Korean J Pediatr.  2011 May;54(5):224-227. 10.3345/kjp.2011.54.5.224.

A sclerosing stromal tumor of the ovary with masculinization in a premenarchal girl

• Park SM
• Kim YN
• Woo YJ
• Choi HS
• Lee JS
• Heo SH
• Kim CJ

• KMID: 2284873
• DOI: http://doi.org/10.3345/kjp.2011.54.5.224

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.

Keyword

Ovary; Ovarian neoplasms; Sex cord-gonadal stromal tumors; Virilism

MeSH Terms

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
Actins
Adrenocorticotropic Hormone
Hydrocortisone
Keratins
Progesterone
S100 Proteins
Steroid 21-Hydroxylase
Testosterone
Vimentin