, Volume 11, Issue 3, pp 356–360 | Cite as

Very premature pubarche in girls is not a pubertal variant

  • Françoise Paris
  • Nicolas Kalfa
  • Pascal Philibert
  • Claire Jeandel
  • Laura Gaspari
  • Charles SultanEmail author
Case report


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 hyper-plasia (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.

Key words

Adrenarche Adrenortical carcinoma Puberty 


  1. 1.
    Idkowiak J, Lavery GG, Dhir V, et al, 2011 Premature adrenarche: novel lessons from early onset androgen excess. Eur J Endocrinol 165: 189–207.CrossRefGoogle Scholar
  2. 2.
    Dacou-Voutetakis C, Dracopoulou M, 1999 High incidence of molecular defects of the CYP21 gene in patients with premature adrenarche. J Clin Endocrinol Metab 84: 1570–1574.CrossRefGoogle Scholar
  3. 3.
    Witchel SF, Smith R, Tomboc M, Aston CE, 2001 Candidate gene analysis in premature pubarche and adolescent hyperandrogenism. Fertil Steril 75: 724–730.CrossRefGoogle Scholar
  4. 4.
    Ibanez L, Virdis R, Potau N, et al, 1992 Natural history of premature pubarche: an auxological study. J Clin Endocrinol Metab 74: 254–257.PubMedGoogle Scholar
  5. 5.
    Oberfield SE, Sopher AB, Gerken AT, 2011 Approach to the girl with early onset of pubic hair. J Clin Endocrinol Metab 96: 1610–1622.CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Saenger P, Reiter EO, 1992 Premature adrenarche: a normal variant of puberty? J Clin Endocrinol Metab 74: 236–238.CrossRefGoogle Scholar
  7. 7.
    Counts DR, Pescovitz OH, Barnes KM, et al, 1987 Dissociation of adrenarche and gonadarche in precocious puberty and in isolated hypogonadotropic hypogonadism. J Clin Endocrinol Metab 64: 1174–1178.CrossRefGoogle Scholar
  8. 8.
    Zhang LH, Rodriguez H, Ohno S, Miller WL, 1995 Serine phosphorylation of human P450c17 increases 17,20-lyase activity: implications for adrenarche and the polycystic ovary syndrome. Proc Natl Acad Sci U S A 92: 10619–10623.CrossRefPubMedPubMedCentralGoogle Scholar
  9. 9.
    Palmert MR, Hayden DL, Mansfield MJ, et al, 2001 The longitudinal study of adrenal maturation during gonadal suppression: evidence that adrenarche is a gradual process. J Clin Endocrinol Metab 86: 4536–4542.CrossRefGoogle Scholar
  10. 10.
    Jabbar M, Pugliese M, Fort P, Recker B, Lifshitz F, 1991 Excess weight and precocious pubarche in children: alterations of the adrenocortical hormones. J Am Coll Nutr 10: 289–296.CrossRefGoogle Scholar
  11. 11.
    Rosenfield RL, 1996 Evidence that idiopathic functional adrenal hyperandrogenism is caused by dysregulation of adrenal steroidogenesis and that hyperinsulinemia may be involved. J Clin Endocrinol Metab 81: 878–880.PubMedGoogle Scholar
  12. 12.
    Cordera F, Grant C, van Heerden J, Thompson G, Young W, 2003 Androgen-secreting adrenal tumors. Surgery 134: 874–880.CrossRefGoogle Scholar
  13. 13.
    Icard P, Goudet P, Charpenay C, et al, 2001 Adrenocortical carcinomas: surgical trends and results of a 253-patient series from the French Association of Endocrine Surgeons study group. World J Surg 25: 891–897.CrossRefGoogle Scholar
  14. 14.
    Macfarlane DA, 1958 Cancer of the adrenal cortex; the natural history, prognosis and treatment in a study of fifty-five cases. Ann R Coll Surg Engl 23: 155–186.PubMedPubMedCentralGoogle Scholar
  15. 15.
    Vassilopoulou-Sellin R, Schultz PN, 2001 Adrenocortical carcinom. Clinical outcome at the end of the 20th century. Cancer 92: 1113–1121.PubMedGoogle Scholar
  16. 16.
    Street ME, Weber A, Camacho-Hubner C, et al, 1997 Girls with virilisation in childhood: a diagnostic protocol for investigation. J Clin Pathol 50: 379–383.CrossRefPubMedPubMedCentralGoogle Scholar
  17. 17.
    Allolio B, Hahner S, Weismann D, Fassnacht M, 2004 Management of adrenocortical carcinoma. Clin Endocrinol (Oxf) 60: 273–287.CrossRefGoogle Scholar
  18. 18.
    Voutilainen R, Perheentupa J, Apter D, 1983 Benign premature adrenarche: clinical features and serum steroid levels. Acta Paediatr Scand 72: 707–711.CrossRefGoogle Scholar
  19. 19.
    Halmi KA, Lascari AD, 1971 Conversion of virilization to feminization in a young girl with adrenal cortical carcinoma. Cancer 27: 931–935.CrossRefGoogle Scholar
  20. 20.
    Bonfig W, Bittmann I, Bechtold S, et al, 2003 Virilising adrenocortical tumours in children. Eur J Pediatr 162: 623–628.CrossRefGoogle Scholar
  21. 21.
    Wolthers OD, Cameron FJ, Scheimberg I, et al, 1999 Androgen secreting adrenocortical tumours. Arch Dis Child 80: 46–50.CrossRefPubMedPubMedCentralGoogle Scholar
  22. 22.
    Paris F, Tardy V, Chalancon A, Picot MC, Morel Y, Sultan C, 2010 Premature pubarche in Mediterranean girls: high prevalence of heterozygous CYP21 mutation carriers. Gynecol Endocrinol 26: 319–324.CrossRefGoogle Scholar

Copyright information

© Hellenic Endocrine Society 2012

Authors and Affiliations

  • Françoise Paris
    • 1
  • Nicolas Kalfa
    • 1
    • 2
    • 3
  • Pascal Philibert
    • 1
    • 2
  • Claire Jeandel
    • 1
    • 2
  • Laura Gaspari
    • 1
    • 2
  • Charles Sultan
    • 1
    • 2
    Email author
  1. 1.Unité d’Endocrinologie-Gynécologie Pédiatrique, Service de Pédiatrie 1Hôpital Arnaud-de-Villeneuve, CHU MontpellierMontpellierFrance
  2. 2.Departement d’Hormonologie du Développement et de la Reproduction, Hôpital LapeyronieCHU Montpellier, et Université Montpellier 1France
  3. 3.Service de Chirurgie Viscérale et Urologie Pédiatrique, Hôpital LapeyronieCHU Montpellier et Université Montpellier 1France

Personalised recommendations