Sex cord-gonadal stromal tumor

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Sex cord-gonadal stromal tumor



Sex cord-gonadal stromal tumor


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Sex cord-gonadal stromal tumor

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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?


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.


Sex cord-stromal ovarian cancer is a type of tumor that develops from either ovarian follicle cells, or connective tissue cells. Some of these tumors are granulosa-theca cell tumors and Sertoli-Leydig cell tumors. Granulosa-theca cell tumors produce excess estradiol, which can cause very specific hormone-associated symptoms like uterine bleeding, breast tenderness, and early puberty in young girls. Sertoli-Leydig cell tumors can secrete excess testosterone, which can cause women to have more masculine features like hirsutism, which is increased hair growth.


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  3. "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
  4. "CURRENT Medical Diagnosis and Treatment 2020" McGraw-Hill Education / Medical (2019)
  5. "Mucinous Tumors of the Ovary: Current Thoughts on Diagnosis and Management" Current Oncology Reports (2014)
  6. "Sex Cord-Gonadal Stromal Tumor of the Rete Testis" Advances in Urology (2009)
  7. "Ovarian sex cord?stromal tumors?a clinicopathological study of 72 cases from the Kiel Pediatric Tumor Registry" Virchows Archiv (2003)
  8. "CD56 Is a Sensitive and Diagnostically Useful Immunohistochemical Marker of Ovarian Sex Cord-Stromal Tumors" International Journal of Gynecological Pathology (2007)

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