| Abstrakt: |
A child was born with ambiguous external genitals at full term. A phallus with a urethral orifice at its base was noted. The chromosomal test done at birth revealed 46,XX pattern. At the age of 13 months, a corrective surgery with vaginoplasty, clitoroplasty and repositioning of the mislocated right ovary was done. She was raised as a girl. At the age of 8 years, she presented with distension of abdomen. CT scan showed right ovarian mass with ascites. Serum inhibin level was raised. She now underwent fertility-sparing right salpingo-oophorectomy surgery. Histology revealed juvenile granulosa cell tumor (JGCT) of ovary. We wish to discuss this rare case of the child born with 46,XX disorder of sex development, which was managed by corrective plastic surgery at infancy and later developed JGCT which was managed by fertility-sparing surgery. A team of urologists, endocrinologists, geneticists, oncologists, pathologists, radiologists, gynecologists, pediatricians, pediatric surgeons and psychologists is needed to deal with this intricate case. |
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