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Androgen Secreting Adenoma Co- Secreting Cortisol First Diagnosed During Pregnancy

Background: Pure androgen secreting adenoma is rare (1) and rarer to have an adenoma that is co-secreting androgen and cortisol, and extremely rare to have it diagnosed during pregnancy. In female, an elevated serum testosterone level with normal adrenal androgens is usually regarded as ovary origin...

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Autores principales: Al Ghareeb, Zahra, Alwosaibi, Abdulaziz, Alobaidan, Hoor
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8089884/
http://dx.doi.org/10.1210/jendso/bvab048.234
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author Al Ghareeb, Zahra
Alwosaibi, Abdulaziz
Alobaidan, Hoor
author_facet Al Ghareeb, Zahra
Alwosaibi, Abdulaziz
Alobaidan, Hoor
author_sort Al Ghareeb, Zahra
collection PubMed
description Background: Pure androgen secreting adenoma is rare (1) and rarer to have an adenoma that is co-secreting androgen and cortisol, and extremely rare to have it diagnosed during pregnancy. In female, an elevated serum testosterone level with normal adrenal androgens is usually regarded as ovary origin (2). Here we report an unusual case of pregnant woman with markedly elevated serum testosterone level and elevated cortisol that first presented during pregnancy. Clinical Case: A 32-year old lady who is 32 weeks pregnant, presented with signs and symptoms of Cushing and acute hirsutism developed and advanced during pregnancy. Clinically She had hypertension, gestational diabetes with sever hirsutism and virilization. Fasting total testosterone was high (52 nmoL/L n 0.2–2.9 nmoL/L), DHEAS (1.98 umol/L n 0.51–11 umol), 24 hours urine cortisol (1343 nmoL/24 hrs n 11.8–485 nmoL/24 hrs), ACTH (0.220 pmol/L n 1.6–13.9pmol/L). Adrenal MRI without contrast showed a large right adrenal mass of 6.5 x 6.2 x 5.3 cm, of heterogenous signal in all pulse sequences, with limited assessment due to the lack of IV contrast. During admission,the patient’s liver enzymes were gradually increasing which lead to the decision of an elective C-section at 34 weeks gestation followed by right robotic adrenalectomy after ruling out pheochromocytoma. The product was viable and healthy baby boy. The immediate post-op hormonal profile showed normalization of urinary cortisol and serum total testosterone level. Histopathology report confirmed an Adrenocortical adenoma. The patient was discharged on the 3(rd) day post-op on hydrocortisone replacement therapy. The hydrocortisone doses were gradually tapered down tell stopped. The course of follow up was uneventful, two years following the surgery, she got another pregnancy with no obvious complications. Conclusion: Co- existing testosterone and cortisol secreting adrenocortical adenoma is a rare tumor, diagnosis and management during pregnancy is challenging, with surgical resection being the only possible definite management. References: (1) L. Barzon, N. Sonino, F. Fallo, G. Palù, and M. Boscaro, “Prevalence and natural history of adrenal incidentalomas,” European Journal of Endocrinology, vol. 149, no. 4, pp. 273–285, 2003.(2) Zhou, Wb., Chen, N. & Li, Cj. A rare case of pure testosterone-secreting adrenal adenoma in a postmenopausal elderly woman. BMC Endocr Disord19, 14 (2019). https://doi.org/10.1186/s12902-019-0342-y
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spelling pubmed-80898842021-05-06 Androgen Secreting Adenoma Co- Secreting Cortisol First Diagnosed During Pregnancy Al Ghareeb, Zahra Alwosaibi, Abdulaziz Alobaidan, Hoor J Endocr Soc Adrenal Background: Pure androgen secreting adenoma is rare (1) and rarer to have an adenoma that is co-secreting androgen and cortisol, and extremely rare to have it diagnosed during pregnancy. In female, an elevated serum testosterone level with normal adrenal androgens is usually regarded as ovary origin (2). Here we report an unusual case of pregnant woman with markedly elevated serum testosterone level and elevated cortisol that first presented during pregnancy. Clinical Case: A 32-year old lady who is 32 weeks pregnant, presented with signs and symptoms of Cushing and acute hirsutism developed and advanced during pregnancy. Clinically She had hypertension, gestational diabetes with sever hirsutism and virilization. Fasting total testosterone was high (52 nmoL/L n 0.2–2.9 nmoL/L), DHEAS (1.98 umol/L n 0.51–11 umol), 24 hours urine cortisol (1343 nmoL/24 hrs n 11.8–485 nmoL/24 hrs), ACTH (0.220 pmol/L n 1.6–13.9pmol/L). Adrenal MRI without contrast showed a large right adrenal mass of 6.5 x 6.2 x 5.3 cm, of heterogenous signal in all pulse sequences, with limited assessment due to the lack of IV contrast. During admission,the patient’s liver enzymes were gradually increasing which lead to the decision of an elective C-section at 34 weeks gestation followed by right robotic adrenalectomy after ruling out pheochromocytoma. The product was viable and healthy baby boy. The immediate post-op hormonal profile showed normalization of urinary cortisol and serum total testosterone level. Histopathology report confirmed an Adrenocortical adenoma. The patient was discharged on the 3(rd) day post-op on hydrocortisone replacement therapy. The hydrocortisone doses were gradually tapered down tell stopped. The course of follow up was uneventful, two years following the surgery, she got another pregnancy with no obvious complications. Conclusion: Co- existing testosterone and cortisol secreting adrenocortical adenoma is a rare tumor, diagnosis and management during pregnancy is challenging, with surgical resection being the only possible definite management. References: (1) L. Barzon, N. Sonino, F. Fallo, G. Palù, and M. Boscaro, “Prevalence and natural history of adrenal incidentalomas,” European Journal of Endocrinology, vol. 149, no. 4, pp. 273–285, 2003.(2) Zhou, Wb., Chen, N. & Li, Cj. A rare case of pure testosterone-secreting adrenal adenoma in a postmenopausal elderly woman. BMC Endocr Disord19, 14 (2019). https://doi.org/10.1186/s12902-019-0342-y Oxford University Press 2021-05-03 /pmc/articles/PMC8089884/ http://dx.doi.org/10.1210/jendso/bvab048.234 Text en © The Author(s) 2021. Published by Oxford University Press on behalf of the Endocrine Society. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs licence (http://creativecommons.org/licenses/by-nc-nd/4.0/ (https://creativecommons.org/licenses/by-nc-nd/4.0/) ), which permits non-commercial reproduction and distribution of the work, in any medium, provided the original work is not altered or transformed in any way, and that the work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Adrenal
Al Ghareeb, Zahra
Alwosaibi, Abdulaziz
Alobaidan, Hoor
Androgen Secreting Adenoma Co- Secreting Cortisol First Diagnosed During Pregnancy
title Androgen Secreting Adenoma Co- Secreting Cortisol First Diagnosed During Pregnancy
title_full Androgen Secreting Adenoma Co- Secreting Cortisol First Diagnosed During Pregnancy
title_fullStr Androgen Secreting Adenoma Co- Secreting Cortisol First Diagnosed During Pregnancy
title_full_unstemmed Androgen Secreting Adenoma Co- Secreting Cortisol First Diagnosed During Pregnancy
title_short Androgen Secreting Adenoma Co- Secreting Cortisol First Diagnosed During Pregnancy
title_sort androgen secreting adenoma co- secreting cortisol first diagnosed during pregnancy
topic Adrenal
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8089884/
http://dx.doi.org/10.1210/jendso/bvab048.234
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