J Korean Endocr Soc.  2007 Feb;22(1):62-67. 10.3803/jkes.2007.22.1.62.

A Case of Cushing's Disease with Renal Cell Carcinomaand End-stage Renal Disease

• Lee JE
• Choi IJ
• Park YJ
• Lee HJ
• Lee KH
• Ko SH
• Song KH
• Ahn YB

• KMID: 2178244
• DOI: http://doi.org/10.3803/jkes.2007.22.1.62

Abstract

Cushing's disease is the most common cause of endogenous Cushing's syndrome caused by the excessive secretion of adrenocorticotropic hormone (ACTH) by pituitary ACTH-secreting tumors. The tumors are usually microadenomas (< 10 mm in diameter). Macroadenomas are rare, and corticotroph hyperplasia and carcinomas are extremely rare. Patients with these tumors exhibit an increase in plasma ACTH and cortisol levels, elevated urinary excretion of cortisol and adrenocortical steroid metabolites, evidence of altered negative feedback of cortisol and disturbances in neuroendocrine regulation. The diagnosis of Cushing's disease can be challenging in patients with renal failure the presence of abnormal plasma cortisol, poor gastrointestinal tract absorption of dexamethasone and altered functioning of the hypophyseal-pituitary-adrenal axis have been reported in end-stage renal disease, and the urinary cortisol increment method is not reliable in these patients. The prevalence of renal cell carcinoma in patients on dialysis is higher than in the general population. We recently treated a patient with Cushing's disease and end-stage renal disease as a result of renal cell carcinoma. The diagnosis was made by laboratory evaluation of plasma and radiological examination. Here we report a case of Cushing's disease with a review of the medical literature.

Keyword

Cushing's disease; End-stage renal disease; Renal cell carcinoma

MeSH Terms

Absorption
Adrenocorticotropic Hormone
Axis, Cervical Vertebra
Carcinoma, Renal Cell
Corticotrophs
Cushing Syndrome
Dexamethasone
Diagnosis
Dialysis
Gastrointestinal Tract
Humans
Hydrocortisone
Hyperplasia
Kidney Failure, Chronic*
Plasma
Prevalence
Renal Insufficiency
Adrenocorticotropic Hormone
Dexamethasone
Hydrocortisone

Figure

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  Fig. 1 Central obesity, moon face (A) and abdominal purple striae (B) in 38 years old women with Cushing's disease.

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  Fig. 2 Coronal section of sellar MRI scan shows l.5 cm sized pituitary mass with a left deviated pituitary stalk. T2 weighted enhanced coronal section image.

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  Fig. 3 Abdmonen CT shows a 2.9 × 2.5 × 2.3 cm sized enhancing mass in the right kidney.

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  Fig. 4 Histology of the pituitary mass. A. Tumor cells have eosinophilic cytoplasm and relatively uniform, polygonal cells arrayed in cords. (H&E stain, ×400) B. Immunohistochemial stain of pituitary adenoma shows diffuse and strongly reactive with ACTH (adrenocorticotropic hormone). (IHC, ×400)

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  Fig. 5 Histological examination of the right kidney shows well differentiated tumor cell with abundant clear cytoplasm. (H&E stain, ×200)

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