Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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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)
  • 투고 : 2010.09.02
  • 심사 : 2010.12.17
  • 발행 : 2011.05.15
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초록

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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참고문헌

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피인용 문헌

  1. Extragonadal sclerosing stromal tumor: A rare case report vol.40, pp.3, 2011, https://doi.org/10.1111/jog.12248
  2. Ovarian Sex Cord Stromal Tumours in Children and Young Girls - A More Than Two Decade Clinicopathological Experience in a Developing Country, Pakistan vol.15, pp.3, 2014, https://doi.org/10.7314/apjcp.2014.15.3.1351
  3. Sclerosing stromal tumor of the ovary treated with 2-incision total laparoscopic cystectomy vol.9, pp.None, 2011, https://doi.org/10.1016/j.ijscr.2015.02.002
  4. Bilateral Sclerosing Stromal Ovarian Tumor in an Adolescent vol.2015, pp.None, 2011, https://doi.org/10.1155/2015/271394
  5. Clitoromegaly in Childhood and Adolescence: Behind One Clinical Sign, a Clinical Sea vol.12, pp.4, 2018, https://doi.org/10.1159/000489385
  6. A 10-Month-Old Infant Presenting With Signs of Precocious Puberty Secondary to a Sclerosing Stromal Tumor of the Ovary in the Absence of Hormonal Elevation vol.22, pp.4, 2011, https://doi.org/10.1177/1093526618819605
  7. MR imaging in discriminating between benign and malignant paediatric ovarian masses: a systematic review vol.30, pp.2, 2011, https://doi.org/10.1007/s00330-019-06420-4