Ovarian sex-cord stromal tumors

With sex cord-stromal ovarian cancer, Ovarian refers to “ovary”, of which women have two that sit along either side of the uterus.

The term sex cord refers to an embryonic structures that develop into ovarian follicles and stromal cells are the connective tissue of any organ.

So a sex cord-stromal ovarian cancer is a type of tumor that develops from either ovarian follicle cells or connective tissue cells.Each ovary has multiple follicles.

Each follicle is made up of an oocyte, which is an immature egg, surrounded by two types of cells - theca cells and granulosa cells.

Granulosa and theca cells work together to support follicle development. Luteinizing hormone stimulates theca cells to generate androgens and follicle stimulating hormone stimulates granulosa cells to convert those androgens to estradiol using the enzyme aromatase.

A large increase in estradiol triggers ovulation.During ovulation, the oocyte pops out of the ovary, causing a bit of damage to the surface.

Fibrocytes detect that damage and differentiate into fibroblasts and lay down collagen to help repair the damage. If any of those cells starts to divide uncontrollably, it can either form a benign tumor which means that it doesn’t invade nearby tissue or spread to other parts of the body, or it can be a malignant tumor which means that it can invade nearby tissue and spread to other parts of the body.

Compared with benign tumor cells, malignant tumor cells have key features like not having a clearly defined border or like having slightly less organized nuclei.The first main type of sex-cord stromal tumor is a granulosa-theca cell tumor.

And these are the most common malignant stromal tumors and they’re associated with middle-aged women. These tumors often end up producing way too much estradiol, and this can cause very specific hormone associated symptoms like uterine bleeding, breast tenderness, and early puberty in young girls.

Under the microscope, these tumors classically develop little fluid pockets scattered throughout the tissue that are called Call-Exner bodies.

The second type of tumors, fibromas, are made of fibroblasts and are benign tumors. Under the microscope, they look like thin needle-like strands with elongated nuclei that are bundled together.

Benign fibromas are often seen in combination with ascites, a fluid buildup in the peritoneal cavity, as well as pleural effusion, a fluid buildup in the pleural cavity.

In fact, the clinical triad of a benign ovarian tumor with ascites and a pleural effusion, is better known as Meigs syndrome.

The exact pathogenesis here, though, is unclear, but it’s thought that the solid ovarian tumor irritates the peritoneal and pleural surfaces which leads to a transudative fluid buildup in both spaces.

Fibromas can occasionally grow to the size of an orange and can cause a pulling sensation in the groin when it compresses the round ligament of the uterus.

The third type of tumors are called Sertoli-Leydig cell tumors because they look like Sertoli and Leydig cells normally found in men.

It turns out that the tumors are actually made of primitive gonadal stroma, which is so undifferentiated that the cells can secrete androgens like testosterone.

Increased levels of testosterone can cause women to have more masculine features like hirsutism, which is increased hair growth.

Under the microscope, classically there are Reinke crystals which are pink rod-like crystals in the cytoplasm of the cells, and these look kinda like bright pink magical crystals you’d collect in a video game.So during ovulation, the follicle ruptures and releases an egg which inadvertently leads to epithelial cell damage.

To fix that damage, the epithelial cells have to undergo cell division to replace and heal the tissue. Each time cells divide there is a chance of a mutation and the possibility of tumor formation.

And this means that with more ovulatory cycles there’s an increased risk of tumor formation. So things that are associated with a decreased risk of Ovarian cancer include things that reduce the number of ovulatory cycles like pregnancy, breastfeeding, and oral contraceptive use.

On the flip side, some things that are associated with an increased risk include certain medical conditions like endometriosis and polycystic ovarian syndrome.

There are also some genetic risk factors like having the BRCA-1 or BRCA-2 mutation, which are both autosomal dominant mutations, which in addition to ovarian cancer, carry with them an increased risk of breast cancer.

There’s also hereditary nonpolyposis colorectal cancer, also known as Lynch syndrome, which increases the risk of developing a number of cancers, including ovarian cancer.

Generally speaking, symptoms of ovarian cancers can be subtle and non-specific. Common early symptoms can include abdominal distension, bloating, as well as abdominal or pelvic pain, which can come from an ovarian torsion - where the ovary gets twisted.

Occasionally, ovarian tumors can cause ascites, abdominal masses, bowel obstruction, or dyspareunia, which is pain during sexual intercourse.

A classic finding is a Sister Mary Joseph nodule which happens when the cancer metastasizes to the umbilicus. This finding is often linked with a few types of cancer - one of which is ovarian cancer.

Diagnosis of ovarian cancer typically involves looking for specific tumor markers like B-hCG as well as having a transvaginal ultrasound.

Tumor biopsies are done to figure out whether a growth is benign or malignant, and imaging with a CT or MRI can be done to look for evidence of metastasis.

Treatment of ovarian cancer typically involves chemotherapy, surgery, and sometimes radiotherapy. Surgery might be enough for malignant tumors confined to the ovary, whereas chemotherapy might be needed for diseases that spread.

Carbohydrate antigen 125, called CA 125, is a protein produced by various types of ovarian tumors, so tracking levels of this biomarker in the blood can help monitor response to therapy and potential relapse.

All right, as a quick recap… Sex cord-stromal ovarian cancers originate from connective tissue in the ovary. Granulosa-theca cell tumors can secrete excess estradiol as well as develop Call-Exner bodies, fibromas can become large and are associated with Meigs syndrome, and Sertoli-Leydig cell tumors can secrete excess testosterone and develop Reinke crystals.

Increased risk for developing ovarian cancers is associated with increased cumulative amount of time a woman spends in ovulation, and therefore decreased risk is associated with pregnancy, breastfeeding, and oral contraceptives.

Hey guys what's up thanks for watching that video on sex cord stromal ovarian cancer Um If you want to do a deeper dive on that topic you should definitely head over to us moses dot org we've got a bunch of flash cards and quiz questions and other awesome studying tools to help you learn medicine um So if you're interested in that you should definitely check it out Otherwise you can always support us by donating on patry in and subscribing to our channel and following us on social media This particular video is written by Fenyang and I believe this was her first script so um I think it turned out really well so congrats for yang and uh thanks for writing that script for us Um It was edited by Rishi as well as Andrea and Kyle Um I did the illustrating in the voiceover and Yifan did the video editing So thank you to everyone who put this video together I think it turned out really well Hopefully you liked it thanks guys until next