Hyperplasia of adrenal rest tissue causing a retroperitoneal mass in a child with 11 beta-hydroxylase deficiency

Helen L Storr; Tara D Barwick; Graeme A I Snodgrass; Robert Booy; Yves Morel; Rodney H Reznek; Martin O Savage

doi:10.1159/000071878

Hyperplasia of adrenal rest tissue causing a retroperitoneal mass in a child with 11 beta-hydroxylase deficiency

Horm Res. 2003;60(2):99-102. doi: 10.1159/000071878.

Authors

Helen L Storr¹, Tara D Barwick, Graeme A I Snodgrass, Robert Booy, Yves Morel, Rodney H Reznek, Martin O Savage

Affiliation

¹ Department of Endocrinology, St Bartholomew's and The Royal London School of Medicine and Dentistry, London, UK.

PMID: 12876421
DOI: 10.1159/000071878

Abstract

We report the case of a child with congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency, with hyperplasia of adrenal rest tissue presenting as a retroperitoneal mass. Complete resolution of the mass was noted after 18 months of hydrocortisone replacement therapy.

Publication types

Case Reports

MeSH terms

Adrenal Hyperplasia, Congenital / blood
Adrenal Hyperplasia, Congenital / complications*
Child, Preschool
Humans
Hydrocortisone / therapeutic use
Magnetic Resonance Imaging
Male
Metabolism, Inborn Errors / complications
Metabolism, Inborn Errors / enzymology*
Retroperitoneal Neoplasms / blood
Retroperitoneal Neoplasms / drug therapy
Retroperitoneal Neoplasms / etiology*
Steroid 11-beta-Hydroxylase / adverse effects
Steroid 11-beta-Hydroxylase / genetics
Steroid 11-beta-Hydroxylase / metabolism*
Virilism / etiology

Substances

Steroid 11-beta-Hydroxylase
Hydrocortisone