Insulinoma and Prolactinoma in a Young Female: A Variant of Multiple Endocrine Neoplasia 1 (MEN-1)
Philippine Journals Online
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| Title |
Insulinoma and Prolactinoma in a Young Female: A Variant of Multiple Endocrine Neoplasia 1 (MEN-1)
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| Creator |
Marbert John T Cardino; Fellow-in-training, Section of Endocrinology, Diabetes and Metabolism
Frances Lina Lantion-Ang; Consultant, Section of Endocrinology, Diabetes and Metabolism |
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| Subject |
Endocrinology
Multiple Endocrine Neoplasia 1; Insulinoma; prolactinoma |
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| Description |
Background: MEN-1, a rare disorder, consists of parathyroid adenoma, entero-pancreatic tumor and pituitary tumor. We report a 20-year old female with insulinoma and prolactinoma without parathyroid adenoma to complete the triad of MEN-1. A MEN-1 variant has been reported with high penetrance of prolactinoma 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 seizures precipitated 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 preliminary 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 an inappropriately 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 localization showed a 2 x 1.8 cm pancreatic mass confirmed by an intra-operative ultrasonography. Subsequently tumor enucleation was done. Intraoperatively, blood sugar was monitored to maintain euglycemia. To workup for 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 biochemical hyperprolactinemia 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 insulinoma after staining with chromogranin and synatophysin. Treatment and Outcome: Surgical removal of the insulinoma was uneventful and post-operatively the blood sugar remained normal even without glucosecontaing fluids. Bromocriptine 5mg/day was given for the prolactinoma. Two months post-operatively she had normal menstruation, lost weight (body mass index of 26kg/m2 from 32kg/m2), and had non-recurrence of hypoglycemic seizures. Conclusion: We report a female with a variant type of MEN-1 composed of insulinoma corrected surgically and prolactinoma treated medically with favorable outcome. Keywords: Multiple Endocrine Neoplasia 1; Insulinoma; prolactinoma DOI: 10.3860/pjim.v47i3.1656 Phil. J. Internal Medicine, 47: 137-142, May-June, 2009
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| Publisher |
The Philippine College of Physicians
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| Contributor |
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| Date |
2010-07-23
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| Type |
Peer-Reviewed Item
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| Format |
application/pdf
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| Identifier |
http://www.philjol.info/index.php/PJIM/article/view/1656
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| Source |
Philippine Journal of Internal Medicine; Vol 47, No 3 (2009); 137-142
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| Language |
en
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| Coverage |
Philippines
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