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

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. Copyright (C) 2010 S. Karger AG, Basel