Adrenalectomy for non-neuroblastic pathology in children

  • Michael D. TraynorJr.
  • Alaa Sada
  • Geoffrey B. Thompson
  • Christopher R. Moir
  • Irina Bancos
  • David R. Farley
  • Benzon M. Dy
  • Melanie L. Lyden
  • Elizabeth B. Habermann
  • Travis J. McKenzieEmail author
Original Article



Adrenalectomy for non-neuroblastic pathologies in children is rare with limited data on outcomes. We reviewed our experience of adrenalectomy in this unique population.


Retrospective study of children (age ≤ 18) who underwent adrenalectomy with non-neuroblastic pathology from 1988 to 2018. Clinical and operative details of patients were abstracted. Outcomes included length of stay and 30-day postoperative morbidity.


Forty children underwent 50 adrenalectomies (12 right-sided, 18 left-sided, 10 bilateral). Six patients (15%) presented with an incidental adrenal mass while 4 (10%) had masses found on screening for genetic mutations or prior malignancy. The remaining 30 (75%) presented with symptoms of hormonal excess. Nineteen patients (48%) underwent genetic evaluation and 15 (38%) had genetic predispositions. Diagnoses included 9 patients (23%) with pheochromocytoma, 8 (20%) with adrenocortical adenoma, 8 (20%) with adrenocortical carcinoma, 7 (18%) with adrenal hyperplasia, 2 (5%) with metastasis, and 6 (14%) with additional benign pathologies. Of 50 adrenalectomies, twenty-five (50%) were laparoscopic. Median hospital length of stay was 3 days (range 0–11). Post-operative morbidity rate was 17% with the most severe complication being Clavien–Dindo grade II.


Adrenalectomy for non-neuroblastic pathology can be done with low morbidity. Its frequent association with genetic mutations and syndromes requires surgeons to have knowledge of appropriate pre-operative testing and post-operative surveillance.


Pediatric adrenalectomy Laparoscopic adrenalectomy Adrenocortical carcinoma Pheochromocytoma Endocrine Primary pigmented nodular adrenocortical disease (PPNAD) 



No funding was necessary for conduct of this study.

Compliance with ethical standards

Conflict of interest

All authors declare no conflicts of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. The study was approved by the Mayo Clinic IRB.


