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Diagnostic Ultrasound - Abdomen and Pelvis

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Sex Cord-Stromal Tumor<br />

TERMINOLOGY<br />

Definitions<br />

• Group of ovarian tumors arising from either embryonic sex<br />

cords or mesenchyme<br />

○ Fibroma, thecoma, fibrothecoma<br />

○ Granulosa cell tumor: Occurs in both adult <strong>and</strong> juvenile<br />

forms<br />

○ Sertoli-Leydig tumor (<strong>and</strong>roblastoma)<br />

○ Sclerosing stromal tumor, steroid cell tumors,<br />

gyn<strong>and</strong>roblastoma, <strong>and</strong> sex cord tumor with annular<br />

tubules<br />

IMAGING<br />

General Features<br />

• Sex cord-stromal tumors are generally solid or have<br />

significant solid components<br />

• Hormonally active tumors may be small <strong>and</strong> difficult to find<br />

Ultrasonographic Findings<br />

• <strong>Ultrasound</strong> findings of sex cord-stromal tumors are diverse<br />

<strong>and</strong> nonspecific<br />

○ Range from small, solid tumors to large, multicystic<br />

masses<br />

• Granulosa cell tumors<br />

○ More often contain cysts, with a sponge-like, Swiss<br />

cheese appearance<br />

○ Cysts may be complex <strong>and</strong> contain hemorrhagic fluid<br />

– May rupture <strong>and</strong> cause hemoperitoneum<br />

○ Adult <strong>and</strong> juvenile forms have similar appearance<br />

○ Cysts will be thick-walled<br />

○ Calcifications are rare<br />

○ May be bilateral in 5%<br />

• Sertoli-Leydig tumors<br />

○ Significant overlap with granulosa cell tumors<br />

○ Not as frequently cystic as granulosa cell tumors<br />

– 70% purely solid<br />

– 95% have solid component<br />

○ Less likely to have hemorrhage<br />

• Fibrothecomas<br />

○ Hypoechoic with dense posterior acoustic attenuation<br />

– Similar to uterine leiomyoma<br />

○ 1% associated with Meigs syndrome<br />

• Steroid cell tumors<br />

○ Typically small, without cysts<br />

MR Findings<br />

• T1WI<br />

○ May see high signal from hemorrhage in granulosa cell<br />

tumor<br />

○ High lipid content may cause steroid tumors to be high<br />

signal<br />

• T2WI<br />

○ Most intermediate signal with cystic area being high<br />

signal<br />

– Granulosa cell tumors may have network of smaller<br />

cysts creating sponge-like appearance<br />

– Fibrothecomas tend to be mild to markedly<br />

hypointense (compared to myometrium)<br />

• T1WI C+<br />

○ Most enhance avidly except fibrothecomas<br />

Imaging Recommendations<br />

• Protocol advice<br />

○ Evaluate uterus carefully<br />

– Hormonal stimulation may cause uterine enlargement<br />

<strong>and</strong> endometrial thickening (hyperplasia, polyps, or<br />

carcinoma) <strong>and</strong> can be tip-off to correct pathology<br />

DIFFERENTIAL DIAGNOSIS<br />

Ovarian Carcinoma<br />

• Most epithelial tumors have dominant cystic component<br />

• Confusion may occur if there is large, solid component<br />

Germ Cell Tumors<br />

• Much more heterogeneous with calcifications, fluid-fluid<br />

levels, dirty posterior shadow<br />

Ovarian Torsion<br />

• Edematous, enlarged ovary with peripheral cysts<br />

• Patient is acutely symptomatic<br />

Hormonally Functioning Ovarian Masses<br />

• Patients may present with symptoms related to hormone<br />

production rather than mass<br />

• May present with either hyper<strong>and</strong>rogenism or<br />

hyperestrogenism (some may do both)<br />

• Hyper<strong>and</strong>rogenism (virilization with hirsutism, male<br />

pattern baldness, loss of female body contour,<br />

clitoromegaly)<br />

○ Sertoli-Leydig tumor<br />

○ Sclerosing stromal tumor<br />

○ Gonadoblastoma<br />

○ Brenner tumor<br />

○ Polycystic ovarian disease<br />

○ Stromal hyperplasia<br />

○ Stromal hyperthecosis<br />

○ Hyperreactio luteinalis<br />

○ Nonovarian causes<br />

– Pituitary (Cushing disease)<br />

– Adrenal (Cushing syndrome)<br />

• Hyperestrogenism (pseudoprecocious puberty,<br />

postmenopausal bleeding)<br />

○ Granulosa cell tumor<br />

○ Thecoma<br />

○ Serous tumors<br />

○ Mucinous tumors<br />

○ Endometrioid tumors<br />

○ Autonomously functioning follicular cyst most common<br />

in isosexual pseudoprecocious puberty<br />

PATHOLOGY<br />

General Features<br />

• Etiology<br />

○ Derive from 2 embryologically distinct groups of cells<br />

– Stromal cells: Fibroblasts, theca cells, <strong>and</strong> Leydig cells<br />

– Sex cords: Granulosa cells <strong>and</strong> Sertoli cells<br />

○ Most tumors have more than 1 cell type<br />

• Associated abnormalities<br />

○ Adult granulosa cell tumor<br />

Diagnoses: Female <strong>Pelvis</strong><br />

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