Comparison Between Sporadic and Multiple Endocrine Neoplasia Type 1-Associated Insulinoma

Area of Science:

• Endocrinology
• Oncology
• Genetics

Background:

• The clinical and demographic differences between sporadic and Multiple Endocrine Neoplasia type 1 (MEN-1)-associated insulinomas are not well-defined.
• Insulinomas, a type of pancreatic neuroendocrine tumor, can occur sporadically or as part of MEN-1 syndrome.

Purpose of the Study:

• To compare the demographics, neoplasm characteristics, clinical presentation, and survival outcomes of patients with sporadic versus MEN-1-associated insulinomas.
• To elucidate the distinct features of benign and malignant insulinomas in the context of MEN-1 syndrome.

Main Methods:

• A retrospective study was conducted to identify and analyze insulinoma patients.
• MEN-1 diagnosis was confirmed through genetic testing or clinical criteria (presence of ≥2 primary MEN-1 tumor types).

Main Results:

• The incidence of malignant insulinoma was similar between MEN-1 (18%) and sporadic (16%) groups.
• MEN-1 patients with malignant insulinoma were significantly younger (median age 33 vs 54 years) than sporadic patients.
• MEN-1 patients with benign insulinoma presented at a younger age (median 38 vs 52 years) and had larger resected tumors (2.0 vs 1.5 cm) compared to sporadic patients. Concurrent insulinomas were more frequent in MEN-1 patients (17% vs 2%).

Conclusions:

• Patients with MEN-1-associated insulinomas tend to present at a younger age and have larger benign pancreatic lesions upon resection compared to sporadic cases.
• Younger individuals, especially those with multifocal pancreatic neuroendocrine tumors and endogenous hyperinsulinism, warrant evaluation for MEN-1 syndrome.