Abstract Introduction Most gonadotropinomas are nonfunctional macroadenomas, usually diagnosed by immunohistochemical staining. Here, a rare case of functional gonadotropinoma will be presented. Clinical Case A 37-year-old female patient applied to the neurology clinic with a severe headache, and a macroadenoma was detected on cranial magnetic resonance imaging(MRI). She was referred to our endocrinology clinic. Her menstrual cycle was irregular, and she had frequent intermenstrual bleeding for about a year. She has never had galactorrhea, and there were no features of acromegaly or Cushing’s disease on physical examination that would suggest a functional adenoma. The biochemical evaluation showed an elevated estradiol (E2) level of 2900 ng/L (normal 12,5 –166 ng/L) and an elevated prolactin(PRL) level of 203 µg/L (normal 2,8-29,2 µg/L) with normal follicular-stimulating hormone (FSH), luteinizing hormone (LH) and beta-human chorionic gonadotropin (b-hCG) levels of 2,8 IU/L, 6,6 IU/L and 0.1 mIU/mL respectively. PRL level was 196 µg/L after dilution. The low-dose dexamethasone suppression test was 2.1 µg/dL.The patient’s laboratory results are given in Table 1. Pituitary MRI revealed a 31*20*28 mm lesion invading both cavernous sinuses and compressing the optic nerve on the left side(Figure 1A). Visual field testing showed no visual field defects. The patient was consulted with the gynecology and obstetrics clinic due to high estradiol levels. Pelvic MRI revealed 5-6 cystic lesions, the largest of which was 45*30 mm in the right ovary and 41*37 mm in the left ovary, which did not enhance contrast and did not contain solid components (Figure 1B). The patient, who was considered for surgery due to suspicion of malignancy, was planned to be closely followed up by gynecology because tumor markers were negative and the lesions did not enhance contrast. While the patient’s gynecological examinations were continuing, the patient was started on cabergoline one tablet/week. After the gynecology department decided to follow up on the lesions, the patient underwent TSS for macroadenoma. Immunohistochemical staining was positive for FSH and LH, negative for PRL and adrenocorticotropic hormone (ACTH), and the ki-67 index was %2. The patient’s E2, cortisol, and PRL levels became in the normal range after the operation, and her hormone levels are given in Table 1. A pelvic ultrasound performed six months after the operation revealed that the previously detected lesions in the ovaries had disappeared. At this stage, the patient was considered to have functional gonadotropinoma and cortisol elevation has been linked to the effect of estradiol. Conclusion In patients with pituitary macroadenoma, it would be beneficial to check pituitary-gonadal axis hormones even if they are not in the follicular phase to avoid missing functional gonadotropinomas and prevent unnecessary surgeries for ovarian pathologies that may be observed in patients with functional gonadotropinomas.Figure 1:Pituitary and pelvic MRI images of the patientA: Pituitary MRI revealed a 31*20*28 mm lesion invading both cavernous sinuses and compressing the optic nerve on the left side. B: Pelvic MRI revealed 5-6 cystic lesions, the largest of which was 45*30 mm in the right ovary and 41*37 mm in the left ovary, which did not enhance contrast and did not contain solid components. Table 1:Laboratory results of the patientTSS: transsphenoidal surgery, ACTH: Adrenocorticotropic hormone, FSH: follicle stimulating hormone, LH: luteinizing hormone, IGF-1: Insülin-like growth factor, TSH: Thyroid stimulating hormone, fT4: free thyroxie fT3: free triiodothyronine, beta HCG: beta-human chorionic gonadotropin
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