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CC BY 4.0 · Indian J Med Paediatr Oncol
DOI: 10.1055/s-0044-1780522
Case Report with Review of Literature

Clinical Conundrum of ACTH-Independent Cushing's Syndrome in a Child with Adrenal Cortical Carcinoma: A Case Report and Review of Literature

Prashant Patil
1   Department of Paediatric Endocrinology, SRCC Children's Hospital, Mumbai, Maharashtra, India
,
Joewin Monteiro
1   Department of Paediatric Endocrinology, SRCC Children's Hospital, Mumbai, Maharashtra, India
,
Sajid Qureshi 
2   Department of Paediatric Surgical Oncology, SRCC Children's Hospital, Mumbai, Maharashtra, India
,
Nupur Karnik
3   Department of Pathology, Tata Memorial Hospital, Mumbai, Maharashtra, India
› Author Affiliations Source of Funding None.
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Abstract

Adrenocorticotropic hormone-independent Cushing's syndrome (CS) secondary to cortisol-secreting adrenal cortical carcinoma (ACC) in children has been seldom reported. We report on a 6-year-old girl diagnosed with CS due to a right-sided ACC. She presented with rapid onset obesity, virilization, and hypertensive urgency. Postserial diagnostic evaluation, control of hypertension, right adrenalectomy with en bloc resection of tumor mass were performed. The child had an excellent clinical and biochemical recovery with significant weight loss and return to normal serum cortisol levels.

Keywords

ACTH-independent Cushing's syndrome - adrenal cortical carcinoma - adrenalectomy - mitotane

Authors' Contributions

P.P. was involved in conceptualization, designing, literature search, data acquisition, manuscript preparation, manuscript editing, and manuscript review. J.M. helped in literature search, data acquisition, data analysis, manuscript preparation, and manuscript editing. S.Q. contributed to data acquisition and manuscript review. N.K. was involved in data acquisition.


Patient Consent

Informed and written consent taken from the patient through the declaration in the Patient Consent Form.




Publication History

Article published online:
16 April 2024

© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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  • References

