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A 12-year old girl presents with dysmenorrhea. Menarche started one year ago, but she has been bleeding heavily and irregularly for the last 6 months. She had been taking oral contraceptives, but her bleeding has not been controlled. Her ultrasound and CT show a large pelvic mass.

 

1.jpeg.30dd791e20b2529d8f3aee15dffe99f4.jpeg. 2.jpeg.0b19f6c97d1885c058e49b41137ff4f6.jpeg. 3.jpeg.ab7d50fae7ae9318902e697abc0dd462.jpeg

 

[size=20px]Serum levels of Inhibin A and B are elevated. At operation, a 20 cm right ovarian mass is seen. It does not involve the ipsilateral tube. Peritoneal washings are obtained. No other lesion is noted. The most appropriate management for this ovarian tumor is:

 

A Ovarian sparing mass excision

 

B Uniteral salpingo-oophorectomy

 

C Unilateral oophorectomy and endometrial biopsy

 

D Unilateral salpingo-oophorectomy and endometrial biopsy

 

E Unilateral salphingo-oophorectomy and contralateral ovarian biopsy[/size]

  • Author

correct answer is

 

B Uniteral salpingo-oophorectomy

 

This tumor is likely a granulosa tumor (GCT), a sex cord-stromal tumor that can cause higher than normal estrogen production in women. There are two distinct types: juvenile (JGCT) and adult (AGCT). Due to the estrogen production, JGCT causes isosexual precocious puberty.

 

For both JGCT and AGCT, stage is the most important prognostic factor. Stage I disease, which is confined to the ovary, has a 84-95% 10 year survival; compared to 50-65% for Stage II disease and 17-33% for Stages III and IV. Patients with Stage I tumor are classified into low and high risk based on tumor size (>20 cm), poor differentiation, high mitotic index or tumor rupture.

 

Like this patient, most JGCT are diagnosed early (Stage I). The recommended surgical treatment for unilateral disease is salpingo-oophorectomy, along with surgical evaluation of the peritoneal surfaces, sampling of abnormal lymph nodes along the iliac vessels and distal aorta/ inferior venal cava, and omental biopsies (if lesions are present). Ovarian sparing mass excision is not recommended in this tumor due to possibility of tumor recurrence and possible spillage. In adult GCT, 10% are associated with endometrial carcinoma prompting some experts to advocate for preoperative endometrial biopsies, especially in patients over age 40.

 

GCTs may also occur in testicular tissue where testicular-sparing procedures are recommended.

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