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Pia Burman

Physician

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11C-Metomidate PET/CT Detected Multiple Ectopic Adrenal Rest Tumors in a Woman with Congenital Adrenal Hyperplasia

Author

  • Pia Burman
  • Henrik Falhammar
  • Erik Waldenström
  • Anders Sundin
  • Ulrika Bitzén

Summary, in English

Context: Women with congenital adrenal hyperplasia (CAH) may present with androgen excess that is difficult to control with conventional suppressive doses of glucocorticoids. Clinical management is challenging, and the woman is at great risk of developing steroid-induced complications. Patients and Methods: A 32-year-old woman with salt-wasting CAH due to 21-hydroxylase deficiency underwent right-sided adrenalectomy because of a large myelolipoma. Over the years, androgens became increasingly difficult to suppress on prednisolone 5+0+2.5 mg daily, and at age 39 years the left adrenal with an enlarging myelolipoma was removed. A month later serum testosterone levels had increased from 4.1 preoperatively to 18.3 nmol/L (reference 0.2-1.8 nmol/L), and adrenocorticotropin levels from 32 to 283 pmol/L (reference <14 pmol/L). No adrenal parenchyma was visualized on computed tomography (CT). In the further search for the source of the markedly elevated testosterone, positron emission tomography (PET) was performed with 2 different tracers, 18fluorodeoxyglucose (18FDG) reflecting glucose metabolism and 11C-metomidate, an inhibitor of 11-β-hydroxylase targeting adrenocortical tissue. Results: 18FDG-PET/CT with cosyntropin stimulation showed ovarian/paraovarian hypermetabolism, suggestive of adrenal rest tumors. Further characterization with 11C-metomidate PET/CT showed uptakes localized to the ovaries/adnexa, behind the spleen, and between the right crus diaphragmaticus and inferior vena cava. Conclusion: Adrenal rest tumors can give rise to high androgen levels in spite of suppressive supraphysiological glucocorticoid doses. This case illustrates, for the first time, the value of 11C-metomidate PET as a sensitive method in documenting adrenal rest tumors, currently considered rare in women with CAH.

Department/s

  • Genomics, Diabetes and Endocrinology
  • EXODIAB: Excellence of Diabetes Research in Sweden

Publishing year

2021-02-01

Language

English

Pages

675-679

Publication/Series

Journal of Clinical Endocrinology and Metabolism

Volume

106

Issue

2

Document type

Journal article

Publisher

Oxford University Press

Topic

  • Endocrinology and Diabetes
  • Radiology, Nuclear Medicine and Medical Imaging

Keywords

  • FDG PET/CT
  • 21-hydroxylase deficiency
  • adrenalectomy
  • cosyntropin
  • ovarian adrenal rest tumors
  • retroperitoneal adrenal rest tumors

Status

Published

Research group

  • Genomics, Diabetes and Endocrinology

ISBN/ISSN/Other

  • ISSN: 0021-972X