  1. 1.
    Wieneke JA, Thompson LDR, Heffess CS (2003) Adrenal cortical neoplasms in the pediatric population: a clinicopathologic and immunophenotypic analysis of 83 patients. Am J Surg Pathol 27:867–881CrossRefGoogle Scholar
  2. 2.
    Edmonds S, Fein DM, Gurtman A (2011) Pheochromocytoma. Pediatr Rev 32:308–310. CrossRefPubMedGoogle Scholar
  3. 3.
    Michalkiewicz E, Sandrini R, Figueiredo B et al (2004) Clinical and outcome characteristics of children with adrenocortical tumors: a report from the International Pediatric Adrenocortical Tumor Registry. J Clin Oncol 22:838–845. CrossRefPubMedGoogle Scholar
  4. 4.
    Pham TH, Moir C, Thompson GB et al (2006) Pheochromocytoma and paraganglioma in children: a review of medical and surgical management at a tertiary care center. Pediatrics 118:1109–1117. CrossRefPubMedGoogle Scholar
  5. 5.
    Kundel A, Thompson GB, Richards ML et al (2014) Pediatric endocrine surgery: a 20-year experience at the Mayo Clinic. J Clin Endocrinol Metab 99:399–406. CrossRefPubMedGoogle Scholar
  6. 6.
    Ribeiro J, Ribeiro RC, Fletcher BD (2000) Imaging findings in pediatric adrenocortical carcinoma. Pediatr Radiol 30:45–51. CrossRefPubMedGoogle Scholar
  7. 7.
    Dokumcu Z, Divarci E, Ertan Y, Celik A (2018) Laparoscopic adrenalectomy in children: a 25-case series and review of the literature. J Pediatr Surg 53:1800–1805. CrossRefPubMedGoogle Scholar
  8. 8.
    Kadamba P, Habib Z, Rossi L (2004) Experience with laparoscopic adrenalectomy in children. J Pediatr Surg 39:764–767. CrossRefPubMedGoogle Scholar
  9. 9.
    St Peter SD, Valusek PA, Hill S et al (2011) Laparoscopic adrenalectomy in children: a multicenter experience. J Laparoendosc Adv Surg Tech A 21:647–649. CrossRefPubMedGoogle Scholar
  10. 10.
    Fascetti F, Scotton G, Pio L et al (2017) Minimally invasive resection of adrenal masses in infants and children: results of a European multi-center survey. Surg Endosc 31:4505–4512. CrossRefGoogle Scholar
  11. 11.
    Lenders JWM, Duh Q-Y, Eisenhofer G et al (2014) Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab 99:1915–1942. CrossRefPubMedGoogle Scholar
  12. 12.
    Babic B, Patel D, Aufforth R et al (2017) Pediatric patients with pheochromocytoma and paraganglioma should have routine preoperative genetic testing for common susceptibility genes in addition to imaging to detect extra-adrenal and metastatic tumors. Surgery 161:220–227. CrossRefPubMedGoogle Scholar
  13. 13.
    Teinturier C, Pauchard MS, Brugières L et al (1999) Clinical and prognostic aspects of adrenocortical neoplasms in childhood. Med Pediatr Oncol 32:106–111CrossRefGoogle Scholar
  14. 14.
    Else T (2012) Association of adrenocortical carcinoma with familial cancer susceptibility syndromes. Mol Cell Endocrinol 351:66–70. CrossRefPubMedGoogle Scholar
  15. 15.
    Stratakis CA (2008) Cushing syndrome caused by adrenocortical tumors and hyperplasias (corticotrophin-independent Cushing syndrome). Endocr Dev 13:117–132. CrossRefPubMedPubMedCentralGoogle Scholar
  16. 16.
    Mayo-Smith WW, Boland GW, Noto RB, Lee MJ (2001) State-of-the-art adrenal imaging. Radiographics 21:995–1012. CrossRefGoogle Scholar
  17. 17.
    McHugh K (2007) Renal and adrenal tumours in children. Cancer Imaging 7:41–51. CrossRefPubMedPubMedCentralGoogle Scholar
  18. 18.
    Stewart JN, Flageole H, Kavan P (2004) A surgical approach to adrenocortical tumors in children: the mainstay of treatment. J Pediatr Surg 39:759–763. CrossRefPubMedGoogle Scholar
  19. 19.
    Batista DL, Riar J, Keil M, Stratakis CA (2007) Diagnostic tests for children who are referred for the investigation of Cushing syndrome. Pediatrics 120:e575–586. CrossRefPubMedGoogle Scholar
  20. 20.
    Elsayes KM, Mukundan G, Narra VR et al (2004) Adrenal masses: MR imaging features with pathologic correlation. RadioGraphics 24:S73–S86. CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Michael D. TraynorJr.
    • 1
  • Alaa Sada
    • 1
  • Geoffrey B. Thompson
    • 1
  • Christopher R. Moir
    • 2
  • Irina Bancos
    • 3
  • David R. Farley
    • 1
  • Benzon M. Dy
    • 1
  • Melanie L. Lyden
    • 1
  • Elizabeth B. Habermann
    • 4
    • 5
  • Travis J. McKenzie
    • 1
    Email author
  1. 1.Department of SurgeryMayo ClinicRochesterUSA
  2. 2.Division of Pediatric SurgeryMayo ClinicRochesterUSA
  3. 3.Division of EndocrinologyMayo ClinicRochesterUSA
  4. 4.Division of Health Care Policy and ResearchMayo ClinicRochesterUSA
  5. 5.Robert D. and Patricia E. Kern Center for the Science of Health Care DeliveryMayo ClinicRochesterUSA

Personalised recommendations