Hypertension and Virilization Caused by a Unique Desoxycorticosterone- and Androgen-Secreting Adrenal Adenoma
| Autor: | A Greco, N Sivaraman, W D Drucker, Raphael David, Sharon E. Oberfield, Dorothy Soranno, Veeramac Prasad |
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| Rok vydání: | 1999 |
| Předmět: |
Adenoma
medicine.medical_specialty Adolescent medicine.drug_class Hydroxycorticosteroids Endocrinology Diabetes and Metabolism Adrenal Gland Neoplasm Adrenal Gland Neoplasms Adrenocorticotropic hormone Dexamethasone Endocrinology Adrenocorticotropic Hormone Internal medicine Hydroxyprogesterones medicine Humans Adrenal adenoma Desoxycorticosterone business.industry Virilization Androgen medicine.disease Virilism Hypertension Pediatrics Perinatology and Child Health Androgens Female medicine.symptom business Glucocorticoid medicine.drug |
| Zdroj: | Journal of Pediatric Endocrinology and Metabolism. 12 |
| ISSN: | 2191-0251 0334-018X |
| Popis: | We describe a rare androgen and desoxycorticosterone (DOC)-secreting adrenal tumor in a non-Cushingoid 14 year-old Haitian girl with secondary amenorrhea, hypertension and virilization. Her steroid pattern simulated an 11 beta-hydroxylation defect with notable elevation of adrenal androgens, 11-desoxycortisol (S), DOC, 17 alpha-hydroxyprogesterone and pregnenelone. Exogenous ACTH stimulated steroidogenesis. A CAT scan unfortunately failed to delineate an adrenal mass. Dexamethasone (DEX) was administered, therefore, which partially suppressed androgen levels, reduced DOC and S by 80% and 82% respectively, and normalized blood pressure. Nevertheless, the response to glucocorticoid was incomplete and an MRI was obtained, which revealed a right adrenal tumor. Post surgery, the patient promptly resumed menses and became normotensive. This case illustrates that ACTH and DEX cannot reliably differentiate tumor from hyperplasia, whereas the simultaneous increase of delta 4 and delta 5 steroids, present here, may favor a tumor. This case also allows speculation that the hypersecretion of DOC may result from inhibition of 11 beta-hydroxylase activity by excess androgens. The importance of appropriate imaging for diagnosis is underscored. |
| Databáze: | OpenAIRE |
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