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Insulinoma

Updated : September 3, 2022





Background

Insulinoma is a sort of functional neuroendocrine tumor characterized by hypoglycemia caused by excessive insulin production.

It typically manifests as a single benign tumor; however, it can occasionally be coupled with type 1 multiple endocrine neoplasia (MEN1 syndrome).

Patients with insulinoma experience hypoglycemic episodes, most commonly fasting hypoglycemia. Insulinoma is typically diagnosed through biochemical testing when clinical suspicion is high.

Surgical removal is the preferred method of treatment. Prior to surgical resection, it is essential to properly describe the tumor using imaging localization. Additional treatment options may be considered after evaluating the stage and severity of the tumor.

Epidemiology

Insulinoma is a very rare malignancy, which annually affects only 1-4 individuals per million. Although they are generally benign, 5.8% of insulinomas are cancerous.

6%-7.6% of insulinomas are related with MEN1 syndrome, and in 7% of cases, it coexists with other benign tumors. On average, surgical diagnosis of this condition occurs between the ages of 47 and 50.

Anatomy

Pathophysiology

Etiology

A definite understanding of insulinoma etiology is absent. Normally, a high plasma glucose content would stimulate the beta cells of the pancreas to secrete insulin, and vice versa.

In individuals with insulinoma, insulin secretion occurs even at low concentrations of plasma glucose. The hyperinsulinemia could be caused by an insulin splice variation that upregulates the translation process.

Once the intracellular insulin reservoir is full, insulin is secreted into the bloodstream. Hypoglycemia is caused by hyperinsulinemia’s enhanced glycogen production, decreased gluconeogenesis, and glycogenolysis.

MEN1 is a condition marked by a susceptibility to a few malignancies, such as enteropancreatic, pituitary, and parathyroid tumors.

In the majority of cases where insulinomas are related with MEN1, it is associated with a mutation of the MEN1 gene on chromosome 11q13.

Genetics

Prognostic Factors

Following successful surgical removal, the 10-year survival rate for insulinoma patients is a solid 88%, and 87.5% of patients are cured after surgery, although there is a chance of remission.

After successful surgical resection, the 10-year survival rate for malignant insulinomas was much lower at 29% in one investigation, and 24 percent in another.

In cases where the insulinoma is associated with MEN1 syndrome, there is a greater risk of initial surgery failure and recurrence of illness.

Malignant insulinomas also present similar risks. Patients with MEN1 have a recurrence rate of around 21% after 10-20 years, while those without MEN1 have a 5% recurrence rate after 10 years, and around 7% after 20 years.

Clinical History

Physical Examination

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

Media Gallary

References

https://www.ncbi.nlm.nih.gov/books/NBK544299/

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Insulinoma

Updated : September 3, 2022




Insulinoma is a sort of functional neuroendocrine tumor characterized by hypoglycemia caused by excessive insulin production.

It typically manifests as a single benign tumor; however, it can occasionally be coupled with type 1 multiple endocrine neoplasia (MEN1 syndrome).

Patients with insulinoma experience hypoglycemic episodes, most commonly fasting hypoglycemia. Insulinoma is typically diagnosed through biochemical testing when clinical suspicion is high.

Surgical removal is the preferred method of treatment. Prior to surgical resection, it is essential to properly describe the tumor using imaging localization. Additional treatment options may be considered after evaluating the stage and severity of the tumor.

Insulinoma is a very rare malignancy, which annually affects only 1-4 individuals per million. Although they are generally benign, 5.8% of insulinomas are cancerous.

6%-7.6% of insulinomas are related with MEN1 syndrome, and in 7% of cases, it coexists with other benign tumors. On average, surgical diagnosis of this condition occurs between the ages of 47 and 50.

A definite understanding of insulinoma etiology is absent. Normally, a high plasma glucose content would stimulate the beta cells of the pancreas to secrete insulin, and vice versa.

In individuals with insulinoma, insulin secretion occurs even at low concentrations of plasma glucose. The hyperinsulinemia could be caused by an insulin splice variation that upregulates the translation process.

Once the intracellular insulin reservoir is full, insulin is secreted into the bloodstream. Hypoglycemia is caused by hyperinsulinemia’s enhanced glycogen production, decreased gluconeogenesis, and glycogenolysis.

MEN1 is a condition marked by a susceptibility to a few malignancies, such as enteropancreatic, pituitary, and parathyroid tumors.

In the majority of cases where insulinomas are related with MEN1, it is associated with a mutation of the MEN1 gene on chromosome 11q13.

Following successful surgical removal, the 10-year survival rate for insulinoma patients is a solid 88%, and 87.5% of patients are cured after surgery, although there is a chance of remission.

After successful surgical resection, the 10-year survival rate for malignant insulinomas was much lower at 29% in one investigation, and 24 percent in another.

In cases where the insulinoma is associated with MEN1 syndrome, there is a greater risk of initial surgery failure and recurrence of illness.

Malignant insulinomas also present similar risks. Patients with MEN1 have a recurrence rate of around 21% after 10-20 years, while those without MEN1 have a 5% recurrence rate after 10 years, and around 7% after 20 years.

https://www.ncbi.nlm.nih.gov/books/NBK544299/

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