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Research Article Open access CC BY 4.0

The Diagnostic Challenges in ACTH Dependent Cushing’s Disease

Enedina T Cuatecontzi Xochitiotzi, Mariana Quintanar Martínez, Tomás Herrera Arzola, René E Lizola Crespo, Roopa Mehta

International Journal of Medical and Pharmaceutical Case Reports · pp. 6–11 · Published 16 Dec 2014

10.9734/IJMPCR/2015/14571

Abstract

Objective: To discuss the difficulties in the diagnostic workup and management of ACTH dependent Cushing’s syndrome. Methods: We report the case of a patient with ACTH dependent Cushing’s syndrome, in whom the identification of the source of ACTH production was a challenge. Results: A 26-year-old man presented with a weight gain of 20kg, hypertension (250/160 mmHg), hypokalemia, purple stretch marks and severe proximal muscle weakness 6 months of evolution. He also had 4 episodes of pneumonia requiring hospitalization. ACTH-dependent hypercortisolism (cortisol 32ng/dl, ACTH 135pg/ml) was diagnosed. The severe clinical presentation suggested the possibility of ectopic ACTH production. The dexamethasone suppression test (DST) showed no inhibition of cortisol. Whole body octreotide scintigraphy and gallium-68 PET-CT did not show any evidence of ectopic tumor. A pituitary MRI was reported as normal. Bilateral inferior petrosal sinus sampling was carried out and was essential in establishing a diagnosis of Cushing’s disease. In a second MRI, a pituitary microadenoma on the left side was identified. The patient underwent transsphenoidal resection. Postoperatively, hypercortisolism persisted, and since the patients clinical condition deteriorated further, bilateral adrenalectomy was successfully performed, providing a definitive cure for the life threatening hypercortisolism. Conclusion: The diagnosis and management of ACTH dependent Cushing´s syndrome remains a challenge. The results of dexamethasone suppression tests should be evaluated in the context of the patient. These tests have a high negative predictive value; however in the context of critical illness (pneumonia) may alter the hypothalamic-pituitary-adrenal axis response. This case confirms the need for a bilateral IPS sampling in order to establish diagnosis.

Cushing’s disease ectopic Cushing’s syndrome pituitary microadenoma bilateral inferior petrosal sinus.

References (9)

  1. 1 The Diagnosis of Cushing's Syndrome: An Endocrine Society Clinical Practice Guideline [DOI]
  2. 2 Cushing's syndrome [DOI]
  3. 3 Treatment of Adrenocorticotropin-Dependent Cushing’s Syndrome: A Consensus Statement [DOI]
  4. 4 Early Morning Cortisol Levels as a Predictor of Remission After Transsphenoidal Surgery for Cushing’s Disease [DOI]
  5. 5 Desmopressin test during petrosal sinus sampling: a valuable tool to discriminate pituitary or ectopic ACTH-dependent Cushing’s syndrome [DOI]
  6. 6 The role of desmopressin in bilateral and simultaneous inferior petrosal sinus sampling for differential diagnosis of ACTH‐dependent Cushing's syndrome [DOI]
  7. 7 Assessment of the Utility of the High-Dose Dexamethasone Suppression Test in Confirming the Diagnosis of Cushing Disease [DOI]
  8. 8 The hypothalamic-pituitary-adrenal axis in critical illness: response to dexamethasone and corticotropin-releasing hormone. [DOI]
  9. 9 Management of Cushing's Disease After Failed Surgery - A Review [DOI]

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