Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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A sclerosing stromal tumor of the ovary with masculinization in a premenarchal girl

  • Park, Soo-Min (Department of Pediatrics, Chonnam National University Medical School) ;
  • Kim, Young-Nam (Department of Pediatrics, Chonnam National University Medical School) ;
  • Woo, Young-Jong (Department of Pediatrics, Chonnam National University Medical School) ;
  • Choi, Ho-Sun (Department of Obstetrics and Gynecology, Chonnam National University Medical School) ;
  • Lee, Ji-Shin (Department of Pathology, Chonnam National University Medical School) ;
  • Heo, Suk-Hee (Department of Radiology, Chonnam National University Medical School) ;
  • Kim, Chan-Jong (Department of Pediatrics, Chonnam National University Medical School)
  • Received : 2010.09.02
  • Accepted : 2010.12.17
  • Published : 2011.05.15
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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 ${\mu}g$/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 $8.9{\times}6.2{\times}6.6$ 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.

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References

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