Spontaneous Ovarian Hyperstimulation Syndrome Caused by a Follicle-Stimulating Hormone-Secreting Pituitary Macroadenoma in an Early Pubertal Girl

Abstract

Gonadotroph adenomas are difficult to diagnose since they usually show as nonsecreting tumors or produce biologically inactive hormones with no clinical effects and classically grow silent until neurological symptoms appear. Presentation with bilateral ovarian masses and ovarian hyperstimulation has been described in fertile years. Gonadotroph adenomas are extremely infrequent in children. We report a 13-year-old postmenarcheal girl referred to our hospital with 6 months of amenorrhea, abdominal palpable mass presumptive of bilateral ovarian tumors. The patient had Tanner IV breast development and a large abdominal mass occupying the whole low hemiabdomen. Laboratory evaluation revealed high estradiol levels with suppressed luteinizing hormone and inappropriately high follicle-stimulating hormone (FSH) levels. Pelvic ultrasound showed enlarged ovaries containing multiple giant cysts. An MRI revealed a pituitary macroadenoma. Transsphenoidal resection of the adenoma was performed with an uneventful postoperative course. Immunohistologic examination only showed staining for FSH, thus confirming pituitary secreting FSH adenoma. Hormonal laboratory levels normalized and ovarian masses showed marked involution 1 month after surgery. Three months later the MRI showed tumor disappearance. Conclusion: The presence of bilateral ovarian tumors requires a careful endocrine and neurological evaluation to exclude the presence of an FSH-producing tumor in order to avoid unnecessary ovarian surgery.

Gonadotroph pituitary macroadenoma inducing ovarian hyperstimulation syndrome: successful response to octreotide therapy

“…3,4,6 Regression of the enlarged multicystic ovaries and restoration of the regular menstrual cycles can be achieved by excising the pituitary adenoma. 3 However, persistence or recurrence of these tumors is common with concomitant chronic exposure of the patient to extremely high estrogen levels.…”

“…1 In their vast majority, these tumors secrete biologically inactive gonadotropin monomer subunits instead of the intact bioactive heterodimers and thus they do not cause a recognizable clinical syndrome. 2 Exceptionally, functional gonadotrophin releasing pituitary adenomas have been described with clinical manifestations varying from precocious puberty to ovarian hyperstimulation syndrome (OHSS) in premenopausal women 3,4 and testicular enlargement in men. 5 Experience in medical management of functional gonadotrophin-secreting pituitary tumors is limited.…”

Ovarian Hyperstimulation Caused by Gonadotroph Pituitary Adenoma – Review

“…Thus, the clinical picture does not involve OHSS [21]. In patients with gonadotroph adenomas a paradoxical responses of LH, FSH or their subunits to thyrotropin releasing hormone (TRH) stimulation tests is found even if basal levels of these hormones are normal [8,9,29,30]. This test can be utilized in the diagnosis [31].…”

“…Dopamine agonists, such as bromocriptine and cabergoline, can effectively reduce prolactin levels [10,17,25,29,39]. Additionally, cabergoline therapy can decrease estradiol and FSH levels [29].…”

“…Additionally, cabergoline therapy can decrease estradiol and FSH levels [29]. Ovarian size reduction and suppression of estradiol levels were observed after the addition of cabergoline to medroxyprogesterone acetate treatment [39].…”