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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

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.

키워드

참고문헌

  1. Chalvardjian A, Scully RE. Sclerosing stromal tumors of the ovary. Cancer 1973;31:664-70. https://doi.org/10.1002/1097-0142(197303)31:3<664::AID-CNCR2820310327>3.0.CO;2-F
  2. Chang W, Oiseth SJ, Orentlicher R, Agarwal G, Yahr LJ, Cayten CG. Bilateral sclerosing stromal tumor of the ovaries in a premenarchal girl. Gynecol Oncol 2006;101:342-5. https://doi.org/10.1016/j.ygyno.2005.10.041
  3. Fefferman NR, Pinkney LP, Rivera R, Popiolek D, Hummel-Levine P, Cosme J. Sclerosing stromal tumor of the ovary in a premenarchal female. Pediatr Radiol 2003;33:56-8. https://doi.org/10.1007/s00247-002-0694-7
  4. Chang YW, Hong SS, Jeen YM, Kim MK, Suh ES. Bilateral sclerosing stromal tumor of the ovary in a premenarchal girl. Pediatr Radiol 2009;39:731-4. https://doi.org/10.1007/s00247-009-1190-0
  5. Marelli G, Carinelli S, Mariani A, Frigerio L, Ferrari A. Sclerosing stromal tumor of the ovary. Report of eight cases and review of the literature. Eur J Obstet Gynecol Reprod Biol 1998;76:85-9. https://doi.org/10.1016/S0301-2115(97)00151-6
  6. Suit PF, Hart WR. Sclerosing stromal tumor of the ovary. An ultrastructural study and review of the literature to evaluate hormonal function. Cleve Clin J Med 1988;55:189-94. https://doi.org/10.3949/ccjm.55.2.189
  7. Cashell AW, Cohen ML. Masculinizing sclerosing stromal tumor of the ovary during pregnancy. Gynecol Oncol 1991;43:281-5. https://doi.org/10.1016/0090-8258(91)90036-5
  8. Ismail SM, Walker SM. Bilateral virilizing sclerosing stromal tumours of the ovary in a pregnant woman with Gorlin's syndrome: implications for pathogenesis of ovarian stromal neoplasms. Histopathology 1990;17:159-63. https://doi.org/10.1111/j.1365-2559.1990.tb00688.x
  9. Torricelli P, Caruso Lombardi A, Boselli F, Rossi G. Sclerosing stromal tumor of the ovary: US, CT, and MRI findings. Abdom Imaging 2002;27:588-91. https://doi.org/10.1007/s00261-001-0096-6
  10. Quinn MA, Oster AO, Fortune D, Hudson B. Sclerosing stromal tumour of the ovary case report with endocrine studies. Br J Obstet Gynaecol 1981;88:555-8. https://doi.org/10.1111/j.1471-0528.1981.tb01032.x
  11. Murthy DP, SenGupta SK, Mola G, Rageau O, Mathias A. Sclerosing stromal tumour of the ovary. P N G Med J 1996;39:48-55.
  12. Calabrese M, Zandrino F, Giasotto V, Rissone R, Fulcheri E. Sclerosing stromal tumor of the ovary in pregnancy: clinical, ultrasonography, and magnetic resonance imaging findings. Acta Radiol 2004;45:189-92. https://doi.org/10.1080/02841850410004148

피인용 문헌

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  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