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11C-Metomidate PET/CT Detected Multiple Ectopic Adrenal Rest Tumors in a Woman With Congenital Adrenal Hyperplasia
- Source :
- The Journal of Clinical Endocrinology & Metabolism. 106:e675-e679
- Publication Year :
- 2020
- Publisher :
- The Endocrine Society, 2020.
-
Abstract
- 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 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.
- Subjects :
- Adult
Myelolipoma
medicine.medical_specialty
medicine.drug_class
Endocrinology, Diabetes and Metabolism
medicine.medical_treatment
Clinical Biochemistry
Water-Electrolyte Imbalance
Androgen Excess
Biochemistry
Inferior vena cava
Neoplasms, Multiple Primary
Endocrinology
Positron Emission Tomography Computed Tomography
Internal medicine
medicine
Adrenal Rest Tumor
Humans
Etomidate
Congenital adrenal hyperplasia
Carbon Radioisotopes
Retroperitoneal Neoplasms
Ovarian Neoplasms
Sweden
Adrenal Hyperplasia, Congenital
business.industry
Adrenalectomy
Biochemistry (medical)
medicine.disease
Androgen
medicine.vein
Prednisolone
Female
Salts
business
Glucocorticoid
medicine.drug
Subjects
Details
- ISSN :
- 19457197 and 0021972X
- Volume :
- 106
- Database :
- OpenAIRE
- Journal :
- The Journal of Clinical Endocrinology & Metabolism
- Accession number :
- edsair.doi.dedup.....f5a837abcfcba716543780b2a322b07b