Steroid Cell Tumor of the Ovary (NOS): Anyone else?

Hi @pandamom, I was unfamiliar with ovarian steroid cell tumors, so I looked it up. They are rare sex cord-stromal tumors of the ovary. Do you know what not otherwise specified (NOS) refers to?

I'm grateful that you are willing to share your experience for new members who may join with this rare condition. While I don't have it myself, I'd like to learn more about your experience.
- Was your tumor benign or malignant?
- Did you have a long journey to get a diagnosis because of its rarity?
- What, if any, treatments did you have?
- How often are your followup appointments and what tests do you do?

Hi @colleenyoung. You asked some excellent questions. I am not a medical professional and the information below is just my lay understanding. I apologize for the delay in responding to your questions.

Someone else much more qualified than I would be in a better position to explain what not otherwise specified (NOS) refers to. There are three subtypes of steroid cell tumors: stromal luteoma, Leydig cell tumor, and steroid cell tumor not otherwise specified (NOS). It appears a diagnosis of NOS is given because the steroid cell tumor I was diagnosed with does not meet the criteria for the first two subtypes but is still a steroid cell tumor. It may be that it has not been more fully described due to its rarity.

Speaking of its rarity, I really do not have a good understanding as to how rare this condition is. The research I have found states that steroid cell tumors of the ovary are rarely identified and only constitute about 0.1% of ovarian tumors and the NOS subtype account for about 56% of all steroid cell tumors. So, I have been unable to figure out what that means as a ratio such as 1 in X number women are diagnosed with this condition.

I had tumors behind each ovary. Neither tumor had features that would have made it clinically malignant (there was no necrosis, they were smaller than 7 cm, there was no hemorrhage, and some other features I’ve forgotten) so my tumors were considered to be benign. Since these growths were benign, the only treatment I have undergone is a total hysterectomy. Strangely, it is my understanding that, even though they were benign, some steroid cell tumors NOS have been found to still *act* malignant, which is very confusing. My gynecologic oncologist explained that the tumors were categorized as a tumor of uncertain malignant potential because it is associated with the risk of malignant degeneration.

I definitely had a long journey to get a diagnosis. This was in part, I’m sure, due to its rarity. It was also in part, however, by my own reluctance to seek treatment due to embarrassment. You see, this type of tumor causes an increase in testosterone levels which, in turns, causes hyperandrogenism, like hair growth in areas usually only experienced by males, deepening of the voice, and male pattern baldness. It can also cause early menopause.

By the time I was diagnosed in 2017, I had significant hair growth not only on my face (in the area where you would expect beard growth), but also on my back, low back, and chest including a circle of hair around my areolas. I was shaving my face twice a day (in the morning and in the evening) to try to keep my face smooth. I also had developed male pattern baldness that had caused my hairline to recede. This was very noticeable and there was very little I could do to hide that.

After my diagnosis, blood work was done to assess my testosterone levels and I was told that my testosterone would be considered high even for a man. Pretreatment CA125 was 39.5 and my free testosterone, total testosterone levels were elevated at 6.2 and 44, respectively. Following my total hysterectomy, these levels dropped down to normal levels within a few months. The hair on my back and chest went away. I still battle with thick coarse hair in the beard area but only have to shave once a day at this point. I believe a little of my hair has regrown as well but my hairline is still high.

I am sure that the rarity of this condition caused a delay in diagnosis. There were some signs, though, that I believe were missed or not investigated as thoroughly as they should have been. I realize I am saying that in retrospect. My period began early at age 9. My periods were always unpredictable, to the point that I began taking birth control pills at age 16 to help control them. My husband had a vasectomy in 2001, so I stopped taking birth control at that time. I was 27 years old at that time. At around age 33, my periods became incredibly sporadic. I would go anywhere between 21 days to 42 days between periods and they were completely unpredictable. Around age 34, my periods began to get further and further apart and finally stopped at age 35, which meant that I officially entered menopause at age 36. I had some pretty severe hot flashes. I was seeing my gynecologist and my PCP regularly during this time. My PCP had told me when I was approximately 34 that I was too young to be going through menopause. I do not think my gynecologist believed my periods were so sporadic or that my cycle had completely stopped. Even the gynecologist I saw who performed the first surgery (a laparoscopic left salpingo-oophorectomy) told my husband and me while I was in preop that she might have to do a total hysterectomy if she saw something concerning during the surgery. I encouraged her to go ahead and just do a total hysterectomy, but she insisted this would throw me into menopause. My husband and I reminded her that I had not had a period in 8 years. My gynecologist's unwillingness to perform a total hysterectomy led to me having to have a second surgery a few weeks later to have a total hysterectomy.

Several years before my diagnosis, my PCP referred me to an endocrinologist due to the hair growth and I was diagnosed with hirsutism and elevated testosterone levels. At one point, when I told my endocrinologist that I had to shave my face daily and he told me that I should not have done that so he could see the severity of the hair growth. At my next appointment with him, I purposefully did not shave that morning and he looked appalled at the hair growth and I felt he was grossed out by it, too. This was, of course, before the pandemic when people regularly wore facial masks, so I was very embarrassed even walking into the doctor's office and being seen by other patients and other people working in his office. This was not investigated any further and no treatment was offered, other than continuing to follow up with the endocrinologist.

Since my tumors were found to be benign, it’s my understanding that I have a good prognosis. Since this is such a rare condition, the treatment course (other than a total hysterectomy) is really unknown other than to monitor me. I follow up with my gynecologic oncologist 3-4 times per year. She performs a pelvic exam and I have labs drawn to monitor my CA125 and testosterone levels. She has asked that I do not undergo laser hair removal as, if this gets worse, this may be a sign that I should report.

Wow! Thank you Pandamom for such a detailed response. You are obviously an "expert by experience", namely patient experience. This information not only helps me understand, it will be really useful information for the next members with NOS who joins the community.

Dealing with facial hair can't be easy. Your frankness about it will encourage others too. Thank you.