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Licensed Unlicensed Requires Authentication Published by De Gruyter January 14, 2016

Pubertal outcome in a female with virilizing adrenocortical carcinoma

  • Emily Breidbart , Tamara Cameo , James H. Garvin , Hanina Hibshoosh and Sharon E. Oberfield EMAIL logo


Adrenocortical tumors are neoplasms that rarely occur in pediatric patients. Adrenocortical carcinoma (ACC) is even more uncommon, and is an aggressive malignancy with 5-year survival of 55% in a registry series. There is a lack of information on long-term endocrine outcome in survivors. We describe a 10-year follow-up in a patient who presented at 3 years 5 months with a 1-year history of axillary odor and 6 months’ history of pubic hair development with an increased clitoral size. Androgen levels were increased and a pelvic sonogram revealed a suprarenal mass of the left kidney. The tumor was successfully removed. At 6 years 11 months, androgen levels increased again. Workup for tumor recurrence was negative and the findings likely represented early adrenarche. The patient had menarche at an appropriate time and attained a height appropriate for her family.

Corresponding author: Sharon E. Oberfield, MD, Division of Pediatric Endocrinology, Diabetes and Metabolism, New York Presbyterian Hospital-Columbia University Medical Center, 622 West 168th Street PH 5E 522, New York, NY 10032, USA, E-mail:


1. Ribeiro RC, Figueiredo B. Childhood adrenocortical tumours. Eur J Cancer 2004;40:1117–26.10.1016/j.ejca.2004.01.031Search in Google Scholar

2. Mcateer JP, Huaco JA, Gow KW. Predictors of survival in pediatric adrenocortical carcinoma: a surveillance, epidemiology, and end results (SEER) program study. J Pediatr Surg 2013;48:1025–31.10.1016/j.jpedsurg.2013.02.017Search in Google Scholar

3. Magro G, Vecchio GM, Alaggio, R. Diagnosis and prognosis of pediatric patients with adrenocortical carcinoma. In: Hayat M, editor. Pediatric cancer, volume 4, diagnosis, therapy, and prognosis. Dordrecht, NL: Springer Science & Business Media, 2013:245–52.10.1007/978-94-007-6591-7_24Search in Google Scholar

4. Mayer SK, Oligny LL, Deal C, Yazbeck S, Gagné N, et al. Childhood adrenocortical tumors: case series and reevaluation of prognosis-a 24-year experience. J Pediatr Surg 1997;32:911–5.10.1016/S0022-3468(97)90649-7Search in Google Scholar

5. Teinturier C, Pauchard MS, Brugières L, Landais P, Chaussain JL, et al. Clinical and prognostic aspects of adrenocortical neoplasms in childhood. Med Pediatr Oncol 1999;32:106–11.10.1002/(SICI)1096-911X(199902)32:2<106::AID-MPO7>3.0.CO;2-JSearch in Google Scholar

6. Michalkiewicz E, Sandrini R, Figueiredo B, Miranda EC, Caran E, et al. Clinical and outcome characteristics of children with adrenocortical tumors: a report from the International Pediatric Adrenocortical Tumor Registry. J Clin Oncol 2004;22:838–45.10.1200/JCO.2004.08.085Search in Google Scholar

7. Adrenal. In: Edge SB, Byrd DR, Compton CC, Fritz AG, Green, FL, et al., editors. AJCC cancer staging manual, 7th ed. New York, NY: Springer, 2010:515–20.10.1007/978-0-387-88441-7_47Search in Google Scholar

8. Ribeiro RC, Pinto EM, Zambetti GP, Rodriguez-Galindo C. The international pediatric adrenocortical tumor registry initiative: contributions to clinical, biological, and treatment advances in pediatric adrenocortical tumors. Mol Cell Endocrinol 2012;351:37–43.10.1016/j.mce.2011.10.015Search in Google Scholar

9. Hagemann K, Zanolari Calderari M, Perren A, Cree I, Mullis PE, et al. A typical presentation of a hormonally active adrenocortical tumor in an adolescent leading to delayed diagnosis. Hormones 2011;10:317–25.10.14310/horm.2002.1324Search in Google Scholar

10. Ng L, Libertino JM. Adrenocortical carcinoma: diagnosis, evaluation and treatment. J Urol 2003;169:5–11.10.1016/S0022-5347(05)64023-2Search in Google Scholar

11. Green AL, Srivatsa A, Rodriguez-Galindo C. Delayed diagnosis and false relapse due to paternal testosterone use in adrenocortical carcinoma. Pediatrics 2014;133:e1772–6.10.1542/peds.2013-1454Search in Google Scholar

12. Datrice NM, Langan RC, Ripley RT, Kemp CD, Steinberg SM. Operative management for recurrent and metastatic adrenocortical carcinoma. J Surg Oncol 2012;105:709–13.10.1002/jso.23015Search in Google Scholar

13. Garge S, Bawa M, Kanojia RP, Gupta K, Rao KL. A rare case of isolated Cushing syndrome in a 3-month-old boy. J Pediatr Endocrinol Metab 2013;26:599–603.10.1515/jpem.2011.343Search in Google Scholar

14. Liou LS, Kay R. Adrenocortical carcinoma in children. Review and recent innovations. Urol Clin North Am 2000;27:403–21.10.1016/S0094-0143(05)70089-6Search in Google Scholar

15. Hermsen IG, Gelderblom H, Kievit J, Romijn JA, Haak HR. Extremely long survival in six patients despite recurrent and metastatic adrenal carcinoma. Eur J Endocrinol 2008;158:911–9.10.1530/EJE-07-0723Search in Google Scholar PubMed

16. Children’s Oncology Group. ARAR0332: Treatment of adrenocortical tumors with surgery plus lymph node dissection and multiagent chemotherapy, A groupwise phase III study. Opened 9/18/2006.Search in Google Scholar

17. Wieneke JA, Thompson LD, Heffess CS. Adrenal cortical neoplasms in the pediatric population: a clinicopathologic and immunophenotypic analysis of 83 patients. Am J Surg Pathol 2003;27:867–81.10.1097/00000478-200307000-00001Search in Google Scholar PubMed

18. Berruti A, Baudin E, Gelderblom H, Haak HR, Porpiqlia F, et al. Adrenal cancer: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2012;23(Suppl 7):vii131.10.1093/annonc/mds231Search in Google Scholar PubMed

19. Libé R, Bertherat J. Molecular genetics of adrenocortical tumours, from familial to sporadic diseases. Eur J Endocrinol 2005;153:477–87.10.1530/eje.1.02004Search in Google Scholar PubMed

20. Gonzalez KD, Noltner KA, Buzin CH. Beyond Li Fraumeni syndrome: clinical characteristics of families with p53 germline mutations. J Clin Oncol 2009;27:1250–6.10.1200/JCO.2008.16.6959Search in Google Scholar PubMed

21. Trapp CM, Speiser PW, Oberfield SE. Congenital adrenal hyperplasia: an update in children. Curr Opin Endocrinol Diabetes Obes 2011;18:166–70.10.1097/MED.0b013e328346938cSearch in Google Scholar PubMed PubMed Central

Received: 2015-3-17
Accepted: 2015-11-30
Published Online: 2016-1-14
Published in Print: 2016-4-1

©2016 by De Gruyter

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