DOI: http://dx.doi.org/10.18203/2349-3291.ijcp20200126

Functioning adrenocortical carcinoma causing virilisation: a case report

Vivek Parameswara Sarma, Sunil S. Menon

Abstract


Adrenocortical carticnoma (ACC) is an uncommon tumor with an incidence of 1-2 cases/million/year. It has two peak incidences; the first one in the first decade and the second one in the fourth decade. Most patients present with features of steroid hormone excess or abdominal mass effects, but about 15% of ACC are diagnosed incidentally. It is hormonally functional in 80 - 100% patients and the predisposing lesions include congenital adrenal hyperplasia and adenoma. ACC has significant syndromic and genetic association. Surgery offers the best chance of cure, especially in localized disease. Here, we present the case of virilization in a young female child secondary to a functioning ACC. The child had classical hormonal and imaging features of functioning ACC and underwent resection of the tumor (Adrenalectomy) with good outcome.


Keywords


Adrenal adenoma, Adrenal tumor, Adrenalectomy, Adrenocortical carcinoma, Clitoromegaly virilisation

Full Text:

PDF

References


Sutter JA, Grimberg A. Adrenocortical tumors and hyperplasias in childhood-etiology, genetics, clinical presentation and therapy. Pediatr endocrinol reviews: PER. 2006;4(1):32.

Ng L, Libertino JM. Adrenocortical carcinoma: diagnosis, evaluation and treatment. J Urol. 2003;169(1):5-11.

Aubert S, Wacrenier A, Leroy X, Devos P, Carnaille B, Proye C, et al. Weiss system revisited: a clinicopathologic and immunohistochemical study of 49 adrenocortical tumors. Am J Surg Pathol. 2002;26(12):1612-9.

Teinturier C, Pauchard MS, Brugieres L, Landais P, Chaussain JL, Bougneres PF. Clinical and prognostic aspects of adrenocortical neoplasms in childhood. Med Pediatr Oncol: Official J SIOP-Inter Soc Pediatr Oncol. 1999;32(2):106-11.

Schteingart DE, Doherty GM, Gauger PG, Giordano TJ, Hammer GD, Korobkin M, et al. Management of patients with adrenal cancer: recommendations of an international consensus conference. Endocrine-Related Cancer. 2005;12(3):667-80.

Ribeiro J, Ribeiro RC, Fletcher BD. Imaging findings in pediatric adrenocortical carcinoma. Pediatr Radiol. 2000;30(1):45-51.

Tacon LJ, Prichard RS, Soon PS, Robinson BG, Clifton-Bligh RJ, Sidhu SB. Current and emerging therapies for advanced adrenocortical carcinoma. Oncol. 2011;16(1):36-48.