To investigate the imaging and pathological characteristics of cystic juvenile granulosa cell tumor (CJGCT) of the ovary.

A retrospective analysis of the clinical symptoms, signs, imaging, and pathological characteristics of 2 patients with CJGCT treated at Nanjing Drum Tower Hospital in 2025, and a review of the relevant literature to summarize the key diagnostic points.

Case 1: a 12-year-old girl presented with lower abdominal discomfort and vomiting for several days. Ultrasonography revealed a large unilocular cystic mass in the abdominopelvic cavity (approximately 20.6 cm × 20.9 cm × 9.7 cm), with the right ovary not visualized; the cyst volume had increased by about 10% within 12 days. Case 2: a 16-year-old girl presented with a rapidly enlarging abdominal mass within 5 days, palpated up to 1 cm above the umbilicus, without abdominal pain or vaginal bleeding. Transrectal color Doppler ultrasound showed a cystic lesion in the pelvis (13.8 cm × 7.0 cm × 16.4 cm). On CT, both tumors appeared as homogeneous low density cystic masses. Serum tumor markers and sex hormone levels were within normal limits.Histopathology demonstrated entirely cystic tumors with smooth external surfaces and cyst walls measuring 0.3-0.5 cm in thickness, containing clear or slightly mucoid fluid. Immunohistochemistry showed steroidogenic factor 1 (+ + ), inhibin-α (weak + ), and calretinin (-). Reticulin staining highlighted a positive network surrounding tumor cell nests. The final pathological diagnosis was CJGCT in both cases. Both patients underwent fertility sparing excision of the ovarian lesion and were followed up for 4-6 months without recurrence.

CJGCT is characterized by complete cystic change, is clinically rare, and may be easily misdiagnosed by imaging examination. Definitive diagnosis relies on postoperative histopathological confirmation in conjunction with clinical and imaging findings. Fertility sparing conservative surgery is the preferred treatment, while the necessity of adjuvant chemotherapy or secondary surgery should be determined based on tumor stage and pathological high risk factors.