  • 1 Newell-Price J, Bertagna X, Grossman AB, Nieman LK. Cushing's syndrome. Lancet 2006; 367 (9522) 1605-1617
    CrossrefPubMedGoogle Scholar
  • 2 Graversen D, Vestergaard P, Stochholm K, Gravholt CH, Jørgensen JO. Mortality in Cushing's syndrome: a systematic review and meta-analysis. Eur J Intern Med 2012; 23 (03) 278-282
    CrossrefPubMedGoogle Scholar
  • 3 Lacroix A, Bourdeau I. Bilateral adrenal Cushing's syndrome: macronodular adrenal hyperplasia and primary pigmented nodular adrenocortical disease. Endocrinol Metab Clin North Am 2005; 34 (02) 441-458 , x
    CrossrefPubMedGoogle Scholar
  • 4 Michalkiewicz E, Sandrini R, Figueiredo B. 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 (05) 838-845
    CrossrefPubMedGoogle Scholar
  • 5 Ng L, Libertino JM. Adrenocortical carcinoma: diagnosis, evaluation and treatment. J Urol 2003; 169 (01) 5-11
    CrossrefPubMedGoogle Scholar
  • 6 Nieman LK, Biller BMK, Findling JW. et al. The diagnosis of Cushing's syndrome: an endocrine society clinical practice guideline. J Clin Endocrinol Metab 2008; 93 (05) 1526-1540
    CrossrefPubMedGoogle Scholar
  • 7 Lam AK. Update on adrenal tumours in 2017 World Health Organization (WHO) of endocrine tumours. Endocr Pathol 2017; 28 (03) 213-227
    CrossrefPubMedGoogle Scholar
  • 8 Sutter JA, Grimberg A. Adrenocortical tumors and hyperplasias in childhood–etiology, genetics, clinical presentation and therapy. Pediatr Endocrinol Rev 2006; 4 (01) 32-39
    PubMedGoogle Scholar
  • 9 Patalano A, Brancato V, Mantero F. Adrenocortical cancer treatment. Horm Res 2009; 71 (Suppl. 01) 99-104
    PubMedGoogle Scholar
  • 10 Almeida MQ, Soares IC, Ribeiro TC. et al. Steroidogenic factor 1 overexpression and gene amplification are more frequent in adrenocortical tumors from children than from adults. J Clin Endocrinol Metab 2010; 95 (03) 1458-1462
    CrossrefPubMedGoogle Scholar
  • 11 Tissier F, Cavard C, Groussin L. et al. Mutations of beta-catenin in adrenocortical tumors: activation of the Wnt signaling pathway is a frequent event in both benign and malignant adrenocortical tumors. Cancer Res 2005; 65 (17) 7622-7627
    CrossrefPubMedGoogle Scholar
  • 12 Ribeiro RC, Sandrini F, Figueiredo B. et al. An inherited p53 mutation that contributes in a tissue-specific manner to pediatric adrenal cortical carcinoma. Proc Natl Acad Sci U S A 2001; 98 (16) 9330-9335
    CrossrefPubMedGoogle Scholar
  • 13 Lefevre M, Gerard-Marchant R, Gubler JP, Chaussain JL, Lemerle J. Adrenocortical carcinoma in children: 42 patients treated from 1958 to 1980 at Villejuif. In: Humphrey GB, Grindey GB, Dehner LP, Acton RT, Pysher TJ. eds. Adrenal and Endocrine Tumors in Children. New York, NY: Springer; 1983: 265-276
    Google Scholar
  • 14 Coulter CL. Fetal adrenal development: insight gained from adrenal tumors. Trends Endocrinol Metab 2005; 16 (05) 235-242
    CrossrefPubMedGoogle Scholar
  • 15 Libé R. Adrenocortical carcinoma (ACC): diagnosis, prognosis, and treatment. Front Cell Dev Biol 2015; 3: 45
    CrossrefPubMedGoogle Scholar
  • 16 Young Jr WF. Clinical practice. The incidentally discovered adrenal mass. N Engl J Med 2007; 356 (06) 601-610
    CrossrefPubMedGoogle Scholar
  • 17 Ribeiro J, Ribeiro RC, Fletcher BD. Imaging findings in pediatric adrenocortical carcinoma. Pediatr Radiol 2000; 30 (01) 45-51
    CrossrefPubMedGoogle Scholar
  • 18 Pacak K, Eisenhofer G, Goldstein DS. Functional imaging of endocrine tumors: role of positron emission tomography. Endocr Rev 2004; 25 (04) 568-580
    CrossrefPubMedGoogle Scholar
  • 19 Maurea S, Klain M, Mainolfi C, Ziviello M, Salvatore M. The diagnostic role of radionuclide imaging in evaluation of patients with nonhypersecreting adrenal masses. J Nucl Med 2001; 42 (06) 884-892
    PubMedGoogle Scholar
