Endocrine Abstracts

Background: Gonadotroph adenomas usually present as non-functioning pituitary adenomas.We describe a patient with a functioning gonadotroph adenoma presenting with ovarian hyperstimulation syndrome: an exceptionally rare presentation of pituitary disease.

Case history: A 26-year-old female presented with a 10-year history of worsening lower abdominal pain, bloating, dysmenorrhea and irregular periods in 2006. She was found by ultrasound to have multiple ovarian cysts that were surgically drained. Her symptoms recurred with re-growth of 19 ovarian cysts that prompted removal in March 2008. Abdominal pain further recurred in June 2008 and the repeat USS showed enlarged 11 ovarian cysts. As part of evaluation for subfertility an elevated prolactin was identified (PRL: 1338 mU/l n<530) leading to a pituitary MRI that revealed a right-sided (11×8×7 mm) pituitary adenoma.

Investigations: After endocrine evaluation, baseline hormone profile revealed extremely high oestradiol levels (20 000 pmol/l). Clinical and biochemical features were considered consistent with ovarian hyperstimilation syndrome (OHSS) in the absence of exogenous gonadotrophins and therefore presumed secondary to a gonadotroph secreting adenoma.The patient underwent endoscopic transphenoidal surgery in December 2008 with subsequent intact anterior pituitary endocrine axes, although requiring DDAVP. Oestradiol fell rapidly from 20 000 pmol/l down to a normal level, her menstrual cycle returned and was followed by two successful pregnancies. The pelvic cysts resolved and pelvic anatomy returned to normal on ultrasound. Serial MRI Pituitary scans have shown no evidence of residual tumour.

Conclusion: Gonadotrophin-secreting pituitary tumours should be considered in patients with multiple recurrent ovarian cysts and extreme elevations of Oestradiol. Although positive immunohistochemistry for gonadotrophins is common amongst pituitary tumours, functioning gonadotrophinomas are very unusual with only a handful reported in the literature and with variable outcomes. In our patient, the recognition occurred after propitious diagnosis by MRI.

Declaration of interest: The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research project.

Funding: This research did not receive any specific grant from any funding agency in the public, commercial or not-for-profit sector.