Adnexal Masses Part IV: Sex Cord Stromal Tumors

Thanks for sticking with us until the end of this adnexal mass journey! Today we’re going to cover some rare tumors that always find themselves on CREOGs — the sex cord stromal tumors. These only comprise about 1.2% of primary ovarian cancers. Most people are fortunately diagnosed at an early stage due to the fact that symptoms tend to be much more overt with these types of tumors.

Granulosa Cell Tumors

There are two subtypes of granulosa cell tumors: adult and juvenile. Adult type comprises 95% of these neoplasms, and generally occur in women aged 50-54 years. Juvenile type typically develops before puberty. It has a higher proliferative rate, but lower risk for late recurrences. Regardless of type, these typically present as a large, unilateral mass clinically, with a mean diameter of 12cm. They can produce estrogen and/or progesterone, so symptoms can be related to hyperestrogenism particularly in juveniles (i.e., precocious puberty). The production of estrogen in adult types is also associated with concomitant endometrial hyperplasia or cancer; with EIN present in 25-50%, and endometrial carcinoma present in 5-10% of patients. Thus, it is important to perform endometrial sampling when one of these tumors is suspected or diagnosed.

The histopathology is classic: “Call-Exner bodies", where the pale, round, coffee-bean shaped nuclei characteristic of granulosa cells arrange themselves into rosettes around a central cavity.

Thecomas

Thecomas are solid, fibromatous, generally benign neoplasms. They are generally unilateral, and are comprised of theca cells. The theca cells appear in normal ovulation as follicles develop into secondary follicles, and under the influence of LH produce androgens. After ovulation, theca cells also help to form the corpus luteum with granulosa cells.

Because of this high production of androgen that will be converted, endometrial hyperplasia or cancer can also be found in these patients, and it is wise to perform endometrial sampling for that reason. Up to 20% of patients may have synchronous endometrial cancer.

Fibromas

These are the most common type of sex cord stromal tumor. They are benign, solid, unilateral neoplasms, generally occurring in postmenopausal women, and are not hormonally active. However these can be implicated in Meigs’ syndrome, where the tumor is associated with extensive ascites or a pleural effusion.

Sertoli / Leydig Cell Tumors

These two are the rarest of the sex cord stromal tumors, accounting for less than 0.5% of these. The histopathology of the hollow tubules (Sertoli) surrounded by fibrous stroma (Leydig) is classic. These will often produce androgens and be associated with virilizing symptoms. They also are unilateral and are often associated with large masses, with a mean size of 16cm at presentation. AFP is often another marker.

Adnexal Masses Part III: Germ Cell Tumors

Germ cell tumors are our next foray into these adnexal masses. They comprise 20-25% of ovarian neoplasms, can be benign or malignant, and occur generally in younger women: between ages 10-30 years.

Many of these for CREOGs are distinguished by specific tumor markers and specific histopathology. We’ve put a brief table together here to help with the episode and get some of those visual references!

(C) CREOGs Over Coffee (2019)

Adnexal Masses Part 1: Imaging

Today we’re embarking on a multi-part series through adnexal masses.

To frame our initial conversation on imaging features of adnexal masses, we’ve relied heavily on a golden piece of literature from the Radiological Society of North America, detailing the features and management of these findings on imaging. This paper contains a super nice table that should be considered a table-side reference for your own viewing of images.

Generally speaking, signs more suggestive of malignancy include:

  • Patient age/menopausal status: One of the biggest contributing risk factors, even before you know what the cyst looks like. In postmenopausal women with asymptomatic adnexal masses, the incidence of malignancy approaches 30%, while it is only 6-11% in premenopausal women.

  • Large size: cysts greater than 5cm should receive consideration for surgical intervention or closer follow up in premenopausal women. In postmenopausal women though, even small 1cm cysts should be considered for close interval follow up at a minimum.

  • Thickness: thicker walls (>3mm) portend more significant pathology.

  • Septations: multiple septations are also concerning for malignancy, though again this corresponds with the thickness; thinner septations may suggest more likely benign disease.

  • Nodularity: cysts with nodules or calcifications, particularly with vascularity, are more concerning.

  • Contents: one of the more nuanced findings; however, can help determine etiology: i.e., cysts with a reticular or lacy appearance are more suggestive of hemorrhagic cysts, while hyper echoic lines and dots with areas of acoustic shadowing are more suggestive of dermoid cysts.

Be sure to also check out ACOG PB 174 (membership required) and/or the OBG Project’s helpful bulleted summary! We definitely think looking through images alongside descriptive text is the primary way to learn this information, and we hope the podcast can help supplement that for some of you.