LBMON232 A Hairy Situation: Ovarian Sex Cord Stromal Tumor As A Rare Cause Of Hyperandrogenism
Authors: Syed Hasan , Krusniak Lara , Brietzke Stephen , Au Pak-Ho , Akofu Anota 
Published: November 2022

Background

There are many types of ovarian tumors. Of the many types, ovarian steroid cell tumors, not otherwise specified (NOS), are among the most rare, and are known to have malignant potential.

Clinical Case

A 36 year old female presented to clinic with a primary complaint of hirsutism. The patient reported needing to shave her chin every 2 days, as well as hair growth on her chest, abdomen, and back, and thinning of the hair on her scalp. The patient underwent menarche at 18 years of age. She was later diagnosed with PCOS. She was started on Spironolactone for hirsutism. Several years prior to presentation, she had undergone fertility treatment without a resulting pregnancy. Due to heavy menses, the patient ultimately chose to undergo a hysterectomy without oophorectomy. Lab work revealed elevated free and total testosterone levels of 58.1 ng/dL (0. 06-1) and 1530 ng/dL (8-60), respectively. Blood levels of 17-OH progesterone and ACTH were obtained to evaluate for possible non-classical (late onset) congenital adrenal hyperplasia (CAH). 17-OH progesterone was found to be elevated at 9173 ng/dL (35-290) with ACTH of 12 pg/mL (7.2-63). Dehydroepiandrosterone sulfate (DHEAS) was 137 mcg/dL (15-70). Therefore, lab work suggested the ovaries as the source of androgen excess, rather than the adrenals, essentially ruling out CAH as the etiology. Abdominal CT revealed a right 12 cm adnexal mass. The patient then underwent right oophorectomy. Pathology of the 12 cm ovarian mass was consistent with a steroid cell tumor, NOS. No metastasis was found on pathology or imaging. Her post-operative total testosterone levels decreased to &lt; 7 ng/dL. Hirsutism also resolved. Future surveillance imaging of pt's abdomen revealed no tumor recurrence or any metastasis.

Conclusion

This is a case of a rare ovarian tumor responsible for the excess androgenic effects found in a young female. Steroid cell tumors comprise &lt; 0.1% of all ovarian tumors1. Of the steroid producing subtypes, NOS is the most common around 60%2. This patient was younger than the average age of occurrence for this tumor, which is estimated at 43 years of age2. This case also demonstrates the importance of a thorough and complete workup prior to invasive treatment. Lab work up prior to her hysterectomy was not found. The hysterectomy resolved her menorrhagia, but did not address the etiology. A thorough biochemical workup prior to the hysterectomy may have identified the etiology sooner, thus alleviating the need for an invasive and costly surgery. References: 1. Wang PH, Chao HT, Lee RC, et al. Steroid cell tumors of the ovary: clinical, ultrasonic, and MRI diagnosis. European Journal of Radiology. 1998;26(3): 269-273 2. K. Li, F. Zhu, J. Xiong, and F. Liu, "A rare occurrence of a malignant ovarian steroid cell tumor not otherwise specified: a case report and literature review,"Oncology Letters, vol. 8, no. 2, pp. 770-774, 2014.

Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.