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Sex cord-gonadal stromal tumor



Reproductive system


Male and female reproductive system disorders
Male reproductive system disorders
Female reproductive system disorders
Reproductive system pathology review

Sex cord-gonadal stromal tumor


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High Yield Notes
13 pages

Sex cord-gonadal stromal tumor

6 flashcards

USMLE® Step 1 style questions USMLE

4 questions

A 65-year-old woman comes to the clinic due to pelvic heaviness and discomfort for the past few months. The patient also discloses bloody spotting on her underwear, which has required intermittently changing throughout the day. The patient has had no urinary symptoms or weight loss. She does not smoke or use alcohol or illicit drugs. The patient’s last menstrual cycle was 6 years ago. Her last Pap smear 2 years ago was normal. Vitals are within normal limits. On physical examination, a right-sided adnexal mass is palpated. Pelvic ultrasound shows a thickened endometrial stripe. Endometrial biopsy reveals an early stage, well-differentiated adenocarcinoma. The patient subsequently undergoes excision of the mass. The histopathological analysis is shown below:

Reproduced from: Wikimedia Commons

Which of the following is most likely to be elevated in this patient?


Content Reviewers:

Rishi Desai, MD, MPH

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 develops into ovarian follicle cells in a woman.

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 the 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 does not invade nearby tissue or spread to other parts of the body, or it can be a malignant tumor which means that it might do both of those things.

Compared with benign tumor cells, malignant tumor cells have key features like not having a clearly defined border or like a slightly less organized nuclei.

The first main type of sex-cord stromal tumor is a granulosa-theca cell tumors 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 it 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 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, and 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 is unclear, but it’s thought that the solid ovarian tumor irritates the peritoneal and pleural surfaces leading to 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.

Sex cord–gonadal stromal tumour is a group of tumors derived from the stromal component of the ovary and testis, which comprises the granulosa, thecal cells and fibrocytes. In contrast, the epithelial cells originate from the outer epithelial lining surrounding the gonad while the germ cell tumors arise from the precursor cells of the gametes, hence the name germ cell.