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Cushing syndrome secondary to CRH-producing Wilms tumor in a 6 year old

  • Moon Hee Lee , Uiju Cho , Jae-Wook Lee , Won-Kyoung Cho , Min Ho Jung , Nak Gyun Chung , Bin Cho , Yeong Jin Choi , Myung Duk Lee and Byung-Kyu Suh EMAIL logo
Published/Copyright: August 15, 2014

Abstract

Cushing syndrome is caused by prolonged exposure to elevated serum cortisol. It is uncommon in children, and etiology includes pituitary adenoma, adrenal tumor, and exogenous glucocorticoid administration. Rarely, it is paraneoplastic in origin. We present a case of paraneoplastic Cushing syndrome due to Wilms tumor that secreted corticotropin-releasing hormone (CRH). A 6-year-old male presented with polyphagia and weight gain. He showed Cushingoid appearance, hypertension, and palpable left flank mass. Serum cortisol and adrenocorticotropic hormone (ACTH) levels were elevated. Computed tomography showed a neoplasm originating from the left kidney. Pathologic diagnosis of Wilms tumor was made upon nephroureterectomy. Immunohistochemical staining was positive for CRH and negative for ACTH. All features of Cushing syndrome disappeared after surgery. This represents a rare case of Cushing syndrome secondary to Wilms tumor in which CRH production has been demonstrated.


Corresponding author: Byung-Kyu Suh, MD, Department of Pediatrics, Seoul St. Mary’s Hospital, 222 Banpo-daero, Seocho-gu, Seoul 137-701, Korea, Phone: +82-2-2258-6185, Fax: +82-2-532-6185, E-mail:
aUiju Cho is co-first author.

References

1. Feinstein KF. Renal neoplasms. In: Kuhn JP, Slovis TL, Haller JO, Caffey J, editors. Caffey’s pediatric diagnostic imaging, 10th ed. St Louis, MO: Mosby, 2004;1788.Search in Google Scholar

2. Ruteshouser EC, Robinson SM, Huff V. Wilms tumor genetics: mutations in WT1, WTX, and CTNNB1 account for only about one-third of tumors. Genes Chromosomes Cancer 2008;47: 461–70.10.1002/gcc.20553Search in Google Scholar

3. Scott RH, Douglas J, Baskcomb L, Huxter N, Barker K, et al. Constitutional 11p15 abnormalities, including heritable imprinting center mutations, cause nonsyndromic Wilms tumor. Nat Genet 2008;40:1329–34.10.1038/ng.243Search in Google Scholar

4. Abeloff MD. Paraneoplastic syndromes: a window on the biology of cancer. N Engl J Med 1987;317:1598–600.10.1056/NEJM198712173172509Search in Google Scholar

5. de Graaf JH, Tamminga RY, Kamps WA. Paraneoplastic manifestations in children. Eur J Pediatr 1994;153:784–91.10.1007/BF01972883Search in Google Scholar

6. Newell-Price J, Trainer P, Besser M, Grossman A. The diagnosis and differential diagnosis of Cushing’s syndrome and pseudo-Cushing’s states. Endocr Rev 1998;19:647–72.Search in Google Scholar

7. More J, Young J, Reznik Y, Raverot G, Borson-Chazot F, et al. Ectopic ACTH syndrome in children and adolescents. J Clin Endocrinol Metab 2011;96:1213–22.10.1210/jc.2010-2276Search in Google Scholar

8. Cummins GE, Cohen D. Cushing’s syndrome secondary to ACTH-secreting Wilms’ tumor. J Pediatr Surg 1974;9:535–9.10.1016/S0022-3468(74)80021-7Search in Google Scholar

9. Hashimoto K, Takahara J, Ogawa N, Yunoki S, Ofuji T, et al. Adrenocorticotropin, beta-lipotropin, beta-endorphin, and corticotropin-releasing factor-like activity in an adrenocorticotropin-producing nephroblastoma. J Clin Endocrinol Metab 1980;50:461–5.10.1210/jcem-50-3-461Search in Google Scholar PubMed

10. Pombo M, Alvez F, Varela-Cives R, Iglesias-Diz JL, Martinón F, et al. Ectopic production of ACTH by Wilms’ tumor. Horm Res 1982;16:160–3.10.1159/000179497Search in Google Scholar PubMed

11. Thomas RJ, Sen S, Zachariah N, Mammen KE, Raghupathy P, et al. Wilms’ tumor presenting as Cushing’s syndrome. Pediatr Surg Int 1998;13:293–4.10.1007/s003830050321Search in Google Scholar PubMed

12. Wang J, Zhang G. Paraneoplastic Cushing syndrome because of corticotrophin-releasing hormone-secreting Wilms’ tumor. J Pediatr Surg 2008;43:2099–101.10.1016/j.jpedsurg.2008.07.014Search in Google Scholar PubMed

13. Segers H, van der Heyden JC, van den Akker EL, de Krijger RR, Zwaan CM, et al. Cushing syndrome as a presenting symptom of renal tumors in children. Pediatr Blood Cancer 2009;53: 211–3.10.1002/pbc.22021Search in Google Scholar

14. Vasei M, Moch H, Mousavi A, Kajbafzadeh AM, Sauter G. Immunohistochemical profiling of Wilms tumor: a tissue microarray study. Appl Immunohistochem Mol Morphol 2008;16:128–34.10.1097/PAI.0b013e31804d6825Search in Google Scholar

15. Magee F, Mah RG, Taylor GP, Dimmick JE. Neural differentiation in Wilms’ tumor. Hum Pathol 1987;18:33–7.10.1016/S0046-8177(87)80190-9Search in Google Scholar

16. Coppes MJ. Serum biological markers and paraneoplastic syndromes in Wilms tumor. Med Pediatr Oncol 1993;21:213–21.10.1002/mpo.2950210311Search in Google Scholar

17. Khan AB, Carachi R, Leckie BJ, Lindop GB. Hypertension associated with increased renin concentrations in nephroblastoma. Arch Dis Child 1991;66:525–6.10.1136/adc.66.4.525Search in Google Scholar

18. Wu AH, Parker OS, Ford L. Hyperviscosity caused by hyaluronic acid in serum in a case of Wilms’ tumor. Clin Chem 1984;30:914–6.10.1093/clinchem/30.6.914Search in Google Scholar

19. Dreicer R, Donovan J, Benda JA, Lund J, Degowin RL. Paraneoplastic erythrocytosis in a young adult with an erythropoietin-producing Wilms’ tumor. Am J Med 1992;93:229–30.10.1016/0002-9343(92)90056-HSearch in Google Scholar

20. Grundy P, Breslow N, Green DM, Sharples K, Evans A, et al. Prognostic factors for children with recurrent Wilms tumor: results from the Second and Third National Wilms’ tumor Study. J Clin Oncol 1989;7:638–47.10.1200/JCO.1989.7.5.638Search in Google Scholar PubMed

21. Perlman EJ, Grundy PE, Anderson JR, Jennings LJ, Green DM, et al. WT1 mutation and 11p15 loss of heterozygosity predict relapse in very low-risk Wilms tumors treated with surgery alone: a children’s oncology group study. J Clin Oncol 2011;29:698–703.10.1200/JCO.2010.31.5192Search in Google Scholar PubMed PubMed Central

Received: 2014-1-14
Accepted: 2014-7-9
Published Online: 2014-8-15
Published in Print: 2014-11-1

©2014 by De Gruyter

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