  • Genetic counseling is recommended

Long-Term Outlook

  • Surgery controls symptoms but does not cure MEN 1

  • With regular monitoring, most patients live a normal and active life

  • Early detection of new tumors leads to better outcomes

Key Points in Simple Words

  • Surgery removes problem-causing tumors and glands

  • Insulinoma surgery prevents dangerous low blood sugar

  • Symptoms usually improve after surgery

  • Tumors can return in MEN 1

  • Lifelong follow-up is essential.


 
 
 

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