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Neonatal bilateral ovarian sex cord stromal tumors
Author(s) -
Capito Carmen,
Flechtner Isabelle,
Thibaud Elisabeth,
Emond Sophie,
Kalfa Nicolas,
Jaubert Francis,
Sarnacki Sabine
Publication year - 2009
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.21775
Subject(s) - medicine , breast enlargement , ovary , testosterone (patch) , ovarian cyst , oophorectomy , umbilical cord , gynecology , cyst , surgery , endocrinology , anatomy , hysterectomy
A child was referred for evaluation after prenatal diagnosis with macrosomia, clitoromegaly, labial hypertrophy, and a left ovarian cyst. The karyotype was 46,XX. The postnatal pelvic ultrasound was normal. High levels of anti‐mullerian hormone and testosterone led to a hCG stimulation test, which was followed by isosexual precocious puberty and the appearance of a bilateral ovarian enlargement with a left tumoral mass. A left ovarian tumorectomy revealed a fibrothecoma. Six weeks later, a tumoral relapse occurred and completion of oophorectomy revealed a juvenile granulosa cell tumor (JGCT). Whereas hormonal levels decreased after surgery, a new rise associated with an enlargement of the right ovary led to the diagnosis of right JGCT. A right oophorectomy was proposed to the parents, who declined further surgery. After 2 months, the hormonal levels normalized. This case illustrates the confusing overlap between developmental and neoplastic biology in neonates. Pediatr Blood Cancer 2009;52:401–403. © 2008 Wiley‐Liss, Inc.