Skip to main content
Log in

A sporadic case of paraganglioma undetected by urine metabolite screening

  • Brief Report
  • Published:
Pediatric Nephrology Aims and scope Submit manuscript

Abstract

Phaeochromocytoma is a rare cause of hypertension in children, but important to be recognised, as hypertension can be severe and surgery is often curative. Here, we report on a 7-year-old boy with a phaeochromocytoma, who had normal levels of commonly assayed catecholamine metabolites in the urine. Postoperatively, the patient developed renal vein thrombosis. Appropriate screening tests for phaeochromocytomas, peri-operative management, and the high incidence of an underlying genetic basis, even in sporadic cases, are discussed.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

References

  1. Davidson DF (2005) Elevated urinary dopamine in adults and children. Ann Clin Biochem 42:200–207

    CAS  PubMed  Google Scholar 

  2. Eisenhofer G, Keiser H, Friberg P, Mezey E, Huynh TT, Hiremagalur B, Ellingson T, Duddempudi S, Eijsbouts A, Lenders JW (1998) Plasma metanephrines are markers of pheochromocytoma produced by catechol-O-methyltransferase within tumors. J Clin Endocrinol Metab 83:2175–2185

    CAS  PubMed  Google Scholar 

  3. Eisenhofer G, Kopin IJ, Goldstein DS (2004) Catecholamine metabolism: a contemporary view with implications for physiology and medicine. Pharmacol Rev 56:331–349

    CAS  PubMed  Google Scholar 

  4. Pacak K, Eisenhofer G, Ahlman H, Bornstein SR, Gimenez-Roqueplo AP, Grossman AB, Kimura N, Mannelli M, McNicol AM, Tischler AS (2007) Pheochromocytoma: recommendations for clinical practice from the First International Symposium. October 2005. Nat Clin Pract Endocrinol Metab 3:92–102

    PubMed  Google Scholar 

  5. Young WF Jr (2007) Clinical practice. The incidentally discovered adrenal mass. N Engl J Med 356:601–610

    CAS  PubMed  Google Scholar 

  6. Desmonts JM, Marty J (1984) Anaesthetic management of patients with phaeochromocytoma. Br J Anaesth 56:781–789

    CAS  PubMed  Google Scholar 

  7. Johnson MD, Smith PG, Mills E, Schanberg SM (1983) Paradoxical elevation of sympathetic activity during catecholamine infusion in rats. J Pharmacol Exp Ther 227:254–259

    CAS  PubMed  Google Scholar 

  8. Newell KA, Prinz RA, Brooks MH, Glisson SN, Barbato AL, Freeark RJ (1988) Plasma catecholamine changes during excision of pheochromocytoma. Surgery 104:1064–1073

    CAS  PubMed  Google Scholar 

  9. Bravo EL, Tagle R (2003) Pheochromocytoma: state-of-the-art and future prospects. Endocr Rev 24:539–553

    CAS  PubMed  Google Scholar 

  10. Hack HA (2000) The perioperative management of children with phaeochromocytoma. Paediatr Anaesth 10:463–476

    CAS  PubMed  Google Scholar 

  11. Gimenez-Roqueplo AP, Lehnert H, Mannelli M, Neumann H, Opocher G, Maher ER, Plouin PF (2006) Phaeochromocytoma, new genes and screening strategies. Clin Endocrinol (Oxf) 65:699–705

    CAS  Google Scholar 

  12. Neumann HP, Bausch B, McWhinney SR, Bender BU, Gimm O, Franke G, Schipper J, Klisch J, Altehoefer C, Zerres K, Januszewicz A, Eng C, Smith WM, Munk R, Manz T, Glaesker S, Apel TW, Treier M, Reineke M, Walz MK, Hoang-Vu C, Brauckhoff M, Klein-Franke A, Klose P, Schmidt H, Maier-Woelfle M, Peczkowska M, Szmigielski C, Eng C (2002) Germ-line mutations in nonsyndromic pheochromocytoma. N Engl J Med 346:1459–1466

    CAS  PubMed  Google Scholar 

  13. Fuentes C, Menendez E, Pineda J, Martinez De Esteban JP, Anda E, Goni MJ, Bausch B, Neumann HP (2006) The malignant potential of a succinate dehydrogenase subunit B germline mutation. J Endocrinol Invest 29:350–352

    CAS  PubMed  Google Scholar 

  14. Opocher G, Schiavi F, Iacobone M, Toniato A, Sattarova S, Erlic Z, Martella M, Mian C, Merante Boschin I, Zambonin L, De Lazzari P, Murgia A, Pelizzo MR, Favia G, Mantero F (2006) Familial nonsyndromic pheochromocytoma. Ann N Y Acad Sci 1073:149–155

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Detlef Bockenhauer.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Bockenhauer, D., Rees, L., Neumann, H. et al. A sporadic case of paraganglioma undetected by urine metabolite screening. Pediatr Nephrol 23, 1889–1891 (2008). https://doi.org/10.1007/s00467-008-0841-y

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00467-008-0841-y

Keywords

Navigation