Endocrine Abstracts

Introduction: Rathke cleft cysts (RCC) are benign cystic lesions of the sellar and suprasellar region, which is believed to arise from the remnants of the Rathke pouch. Symptomatic RCC are rare. Endocrine symptoms are usually caused by compression of the surrounding pituitarygland. It can therefore cause hypopituitarism, but it is rarely associated with primary endocrine dysfunctions. In this case, we report a co-occurrence of premature ovarian insufficiency and RCC.

Case summary: We report the case of a 29-year-old female patient, who first presented with headaches, irregular menses and galactorrhea. Initial investigation showed a hyperprolactinemia with a level of 154 ng/mL without other hormonal abnormalities (TSH:1,27 µUI/ml, fT4: 16,7 pmol/l, cortisol at 428 nmol/l and 603 nmol/l after ACTH stimulation test). Initial hormonal investigation for FSH and LH were at normal levels. Cerebral MRI showed intrasellar cystic low density lesion of 7mm with ring enhancement which evoked a Rathke cleft cyst. She was treated initially with bromocriptine with a well evolution. Interestingly, despite normalization of prolactine levels after two years and disappearance of the galactorrhea, our patient began to have secondary amenorrhea with hot flashes. Hormonal investigation showed hypergonadotropic hypogonadism with high levels of FSH (79,61 mUI/mL) and LH (37,21 mUI/mL). Pelvic ultrasound revealed decrease in both ovaries volume but did not show any other abnormalitites. Genetic testing showed a normal karyotype. The diagnosis of primary ovarian insufficiency was held. The patient received hormone replacement therapy and reported better quality of life.

Conclusion: Rathke cleft cyst occurs mostly in females and may result in a variety of symptoms. Hyperprolactinaemia is the commonest associated endocrine dysfunction, followed by hypopituitarism. This hyperprolactinaemia due to RCC may predispose to autoimmune thyroiditis; however, the association between RCC and primary thyroid disease remains to be clarified.