Very premature pubarche in girls is not a pubertal variant.
Premature pubarche (PP) in girls is considered to be a benign phenomenon and is the clinical expression of premature adrenarche. Since it does not usually increase the risk of either abnormalities in pubertal development or a reduced final adult height, a non-interventional approach is generally adopted after exclusion of non-classical (NC)congenital adrenal hyperplasia (CAH). Extremely premature pubarche is nevertheless not a pubertal variant. We here report two cases of adrenocortical tumors (ACT) for which precocious pubarche was the initial sign. This report suggests that premature pubarche is not always a mere pubertal variant and that a delay in the etiological diagnosis can be harmful. Therefore, pubarche before the age of four years should be considered as a feature of ACT or NC CAH until proven otherwise.
Unité d'Endocrinologie-Gynécologie Pédiatrique, Service de Pédiatrie 1, Hôpital Arnaud-de-Villeneuve, and Departement d'Hormonologie du Développement et de la Reproduction, Hôpital Lapeyronie, CHU Montpellier, et Université Montpellier 1, France.
Françoise
F
Paris
Nicolas
N
Kalfa
Pascal
P
Philibert
Claire
C
Jeandel
Laura
L
Gaspari
Charles
C
Sultan
D000306:Adrenal Cortex Neoplasms / Q000175:diagnosis*; D000312:Adrenal Hyperplasia, Congenital / Q000175:diagnosis; D050499:Adrenarche / Q000502:physiology; D018268:Adrenocortical Carcinoma / Q000175:diagnosis*; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D011627:Puberty / Q000502:physiology*; D011629:Puberty, Precocious / Q000175:diagnosis*