Neonatal bilateral ovarian sex cord stromal tumors.
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.
Capito
Carmen
C
Department of Pediatric Surgery, Malades Hospital, Paris V University, Paris, France.
Flechtner
Isabelle
I
Thibaud
Elisabeth
E
Emond
Sophie
S
Kalfa
Nicolas
N
Jaubert
Francis
F
Sarnacki
Sabine
S
D005260:Female; D006801:Humans; D007231:Infant, Newborn; D010051:Ovarian Neoplasms / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D018312:Sex Cord-Gonadal Stromal Tumors / Q000235:genetics / Q000473:pathology* / Q000601:surgery