A sclerosing stromal tumor of the ovary with masculinization in a premenarchal girl / 소아과
Korean Journal of Pediatrics
; : 224-227, 2011.
Article
in En
| WPRIM
| ID: wpr-91729
Responsible library:
WPRO
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.
Key words
Full text:
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Index:
WPRIM
Main subject:
Ovarian Neoplasms
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Ovary
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Progesterone
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Reference Values
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Testosterone
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Vimentin
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Virilism
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Voice
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Hydrocortisone
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Magnetic Resonance Imaging
Limits:
Female
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Humans
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Male
Language:
En
Journal:
Korean Journal of Pediatrics
Year:
2011
Type:
Article