A sclerosing stromal tumor of the ovary with masculinization in a premenarchal girl.
10.3345/kjp.2011.54.5.224
- Author:
Soo Min PARK
1
;
Young Nam KIM
;
Young Jong WOO
;
Ho Sun CHOI
;
Ji Shin LEE
;
Suk Hee HEO
;
Chan Jong KIM
Author Information
1. Department of Pediatrics, Chonnam National University Medical School, Gwangju, Korea. cjkim@jnu.ac.kr
- Publication Type:Case Report
- Keywords:
Ovary;
Ovarian neoplasms;
Sex cord-gonadal stromal tumors;
Virilism
- MeSH:
Actins;
Adrenocorticotropic Hormone;
Female;
Hair;
Heterozygote;
Hirsutism;
Humans;
Hydrocortisone;
Karyotype;
Keratins;
Magnetic Resonance Imaging;
Male;
Muscle, Smooth;
Ovarian Neoplasms;
Ovary;
Pelvic Pain;
Progesterone;
Reference Values;
S100 Proteins;
Sex Cord-Gonadal Stromal Tumors;
Steroid 21-Hydroxylase;
Testosterone;
Vimentin;
Virilism;
Voice
- From:Korean Journal of Pediatrics
2011;54(5):224-227
- CountryRepublic of Korea
- Language:English
-
Abstract:
A sclerosing stromal tumor of the ovary is an extremely rare benign tumor; it usually is found during the second and third decades of life. Patients present with pelvic pain or a palpable abdominal mass. Hormonal effects such as masculinization are uncommon. Here, an 11-year old premenarchal girl presented with deepening of the voice. In addition, clitoromegaly and hirsutism with a male suprapubic hair pattern were observed. The laboratory findings showed that the testosterone level was elevated to 3.67 ng/mL, andostenedione to above 10 ng/mL, dehydroepiandrosterone-sulfate to 346 microg/dL and 17-hydroxy progesterone (17-OHP) to 11.28 ng/mL. The chromosome evaluation revealed a 46,XX female karyotype. An adrenocorticotropic hormone stimulation test was performed. The 17-OHP to cortisol ratio in 30 minutes was 0.045, which suggested a heterozygote for the 21-hydroxylase deficiency. However, the CYP21A2 gene encoding steroid 21-hydroxylase showed normal. The pelvic ultrasound showed a heterogeneous mass consisting of predominantly solid tissue in the pelvic cavity. The pelvic magnetic resonance imaging revealed an 8.9x6.2x6.6 cm mass of the left ovary. A left oophrectomy was performed and microscopic examination confirmed a sclerosing stromal tumor. Immunohistochemical studies showed that the tumor was positive for smooth muscle actin and vimentin, but negative for S-100 protein and cytokeratin. Following surgery, the hormone levels returned to the normal range and the hirsutism resolved.
