Insulinoma and prolactinoma in a young female: a variant of multiple endocrine neoplasia 1 (Men-1).
- Author:
Marbert John T. CARDINO
;
Frances Lina LANTION-ANG
- Publication Type:Journal Article, Original
- Keywords:
Multiple Endocrine Neoplasia 1
- MeSH:
Insulinoma;
Prolactinoma
- From:
Philippine Journal of Internal Medicine
2009;47(3):137-142
- CountryPhilippines
- Language:English
-
Abstract:
Background: MEN-1, a rare disorder, consists of parathyroid adenoma, entero-pancreatic tumor andpituitary tumor. We report a 20-year old female with insulinoma and prolactinoma without parathyroidadenoma to complete the triad of MEN-1. A MEN-1 variant has been reported with high penetrance ofprolactinoma and another endocrine tumor, however, validity of this variant is not yet universally accepted.Clinical Presentation: A 20 year old female was referred for recurrent episodes of grandmal seizuresprecipitated by hypoglycemia for 5 years and a 2 x 1.8 cm pancreatic nodule on magnetic resonance imaging.Symptoms were relieved by eating. She gained 18 kg in 4 months prior to the referral. She was obese (BMI:32kg/m2) with amenorrhea for 1 year. She had no acne, galactorrhea, and hirsutism.Diagnostics: She was initially under a neurologist and preliminar y investigations revealed normal electroencephalogram and brain computed tomography scan. Fasting blood sugar was 43mg/dl after 12-hour fast thus definitive 72 hour fast test was done. Fasting blood sugar of 18mg/dl and aninappropriately elevated insulin (41uU/ml) and Cpeptide (25.6ng/ml) were noted after an 18-hour fast,thus insulinoma was considered. Baseline cortisol was normal (880nmol/L). Pre-operative localizationshowed a 2 x 1.8 cm pancreatic mass confirmed byan intra-operative ultrasonography. Subsequentlytumor enucleation was done. Intraoperatively, blood sugar was monitored to maintain euglycemia. To workupfor her oligomenorrhea, a normal LH (2.4pg/ml), FSH (5.2pg/ml), testosterone (0.99ng/ml), estradiol(27.9pg/ml), and 17-hydroxyprogesterone (0.416ng/ ml) were documented however prolactin (4423 U/ml)was elevated, thus MEN-1 was considered. Pituitary MRI showed a 1.2 x 1.3 adenoma with biochemicalhyperprolactinemia confirming prolactinoma. Normal ionized calcium (1.16mmol/L), parathyroid hormone(47.8pg/ml) ruled out hyperparathyroidism to complete the triad of MEN-1. Histopathology confirmed insulinomaafter staining with chromogranin and synatophysin.
