Diagnostic Ultrasound - Abdomen and Pelvis

Sex Cord-Stromal Tumor
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Diagnoses: Female Pelvis
– Endometrial hyperplasia, polyps, and carcinoma
○ Juvenile granulosa cell tumor
– Pseudoprecocious puberty
– Ollier disease (multiple enchondromas)
– Maffucci syndrome (multiple enchondromas and
hemangiomas)
○ Sertoli-Leydig most common virilizing tumor
– Amenorrhea, hirsutism, deepening voice, male
pattern baldness
○ Sex cord tumor with annular tubules
– Peutz-Jeghers syndrome (autosomal dominant
disorder with multiple gastrointestinal hamartomas
and mucocutaneous pigmentation); ovarian tumors
often bilateral
○ Fibrothecoma
– Gorlin syndrome (odontogenic keratocysts of jaw,
basal cell carcinoma, intracranial calcification, plantar
and palmer pits, and craniofacial anomalies)
Microscopic Features
• Granulosa cell tumors are composed of granulosa cells
growing in numerous patterns
○ Frequently accompanied by theca cells and fibroblasts
• Sertoli-Leydig cell tumors are composed of Sertoli cells,
Leydig cells, and fibroblasts
○ May have tumors from single cell line
• Steroid cell tumors contain lutein cells, Leydig cells, and
adrenocortical cells
CLINICAL ISSUES
Presentation
• Smaller masses may be incidental findings
• Pelvic pain/discomfort from larger masses
• Symptoms related to hormone production
• Fibromas may have elevated CA125
• May be associated with Meigs syndrome (pleural effusion,
ascites, which resolve upon removal of benign mass)
• Granulosa cell tumors and thecomas are estrogen
producing tumors
○ Clinical effects depend on patient age
○ Pseudoprecocious puberty in pediatric population
– Not true precocious puberty because no ovulation or
progesterone production
– Present in 80% of juvenile granulosa cell tumors
– Juvenile granulosa cell tumors account for 10% of
precocious puberty cases
○ Uterine bleeding in postmenopausal patient
– Endometrial stimulation with hyperplasia or
carcinoma
– 30-50% have hyperplasia
– 3-25% have endometrial carcinoma
○ Women in reproductive age group may have irregular,
heavy periods
• Sertoli-Leydig tumors are androgen-producing
○ Symptoms in 30% of patients
Demographics
• Epidemiology
○ Sex cord-stromal tumors represent 5-10% of ovarian
neoplasms and 2% of ovarian malignancies
○ Distribution of sex cord-stromal tumors
– ~ 50% are fibrothecomas
– 10-20% granulosa cell tumors
– 5% Sertoli-Leydig tumors
– Remainder include sclerosing stromal tumor, steroid
cell tumors, gynandroblastoma, and sex cord tumor
with annular tubules
○ Granulosa cell tumors occur in 2 distinct groups (juvenile
and adult)
– 5% are juvenile granulosa cell tumor and present < 30
years
– Mean age for juvenile granulosa cell tumors is 13
years, with many presenting before puberty
– 95% are adult granulosa cell tumors and present in
perimenopausal and postmenopausal women (mean
age: 52 years)
○ Sertoli-Leydig tumor, mean age: 25 years
Natural History & Prognosis
• Many are low-grade malignancies and surgery is curative
• Juvenile granulosa cell tumors have excellent prognosis
○ Most are stage 1
• Adult granulosa cell tumors may act in more aggressive
fashion with late recurrences (potentially decades) not
uncommon
○ > 90%: Stage 1
○ 90-95%: 5-year survival for stage 1
○ 25-50%: 5-year survival for advanced disease
○ Mean survival after recurrence: 5 years
• 80-90% of Sertoli-Leydig cell tumors are stage 1 and are
cured with resection
○ 10-20%: Behave in more malignant fashion
○ Most recurrences are in 1st 5 years
• Fibrothecomas are benign
DIAGNOSTIC CHECKLIST
Consider
• Key features differentiating sex cord-stromal tumors from
more common epithelial neoplasms
○ More likely to present with symptoms from hormone
production
○ Most are stage 1 with good prognosis
○ Affect all age groups, including pediatrics
○ More often solid
○ Cystic masses less likely to have papillary projections
Image Interpretation Pearls
• Multicystic lesion with hemorrhage in patient under 30
strongly suggests juvenile granulosa cell tumor
• Granulosa tumors are most common hormonally active
tumor and produce estrogen
○ Thecomas: 2nd most common estrogen producing
ovarian tumor
• Sertoli-Leydig cell tumor most common virilizing ovarian
tumor
SELECTED REFERENCES
1. Heo SH et al: Review of ovarian tumors in children and adolescents:
radiologic-pathologic correlation. Radiographics. 34(7):2039-55, 2014
2. Yen P et al: Ovarian fibromas and fibrothecomas: sonographic correlation
with computed tomography and magnetic resonance imaging: a 5-year
single-institution experience. J Ultrasound Med. 32(1):13-8, 2013