Unusual presentation of juvenile granulosa cell tumor of the ovary

We present a case report of juvenile granulosa cell tumor of the ovary (JGCT) with an unusual clinical presentation and hormonal secretion.

A 16-yr-old girl had developed spontaneous menarche at the age of 12 yr, but after this initial menstrual bleeding she had no further periods for 4 yr. She had no clinical signs of virilization. Endocrinological studies detected high levels of DHEA, 17 hydroxyprogesterone (17OH-P), insulin and PRL, an exaggerated DHEA response after ACTH stimulation, and low FSH and high LH values after GnRH. An ultrasound examination revealed an irregular structure and increased diameters of her right ovary, due to the presence of a cyst. Because exploratory laparoscopy revealed the presence of a right ovarian mass, her right ovary was removed. JGCT was diagnosed. Ten days after surgery, menstrual bleeding initiated. Endocrinological evaluation after the operation showed that 17OH-P, insulin and basal FSH and LH serum values had returned to normal, while DHEA levels had decreased to within the upper limit of the normal range. Only PRL levels remained unchanged.

Our patient presented some unusual characteristics. She did not have precocious puberty, but secondary amenorrhea. Hormonal secretion consisted mainly of androgens, even though clinical signs of virilization were not present.