Adrenal Tumors in Children: Spectrum of Presentation and Surgical Approach in a Tertiary Care Institute

Abstract

Context: Adrenal tumors can arise either from cortex or from medulla; both the regions being structurally and functionally different. Current knowledge on childhood adrenocortical tumors (ACTs), the management approach, and the outcome is limited due to limited number of cases and studies. Neuroblastoma is a neoplasm of the neural crest origin and 50% arise from adrenal medulla. Pheochromocytoma (PCC) is an exciting and rare neoplasm in childhood. Aims: To evaluate the clinical presentation, spectrum of pathology, and management of adrenal tumors in children. Subjects and Methods: The study was conducted in a tertiary care pediatric institute over 5 years on children with intra-abdominal solid tumors. Adrenal tumors were diagnosed on radiological studies and postoperatively confirmed by histopathology. Results: Sixty-three patients were operated for intra-abdominal solid tumors, and Wilms tumor (39) was the most common finding. Of 11 cases of adrenal tumors confirmed by histopathology, eight were adrenal neuroblastoma, one 14-month-old female baby was detected as PCC, while two female children presented with the features of Cushing syndrome and virilization at the age of 3 and 7 years. Postoperatively, one of them was found to be adrenal adenoma and one was found to be adrenocortical carcinoma. Conclusions: ACTs are rare and potentially fatal in childhood. Complete surgical resection is the mainstay of therapy in adrenal tumors and is curative for associated hypertension. Neuroblastoma was the third common intra-abdominal solid tumor after Wilms tumor and retroperitoneal teratoma. The patient with PCC contributed to “Rule of 10s” because of the absence of hypertension and childhood occurrence.

Keywords

Adrenal adenoma - adrenocortical carcinoma - neuroblastoma - pheochromocytoma

Publication History

Received: 12 August 2018

Accepted: 06 February 2020

Article published online:
28 June 2021

• References

• 1 Stewart JN, Flageole H, Kavan P. A surgical approach to adrenocortical tumors in children: The mainstay of treatment. J Pediatr Surg 2004; 39: 759-63
• 2 Mahendraraj K, Lau CS, Sidhu K, Chamberlain RS. Adrenocortical carcinoma in adults and children: A population-based outcomes study involving 1,623 patients from the surveillance, epidemiology, and end result (SEER) database (1973-2012). Clin Surg 2016; 1: 1017-7
• 3 Waguespack SG, Rich T, Grubbs E, Ying AK, Perrier ND, Ayala-Ramirez M. et al. A current review of the etiology, diagnosis, and treatment of pediatric pheochromocytoma and paraganglioma. J Clin Endocrinol Metab 2010; 95: 2023-37
• 4 Armstrong R, Sridhar M, Greenhalgh KL, Howell L, Jones C, Landes C. et al. Phaeochromocytoma in children. Arch Dis Child 2008; 93: 899-904
• 5 Tripathy PK, Pattnaik K, Nayak M, Mohanty HK. Pheochromocytoma in a child without hypertension: A contribution to the “Rule of 10s”. Indian J Med Paediatr Oncol 2017; 38: 59-61
• 6 Kushner BH. Neuroblastoma: A disease requiring a multitude of imaging studies. J Nucl Med 2004; 45: 1172-88
• 7 McHugh K. Renal and adrenal tumours in children. Cancer Imaging 2007; 7: 41-51
• 8 Hiorns MP, Owens CM. Radiology of neuroblastoma in children. Eur Radiol 2001; 11: 2071-81
• 9 Bittman ME, Lee EY, Restrepo R, Eisenberg RL. Focal adrenal lesions in pediatric patients. AJR Am J Roentgenol 2013; 200: W542-56
• 10 Rich BS, Quaglia MP. Neuroblastoma. In: Coran AG, Adzick NS, Krummel TM, Laberge J, Shamberger RC. editors Pediatric Surgery. Philadelphia: Elsevier Saunders; 2012. 7. 441-58
• 11 Chudler RM, Kay R. Adrenocortical carcinoma in children. Urol Clin North Am 1989; 16: 469-79
• 12 Ribeiro RC, Sandrini Neto RS, Schell MJ, Lacerda L, Sambaio GA, Cat I. Adrenocortical carcinoma in children: A study of 40 cases. J Clin Oncol 1990; 8: 67-74
• 13 Ribeiro RC, Michalkiewicz EL, Figueiredo BC, DeLacerda L, Sandrini F, Pianovsky MD. et al. Adrenocortical tumors in children. Braz J Med Biol Res 2000; 33: 1225-34
• 14 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
• 15 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: 838-45
• 16 Kerkhofs TM, Ettaieb MH, Verhoeven RH, Kaspers GJ, Tissing WJ, Loeffen J. et al. Adrenocortical carcinoma in children:First population-based clinicopathological study with long-term follow-up. Oncol Rep 2014; 32: 2836-44
• 17 Liou LS, Kay R. Adrenocortical carcinoma in children. Review and recent innovations. Urol Clin North Am 2000; 27: 403-21
• 18 Ribeiro RC, Fighueiredo B. Childhood adrenocortical tumors. Eur J Cancer 2004; 40: 1117-26
• 19 Gundgurthi A, Kharb S, Dutta MK, Garg MK, Khare A, Jacob MJ. et al. Childhood adrenocortical carcinoma: Case report and review. Indian J Endocrinol Metab 2012; 16: 413-5
• 20 Diesen DL, Skinner MA. et al. Endocrine disorders and tumors. In: Holcomb GW 3rd, Murphy JP, Ostlie DJ, editors. Ashcraft's Pediatric Surgery. Philadelphia: Elsevier Saunders 2014; 6: 1067-85
• 21 Wanis KN, Kanthan R. Diagnostic and prognostic features in adrenocortical carcinoma: A single institution case series and review of the literature. World J Surg Oncol 2015; 13: 117
• 22 Lau SK, Weiss LM. The Weiss system for evaluating adrenocortical neoplasms: 25 years later. Hum Pathol 2009; 40: 757-68
• 23 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: 1612-9
• 24 Cho MJ, Kim DY, Kim SC, Kim TH, Kim IK. Adrenocortical tumors in children 18 years old and younger. J Korean Surg Soc 2012; 82: 246-50
• 25 Ciftci AO, Senocak ME, Tanyel FC, Büyükpamukçu N. Adrenocortical tumors in children. J Pediatr Surg 2001; 36: 549-54
• 26 Libé R. Adrenocortical carcinoma (ACC): Diagnosis, prognosis, and treatment. Front Cell Dev Biol 2015; 3: 45
• 27 Beuschlein F, Weigel J, Saeger W, Kroiss M, Wild V, Daffara F. et al. Major prognostic role of Ki67 in localized adrenocortical carcinoma after complete resection. J Clin Endocrinol Metab 2015; 100: 841-9
• 28 Eren E, Saglam H, Caliskan Y, Kiristioglu I, Tarim O, Sarin YK. Pediatric patients with pheochromocytoma: Experience of a tertiary health center. Pediatr Int 2015; 57: 875-9
• 29 Mishra A, Mehrotra PK, Agarwal G, Agarwal A, Mishra SK. Pediatric and adolescent pheochromocytoma: Clinical presentation and outcome of surgery. Indian Pediatr 2014; 51: 299-302