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A patient with ectopic cortisol production derived from malignant testicular masses

Nature Clinical Practice Endocrinology & Metabolism volume 4, pages 695–700 (2008)Cite this article

Abstract

Background A 65-year-old man presented to an oncology clinic with bilateral testicular masses, lower extremity edema, and cushingoid appearance.

Investigations Measurements of serum cortisol and adrenocorticotropic hormone levels, testicular ultrasound and abdominal CT scans, and review of histopathology to identify the cellular origin of the ectopic cortisol production.

Diagnosis Cushing syndrome was diagnosed on the basis of a markedly elevated 24-hour urine free cortisol level and classic cushingoid features. The etiology of Cushing syndrome was determined to be an adrenocortical carcinoma arising from testicular adrenal rest cells. Nevertheless, the possibility of a malignant Leydig cell tumor with ectopic cortisol production could not be excluded.

Management Mitotane and metyrapone were used to decrease cortisol production. Excess mineralocorticoid activity was blocked with spironolactone; sodium retention was also managed with sodium restriction and diuretics. Despite initial success with this regimen, the patient died as a result of tumor progression and complications of poorly controlled hypercortisolism.

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Figure 1: Photomicrograph of a tissue specimen from the left testicular tumor of the case patient.
Figure 2: Photomicrograph of a tissue specimen from the left testicular tumor of the case patient.
Figure 3: Adrenal steroid pathway illustrating the sites of action for steroidogenic enzyme inhibitors.

References

  1. Nieman LK et al. (2008) The diagnosis of Cushing's syndrome: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab 93: 1526–1540

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. Papadimitris C et al. (2000) Cushing syndrome as the presenting feature of metastatic Leydig cell tumor of the testis. Urology 56: 153

    Article  CAS  PubMed  Google Scholar 

  3. Knyrim K et al. (1981) Autonomous cortisol secretion by a metastatic Leydig cell carcinoma associated with Klinefelter's syndrome. J Cancer Res Clin Oncol 100: 85–93

    Article  CAS  PubMed  Google Scholar 

  4. Fore WW et al. (1972) Cortisol production by testicular tumors in adrenogenital syndrome. Arch Intern Med 130: 59–63

    Article  CAS  PubMed  Google Scholar 

  5. Barwick TD et al. (2005) Embryology of the adrenal glands and its relevance to diagnostic imaging. Clin Radiol 60: 953–959

    Article  CAS  PubMed  Google Scholar 

  6. Rutgers JL et al. (1988) The testicular “tumor” of the adrenogenital syndrome: a report of six cases and review of the literature on testicular masses in patients with adrenocortical disorders. Am J Surg Pathol 12: 503–513

    Article  CAS  PubMed  Google Scholar 

  7. Feldman PS et al. (1982) Malignant Leydig cell tumor: clinical, histologic and electron microscopic features. Cancer 49: 714–721

    Article  CAS  PubMed  Google Scholar 

  8. Emerson RE and Ulbright TM (2007) Morphological approach to tumours of the testis and paratestis. J Clin Pathol 60: 866–880

    Article  PubMed  PubMed Central  Google Scholar 

  9. Guo T et al. (2006) Simultaneous determination of 12 steroids by isotope dilution liquid chromatography-photospray ionization tandem mass spectrometry. Clin Chim Acta 372: 76–82

    Article  CAS  PubMed  Google Scholar 

  10. Nieman LK (2002) Medical therapy of Cushing's disease. Pituitary 5: 77–82

    Article  CAS  PubMed  Google Scholar 

  11. Sartor O and Cutler GB II (1996) Mifepristone: treatment of Cushing's syndrome. Clin Obstet Gynecol 39: 506–510

    Article  CAS  PubMed  Google Scholar 

  12. Schulte HM et al. (1990) Infusion of low dose etomidate: correction of hypercortisolemia in patients with Cushing's syndrome and dose-response relationship in normal subjects. J Clin Endocrinol Metab 70: 1426–1430

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgements

Désirée Lie, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the Medscape-accredited continuing medical education activity associated with this article.

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Authors and Affiliations

  1. SH Jain is a Clinical and Research Fellow and RG Dluhy is a Professor of Medicine in the Division of Endocrinology, Diabetes, and Hypertension at Brigham and Women's Hospital and Harvard Medical School. PM Sadow is an Assistant Pathologist at Massachusetts General Hospital and an Instructor in Pathology at Harvard Medical School. V Nosé is the Associate Director of Surgical Pathology at Brigham and Women's Hospital and an Associate Professor of Pathology at Harvard Medical School, Boston, MA, USA.,

    Shilpa H Jain, Peter M Sadow, Vania Nosé & Robert G Dluhy

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  1. Shilpa H Jain
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  2. Peter M Sadow
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  3. Vania Nosé
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  4. Robert G Dluhy
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Correspondence to Shilpa H Jain.

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The authors declare no competing financial interests.

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Jain, S., Sadow, P., Nosé, V. et al. A patient with ectopic cortisol production derived from malignant testicular masses. Nat Rev Endocrinol 4, 695–700 (2008). https://doi.org/10.1038/ncpendmet0985

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