Cyclic Cushing syndrome due to an ectopic pituitary adenoma

Rahfa K. Zerikly, Esin Eray, Charles Faiman, Richard Prayson, Robert R. Lorenz, Robert J. Weil, Amir H. Hamrahian

Research output: Contribution to journalArticle

Abstract

Background: A 39-year-old man was referred to an endocrinology clinic for evaluation of his Cushing syndrome. He had gained 20 kg over 5 years and complained of intermittent headaches and easy bruisability. His medical history included a left foot fracture associated with minimal trauma 2 years earlier, hypertension, and stable Crohn disease with no use of exogenous glucocorticoids for at least 10 years. Investigations: Measurements of plasma adrenocorticotropic hormone, 24 h urine free cortisol excretion, late-night salivary cortisol, serum cortisol levels before and after corticotropin- releasing hormone administration during a dexamethasone suppression/ corticotropin-releasing hormone-stimulation test, pituitary MRI, and inferior petrosal sinus sampling. Diagnosis: Cyclic Cushing syndrome secondary to an ectopic pituitary adenoma. Management: The cyclic nature of Cushing syndrome was suggested by the absence of hypercortisolemia during inferior petrosal sinus sampling, and was confirmed by multiple 24 h urine free cortisol measurements. The patient underwent transsphenoidal surgery, during which a 5 mm firm, round, midline sphenoid sinus lesion was identified and resected. In preoperative imaging studies, this lesion had been interpreted as being a mucosal polyp. At microscopic examination, the lesion was found to be a pituitary adenoma, which stained diffusely with antiadrenocorticotropic-hormone antibodies. Explorations of the sella and pituitary did not reveal any abnormalities. Postoperatively, the patient became hypocortisolemic and his cushingoid features resolved. His adrenal function normalized 3 months after surgery. At 18 months, the patient continued to be symptom-free with normal levels of urinary-free cortisol and midnight salivary cortisol.

Original languageEnglish (US)
Pages (from-to)174-179
Number of pages6
JournalNature Clinical Practice Endocrinology and Metabolism
Volume5
Issue number3
DOIs
StatePublished - Mar 1 2009
Externally publishedYes

Keywords

  • Cushing syndrome
  • Cyclic Cushing disease
  • Ectopic pituitary adenoma
  • Inferior petrosal sinus sampling
  • Transsphenoidal pituitary surgery

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Endocrinology

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