Insulinoma and Prolactinoma in A Young Female - Philippine ...

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140 Cardino MJ T and Lantion-Ang FLDuring the recuperation period, she had norecurrence of the hypoglycemic episodes, seizuresand other neuroglycopenic symptoms. She wasdischarged improved on the 5th post-operative day.Two weeks from discharge, patient had a repeatinsulin determination after a 12 hour fast and resultsshowed blood sugar of 100 mg/dl and suppressedserum insulin of 0.5 uU/ml.On the 2nd month of follow-up, patient had norecurrence of any seizure episodes. No hypoglycemicattacks were claimed. Patient had significantly lostweight with a BMI of 26 kg/m 2 from a previous BMIof 32kg/m 2 .(LH), follicule stimulating hormone (FSH), thyroidstimulating hormone (TSH), adenocorticotrophichormone (ACTH), estradiol, progesterone, growthhormone, insulin like growth factor-1 (IGF-1) whichwere all normal except for elevated serum prolactinat 4423 U/ml (refer to appendix 1 for the results andreference values). Patient had normal secondarysexual characteristics. She had well-developed breastand female genital tract (Tanner V). She had coarseaxillary and pubic hairs. She had no palpable neckmasses. She had a normal free thyroxine (FT4)and thyroid stimulating hormone (TSH). To assessthe parathyroid axis, serum calcium, inorganicphosphorus and parathyroid hormone were taken andwere all normal (refer to appendix 1 for the resultsand reference values).In the stepwise approach for the diagnosis ofhyperprolactinemia, physiologic and drug-inducedetiologies must be ruled out. Pregnancy test wasnegative and she had no previous intake of neurolepticsand dopamine blockers. She had no galactorrhea.She had amenorrhea of a year duration. Ultrasoundof the ovaries showed polycystic ovaries. Polycysticovary syndrome (PCOS) may also be associated withhyperprolactinemia and may present with menstrualirregularities causing the distinction of betweenthe 2 conditions difficult. She had no evidence ofhyperandrogenism and the LH and FSH were normalmaking the consideration of PCOS unlikely. A pituitaryMRI was done. It showed a macroadenoma measuring1.2 x 1.5 x 1.3 cm enhanced by contrast thusprolactinoma was considered. Hyperprolactinemiafrom a pituitary stalk compression was less likelybecause the prolactin level was very high (4423 U/ml). Medical therapy remains to be the cornerstoneof therapy for both the microprolactinoma andmacroprolactinoma. Surgical therapy is reserved fortumor growth despite therapy and drug resistance tomore than 2 dopamine agonist drugs. Bromocriptine2.5mg three times a day was started.Fig 4.Patient at 2 Months Post-Pancreatic Encucleation.Insulinoma is common in patients 30-60 yearsold. It is a tumor of the beta cell of the pancreaticislets. The incidence is very low at 4 cases per 1million-years. 5 Diagnosis of insulinoma requires thedocumentation of hypoglycemia associated withhypersecretion of insulin and presence of pancreaticnodules by imaging techniques. Cases involvingthe younger age group are associated with multipleendocrine disorders. Four percent of insulinoma mayhave multiple endocrine neopasia-1. 2 Generally,they occur in individuals younger than 20 years old.To assess for involvement of the pituitary axis thefollowing were taken: serum luteinizing hormoneOn the 2nd month of follow-up, she hadresumption of her regular menstruation. She hadno occurrence of blurring of vision. A repeat serumprolactin of 405 U/ml was documented 6 monthsafter. Because of the significant drop in the prolactinto physiologic level and the normalization of hermenstruation, bromocriptine was decreased to2.5 mg twice a day. Prolactinoma may be the firstmanifestation of MEN-1 in fewer than 10% of patientsand less than 3% of patients with anterior pituitarytumors will have MEN-1. 6A multiple endocrine neoplasia was consideredin this case because of the involvement of 2 or moreendocrine glands. Multiple endocrine neoplasia
Insulinoma and Prolactinoma in A Young Female 141(MEN) is characterized by occurrence of tumorsinvolving two or more endocrine organs within thesame patient. The combined appearance of thetumors of the parathyroid, pancreatic islets andanterior pituitary is characteristic of MEN-1 alsoknown as WermerÊs Syndrome. However, 94.5% ofMEN-1 presented with primary hyperparathyroidismwhich was absent in our case. A variant of MEN-1with high penetrance of prolactinoma plus anotherendocrine tumor has been described.Fig 5.Pituitary Microadenoma Measuring 1.2 x 1.5 x 1.3 cm, Enhanced on Contrast with Pituitary MRIDISCUSSIONIn a review of cases of MEN-1 at the US-NationalInstitute of Health, 94.5% of cases had primaryhyperparathyroidism as the initial presentation. 6 Thedistribution of cases is depicted below.second decade which is compatible with our casehowever it is also at this age bracket where we expectpeak incidence of primary hyperparathyroidism as acomponent of MEN-1 which was not evident in ourcase. Please refer to the figure below.Parathyroid tumorGastrinomaInsulinomaProlactinomaFig 6.Schemic Representation of 220 Patients Seen withMEN-1Fig 7.Adapted from Marx et al, Annals of InternalMedicine, 1998 6As we shall see from the representation above,94.5% of cases willl have primary hyperparathyroidism.Twelve percent have combined parathyroid,pancreatic and pituitary tumors. 2 A combination ofboth a pancreatic and pituitary tumor occurs in 0.5%only. 2The peak of onset of MEN-1 occurs during theThe gene for MEN-1 is localized in chromosome11q13 by mapping studies. Menin is nuclear proteinwhich is the by-product of the gene mutation involvedin transcription regulation, genome instability andcell division. The utility of MEN-1 mutation is forearly detection amongst immediate family membersand first degree relatives. However, our case was
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