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

• Published in: Clinical and experimental pediatrics Vol. 54; no. 5; pp. 224 - 227
• Main Authors: Park, Soo Min, Kim, Young Nam, Woo, Young Jong, Choi, Ho Sun, Lee, Ji Shin, Heo, Suk Hee, Kim, Chan Jong
• Format: Journal Article
• Language: English
• Published: Korea (South) Clinical and Experimental Pediatics / Korean Pediatric Society 01.05.2011; The Korean Pediatric Society; Korean Pediatric Society; 대한소아청소년과학회
• Subjects: Age; Case Report; Cytokeratin; Fibroblasts; Histopathology; Magnetic resonance imaging; Medical schools; Menstruation; Ovarian neoplasms; Ovaries; Ovary; Proteins; Sex cord-gonadal stromal tumors; Smooth muscle; Surgery; Testosterone; Tumors; Virilism; 소아과학; Ovary; Ovarian neoplasms; Sex cord-gonadal stromal tumors; Virilism
• ISSN: 1738-1061; 2092-7258; 2092-7258; 2713-4148
• DOI: 10.3345/kjp.2011.54.5.224

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 µg/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 8.9×6.2×6.6 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.