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Adrenocorticotropin-independent Cushing’s syndrome in pregnancy related to overexpression of adrenal luteinizing hormone/human chorionic gonadotropin receptors

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Abstract

Cushing’s syndrome during pregnancy is rare, and rather than being of pituitary origin most patients exhibit ACTH-independent adrenal hypercortisolism. In some cases the syndrome has spontaneously resolved post partum, suggesting the presence of a pregnancy-associated stimulatory factor(s). We describe a case with aberrant adrenal LH/hCG receptors in a large adrenal tumor as a possible explanation for cortisol hypersecretion and tumor growth in Cushing’s syndrome during pregnancy. A 27-yr-old woman presented with hypertension and diabetes mellitus in early pregnancy. Investigations revealed hypercortisolemia, suppressed ACTH-levels, and a 6.4-cm right adrenal tumor. The tumor was successfully removed by laparoscopy at 26th week of pregnancy. Hypercortisolism and hypertension resolved post-operatively. The tumor displayed higher LH/hCG receptor mRNA and protein positivity than adjacent normal adrenal tissue as examined by in situ hybridization and immunocytochemistry. High physiological levels of hCG, in conjunction with aberrant adrenal LH/hCG receptor overexpression, may have contributed to the development of Cushing’s syndrome in pregnancy.

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Authors and Affiliations

  1. Department of Medicine, Örebro University Hospital, 70185, Örebro, Sweden

    E. Rask MD, PhD & E. Schvarcz

  2. Department of Surgery, Institute of Medical Sciences, Uppsala University Hospital, Uppsala, Sweden

    P. Hellman & J. Hennings

  3. Internal Medicine, Division of Research, Uppsala University Hospital, Uppsala, Sweden

    F. A. Karlsson

  4. Florida International University College of Medicine, Miami, Florida, USA

    C. V. Rao

Authors
  1. E. Rask MD, PhD
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  2. E. Schvarcz
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  3. P. Hellman
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  4. J. Hennings
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  5. F. A. Karlsson
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  6. C. V. Rao
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Correspondence to E. Rask MD, PhD.

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Rask, E., Schvarcz, E., Hellman, P. et al. Adrenocorticotropin-independent Cushing’s syndrome in pregnancy related to overexpression of adrenal luteinizing hormone/human chorionic gonadotropin receptors. J Endocrinol Invest 32, 313–316 (2009). https://doi.org/10.1007/BF03345718

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  • DOI: https://doi.org/10.1007/BF03345718

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