  • 20 Mackie GC, Shulkin BL, Ribeiro RC. et al. Use of [18F]fluorodeoxyglucose positron emission tomography in evaluating locally recurrent and metastatic adrenocortical carcinoma. J Clin Endocrinol Metab 2006; 91 (07) 2665-2671
    CrossrefPubMedGoogle Scholar
  • 21 Leboulleux S, Dromain C, Bonniaud G. et al. Diagnostic and prognostic value of 18-fluorodeoxyglucose positron emission tomography in adrenocortical carcinoma: a prospective comparison with computed tomography. J Clin Endocrinol Metab 2006; 91 (03) 920-925
    CrossrefPubMedGoogle Scholar
  • 22 Schteingart DE, Doherty GM, Gauger PG. et al. Management of patients with adrenal cancer: recommendations of an international consensus conference. Endocr Relat Cancer 2005; 12 (03) 667-680
    CrossrefPubMedGoogle Scholar
  • 23 Aubert S, Wacrenier A, Leroy X. et al. Weiss system revisited: a clinicopathologic and immunohistochemical study of 49 adrenocortical tumors. Am J Surg Pathol 2002; 26 (12) 1612-1619
    CrossrefPubMedGoogle Scholar
  • 24 Li J, Zhang W, Hu H, Zhang Y, Wen Y, Huang D. Adrenocortical carcinoma in eight children: a report and literature review. Cancer Manag Res 2021; 13: 1307-1314
    CrossrefPubMedGoogle Scholar
  • 25 Martins-Filho SN, Almeida MQ, Soares I. et al. Clinical impact of pathological features including the Ki-67 labeling index on diagnosis and prognosis of adult and pediatric adrenocortical tumors. Endocr Pathol 2021; 32 (02) 288-300
    CrossrefPubMedGoogle Scholar
  • 26 Teinturier C, Pauchard MS, Brugières L, Landais P, Chaussain JL, Bougnères PF. Clinical and prognostic aspects of adrenocortical neoplasms in childhood. Med Pediatr Oncol 1999; 32 (02) 106-111
    CrossrefPubMedGoogle Scholar
  • 27 Ardicli B, User IR, Ciftci AO. et al. Adrenocortical tumours in children: a review of surgical management at a tertiary care centre. ANZ J Surg 2021; 91 (05) 992-999
    CrossrefPubMedGoogle Scholar
  • 28 Cecchetto G, Ganarin A, Bien E. et al. Outcome and prognostic factors in high-risk childhood adrenocortical carcinomas: a report from the European Cooperative Study Group on Pediatric Rare Tumors (EXPeRT). Pediatr Blood Cancer 2017; 64 (06) 64
    CrossrefPubMedGoogle Scholar
  • 29 Gulack BC, Rialon KL, Englum BR. et al. Factors associated with survival in pediatric adrenocortical carcinoma: an analysis of the National Cancer Data Base (NCDB). J Pediatr Surg 2016; 51 (01) 172-177
    CrossrefPubMedGoogle Scholar
  • 30 Terzolo M, Angeli A, Fassnacht M. et al. Adjuvant mitotane treatment for adrenocortical carcinoma. N Engl J Med 2007; 356 (23) 2372-2380
    CrossrefPubMedGoogle Scholar
  • 31 Redlich A, Boxberger N, Strugala D. et al. Systemic treatment of adrenocortical carcinoma in children: data from the German GPOH-MET 97 trial. Klin Padiatr 2012; 224 (06) 366-371
    Article in Thieme ConnectPubMedGoogle Scholar
  • 32 Virgone C, Roganovic J, Vorwerk P. et al. Adrenocortical tumours in children and adolescents: the EXPeRT/PARTNER diagnostic and therapeutic recommendations. Pediatr Blood Cancer 2021; 68 (Suppl. 04) e29025
    CrossrefPubMedGoogle Scholar
  • 33 Rodriguez-Galindo C, Krailo MD, Pinto EM. et al. Treatment of pediatric adrenocortical carcinoma with surgery, retroperitoneal lymph node dissection, and chemotherapy: the Children's Oncology Group ARAR0332 protocol. J Clin Oncol 2021; 39 (22) 2463-2473
    CrossrefPubMedGoogle Scholar
  • 34 Zancanella P, Pianovski MA, Oliveira BH. et al. Mitotane associated with cisplatin, etoposide, and doxorubicin in advanced childhood adrenocortical carcinoma: mitotane monitoring and tumor regression. J Pediatr Hematol Oncol 2006; 28 (08) 513-524
    CrossrefPubMedGoogle Scholar
  • 35 Tacon LJ, Prichard RS, Soon PS, Robinson BG, Clifton-Bligh RJ, Sidhu SB. Current and emerging therapies for advanced adrenocortical carcinoma. Oncologist 2011; 16 (01) 36-48
    CrossrefPubMedGoogle Scholar