Pseudo-precocious puberty caused by a juvenile granulosa cell tumour secreting androstenedione, inhibin and insulin-like growth factor-I.
We report a female child who presented at age 3.92 years with a 2-year history of consonant pubertal development caused by a large right-sided ovarian juvenile granulosa cell tumour (JGCT). Although JGCTs causing pseudo-precocious puberty have been previously described, they remain rare and endocrine data are often incomplete. In this case the tumour was associated with raised serum oestradiol, androstenedione, inhibin and IGF-I. Histological changes were consistent with JGCT. Immunohistochemical studies revealed positive reactivity to MIC-2, inhibin, melan A, IGF-I and IGFBP-2.
Chan
L F
LF
Department of Paediatric Endocrinology, St Bartholomew's and The Royal London School of Medicine and Dentistry, London, UK.
Storr
H L
HL
Scheimberg
I
I
Perry
L A
LA
Banerjee
K
K
Miraki-Moud
F
F
Camacho-Hübner
C
C
Savage
M O
MO
D000735:Androstenedione / Q000378:metabolism*; D002675:Child, Preschool; D005260:Female; D006106:Granulosa Cell Tumor / Q000150:complications / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D007265:Inhibins / Q000378:metabolism*; D007334:Insulin-Like Growth Factor I / Q000378:metabolism*; D010051:Ovarian Neoplasms / Q000150:complications / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000473:pathology; D011629:Puberty, Precocious / Q000209:etiology*; D014057:Tomography, X-Ray Computed
D000735:Androstenedione; D007265:Inhibins; D007334:Insulin-Like Growth Factor I