Insulinomas in MEN-I patients: early detection and treatment of insulinomas in patients with the multiple endocrine neoplasia syndrome type-I.
In the multiple endocrine neoplasia syndrome type I (MEN-I syndrome), periodic screening of patients and their close relatives may improve prognosis and life expectancy. Although there is diffuse involvement of the pancreas with microadenomatosis, insulinomas in the MEN-I syndrome usually occur as single tumours. This is illustrated here by two patients with insulinomas and the MEN-I syndrome. Preoperative localization of the tumours was achieved accurately by digital subtraction angiography combined with dynamic computerized tomography after a bolus injection of contrast medium. At present, two and three years after elective surgery both patients are asymptomatic. The early detection and treatment of insulinomas is extremely important because of the high risk of cerebral damage associated with late diagnosis. Periodic investigation of MEN-I family members can promote the early diagnosis and treatment of insulinomas, especially in young patients, whose life expectancy and quality of life may be improved.
Jadoul
M
M
Department of Internal Medicine, University Hospital, Utrecht.
Koppeschaar
H P
HP
Bax
M A
MA
Mali
W P
WP
Wit
J M
JM
Huber
J
J
Vasen
H F
HF
Van der Sluys Veer
J
J
Struyvenberg
A
A
Lips
C J
CJ
D000293:Adolescent; D006801:Humans; D007340:Insulinoma / Q000175:diagnosis* / Q000235:genetics; D008297:Male; D009377:Multiple Endocrine Neoplasia / Q000175:diagnosis* / Q000235:genetics; D010190:Pancreatic Neoplasms / Q000175:diagnosis* / Q000235:genetics; D010375:Pedigree