11C-Metomidate PET/CT Detected Multiple Ectopic Adrenal Rest Tumors in a Woman With Congenital Adrenal Hyperplasia
| Autor: | Pia Burman, Erik Waldenström, Ulrika Bitzén, Henrik Falhammar, Anders Sundin |
|---|---|
| Rok vydání: | 2020 |
| Předmět: |
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 |
| Zdroj: | The Journal of Clinical Endocrinology & Metabolism. 106:e675-e679 |
| ISSN: | 1945-7197 0021-972X |
| DOI: | 10.1210/clinem/dgaa870 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|