Endocrine Abstracts

Introduction: Mucinous ovarian cystoadenoma with stromal luteinization and hilar cell hyperplasia is a rare cause of hirsutism and virilization and only few cases are described in literature. We present a rare case of mucinous cystadenoma with hypertestosteronemia.

A 38-years old woman came to our observation for severe hirsutism and oligomenorrhea. Hormonal work-up showed severe hypertestosteronemia (total testosterone: 4.39 ng/ml) with high levels of DHEA-S (3574 ng/ml) and 17 OH progesterone (3.6 ng/ml). 17 OH progesterone after ACTH test stimulation was 18 ng/ml leading to the diagnosis of late onset congenital adrenal hyperplasia. However testosterone levels were, in our opinion, too high to be related only to a 21-hydroxilase deficiency. In the suspicion of an androgen secreting tumor, abdominal US and CT scan were performed, showing a right ovarian cystic mass of about 10 cm. Then the patient underwent right ovariectomy. Histology confirmed a mucinous cystoadenoma with hilar cell hyperplasia and stromal luteinization. After surgery, testosterone levels suddenly decreased, confirming our suspicion of an ovarian secretion of testosterone.

Conclusion: Mucinous cystoadenoma represents 15–20% of ovarian tumors. They often become very large and can extend up into the abdomen, so that the typical clinical presentation is usually related to the abdominal mass. Hypertestosteronemia is a rare clinical finding when hyperplasia of stromal cells and Leydig cells occur.

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.