We report a case of sex cord tumor with annular tubules featuring a giant multilocular cyst, grossly similar to cystadenoma, in the ovary of an 8.5 year old girl. Estrogen-related symptoms, including precocious puberty and irregular uterine bleeding, immediately improved after tumor resection.
Sex cord tumor with annular tubules (SCTAT) is a rare but distinct subtype of sex cord-stromal tumor of the ovary. SCTAT often produces estrogen and causes gonadotropin-independent precocious puberty and menstrual irregularities. Its clinical features are dependent on the association with Peutz-Jeghers syndrome (PJS). SCTAT associated with PJS manifests as typically bilateral, multifocal, and small (frequently microscopic) cysts and is clinically benign. By contrast, SCTAT not associated with PJS often manifests as a unilateral large cyst; 20% of such tumors have malignant potential.
We report a case of SCTAT showing a giant multilocular cyst, grossly similar to cystadenoma, in which the diagnosis had not been suspected before surgery.
Case Report
An 8.5 year old girl was admitted to our hospital presenting with breast development and a spurt in height during the previous 9 months as well as menarche followed by irregular uterine bleeding. PJS was not observed in the past history. Her height (143 cm), breast development (Tanner stage 3), and pubic hair (Tanner stage 2) were compatible with the features of precocious puberty. She also complained of a rapid increase in abdominal girth and body weight (4 kg) during the previous 3 months.
Laboratory examinations showed microcytic hypochromic anemia (hemoglobin 9.2 g/dL) and hyperestrogenemia (estradiol 138 pg/mL). Other hormone levels were as follows: testosterone, less than 0.08 ng/mL; luteinizing hormone (LH), less than 0.07 mIU/mL; follicle stimulating hormone (FSH), less than 0.05 mIU/mL; thyroid-stimulating hormone, 1.135 μIU/mL; free T3, 3.09 pg/mL; and free T4, 1.21 ng/dL. Levels of LH and FSH remained undetectable, even after an injection of LH-releasing hormone.
Ovarian tumor markers, including carcinoembryonic antigen, carbohydrate antigen 19-9, carbohydrate antigen 125, α-fetoprotein, and human chorionic gonadotropin were within the normal range. Magnetic resonance imaging (MRI) revealed a giant multilocular cyst (25 × 14 × 10 cm) in the lower abdomen ( Figure 1 ) .
