Insulinoma and extreme obesity in a patient with MEN1 syndrome

We report a patient aged 16 experiencing headache, galactorrhea and ammenorrhea. Laboratory examina- tion showed an increased prolactin (PRL) level (> 400 ng/mL ; normal range 5-30 ng/mL ) and computed tomography (CT) of pituitary gland revealed a tumor mass, 1.5 ?? 0.9 cm in size. Transnasal selective partial adenomectomy was performed, and postoperative exci- sional biopsy showed a prolactinoma. Postoperatively, she developed hypopituitarism, for which levothyroxin (100 µg/day) and hydrocortison (30 mg/day) replacement therapy were prescribed. Further laboratory tests showed decreased glucose levels (3.0 mmol/L ; normal range 3.5- 6.5 mmol/L), never completely explained, because the patient left the hospital on her demand. She returned to our department at the age of 21, with long-standing symptoms of weakness, sweating, tremor and mental confusion. She was 150 cm high and had 158 kg (90 kg more than at previous hospitalization), BMI 70.2 kg/m2. Laboratory examination revealed persistent hypoglyce- mia (2.5-3.1mmol/L), hyperinsulinemia (105pmol/L; normal range < 5 pmol/L) and increased C-peptide level (95 µg/L ; normal range 1.1-5.0 µg/L). The serum levels of IGF-1, cortisol, vanillylmandelic acid, metanephrines, parathormone, PRL, thyroid stimulating hormone, growth hormone, follicle-stimulating hormone and luteinizing hormone were within normal limits. Abdominal ultra- sonography revealed a tumor mass in the distal part of the pancreatic tail, 2.6 cm in size (Fig. 1).