PAEDIATRIC ADRENOCORTICAL CARCINOMA: A CASE REPORT
Main Article Content
Adrenocortical carcinoma (ACC) is a relatively rare but an aggressive neoplasm that can have variety of manifestations. It can manifest in the form of virilization, hyperaldosteronism, cushingoid features or combination of that. Due to rapid appearance of signs and symptoms in children, these tumours can be diagnosed early however if delayed can bear worse prognosis as they tend to metastasize early in course of disease progression. We report a case of 4 years old female patient from Chitral, Pakistan who was referred to Endocrinology Department from Paediatric Department of Hayatabad Medical Complex, Peshawar Patient presented at paediatric OPD with complaints of weight gain, hirsutism, rashes over the face and acne. Patient had typical moon facies, buffalo hump and distended abdomen without striae as well as mass in the left hypochondrium on examination. On further evaluation clitoromegaly and pubic hair tanner stage 4 were found. Both Ultrasound and CT-Scan showed left supra-renal soft tissue mass invading the inferior venacava (IVC). She was further evaluated and her overnight dexamethasone suppression test (ODST) and serum testosterone levels were high although serum sodium and potassium were normal ruling out hyperaldosteronism. Next, biopsy was performed which was consistent with adrenocortical carcinoma. ACC although rare is highly aggressive tumor that requires early detection and diagnosis specially in children. If diagnosis is delayed prognosis may not be favourable. It therefore requires avid clinical suspicion to proceed onto diagnostic lines for patient’s benefit as well as physician’s satisfaction.
Work published in JPMI is licensed under a
Creative Commons Attribution-NonCommercial 2.0 Generic License.
Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work.
Bellantone R, Ferrante A, Boscherini M, Lombardi CP, Crucitti P, Crucitti F, et al. Role of reoperation in recurrence of adrenal cortical carcinoma: results from 188 cases collected in the Italian National Registry for Adrenal Cortical Carcinoma. Surgery. 1997;122(6):1212-8. DOI:10.1016/s0039-6060(97)90229- 4.
Ng L, Libertino JM. Adrenocortical carcinoma: diagnosis, evaluation and treatment. J Urol. 2003;169(1):5-11. DOI:10.1097/01. ju.0000030148.59051.35..
Michalkiewicz E, Sandrini R, Figueiredo B, Miranda EC, Caran E, Oliveira-Filho AG, 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(5):838-45. DOI:10.1200/ JCO.2004.08.085.
Pommier RF, Brennan MF. An eleven-year experience with adrenocortical carcinoma. Surgery. 1992;112(6):963- 70; discussion 970-1.
Zachariah C, Adity S, Helina R. Farrago of Pediatric Solid Malignancies from a Tertiary Care Centre in Northeast India: A Retrospective Analysis. Int J Sci Study. 2021;8(10):101-7.
Sutter JA, Grimberg A. Adrenocortical tumors and hyperplasias in childhood--etiology, genetics, clinical presentation and therapy. Pediatr Endocrinol Rev. 2006;4(1):32-9.
Crucitti F, Bellantone R, Ferrante A, Boscherini M, Crucitti P. The Italian Registry for Adrenal Cortical Carcinoma: analysis of a multiinstitutional series of 129 patients. The ACC Italian Registry Study Group. Surgery. 1996;119(2):161-70. DOI:10.1016/ s0039-6060(96)80164-4.
Rahar S, Chauhan DS, Singh S, Ahuja A, Bhardwaj M, Sen A. Adrenocortical Carcinoma in a 6-Month-Old Infant. J Indian Assoc Pediatr Surg. 2020;25(5):310-313. DOI: 10.4103/ jiaps.JIAPS_155_19. .
Icard P, Goudet P, Charpenay C, Andreassian B, Carnaille B, Chapuis Y, et al. Adrenocortical carcinomas: surgical trends and results of a 253-patient series from the French Association of Endocrine Surgeons study group. World J Surg. 2001;25(7):891-7. DOI:10.1007/ s00268-001-0047-y.
Becherer A, Vierhapper H, Potzi C, Karanikas G, Kurtaran A, Schmaljohann J, et al. Cancer Biother Ra-diopharm. 2001;16(4):289-95. DOI:10.1089/108497801753131363.
Young WF Jr. Clinical practice. The incidentally discovered adrenal mass. N Engl J Med. 2007;356(6):601-10. DOI:10.1056/NEJMcp065470.
Teinturier C, Pauchard MS, Brugieres L, Landais P, Chaussain JL, Bougneres PF. Clinical and prognostic aspects of adrenocortical neoplasms in childhood. Med Pediatr Oncol. 1999 Feb;32(2):106- 11. DOI:10.1002/(sici)1096- 911x(199902)32:2<106::aid-mpo7>3.0.co;2-j.
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. Endocr Relat Cancer. 2005;12(3):667- 80. DOI:10.1677/erc.1.01029.