Recurrent Cushing’s Syndrome

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This is a 28 year old woman with Cushing's Syndrome
  • 28-year-old woman
  • Symptoms of amenorrhoea
  • Central weight gain
  • Severe depression

 

  • On examination, the patient had some features of Cushing’s Syndrome (CS).
  • Investigation with the low-dose (48 hour) dexamethasone suppression test revealed her 09:00 serum cortisol level fell from 525 nmol/L to 220 nmol/L.
  • A subsequent corticotrophin-releasing hormone test showed a rise in serum cortisol from 525 nmol/L basally to 1250 nmol/L at 60 minutes with a concomitant rise in ACTH from 45 ng/L to 260 ng/L.
  • A pituitary MRI was reported to be normal. 

What would you do next?

  1. Undertake a bilateral petrosal sinus sampling (BIPSS) to assess for Cushing’s Disease
  2. Request an adrenal MRI to identify any adrenal lesions
  3. Provide medical therapy to address excessive cortisol levels
  • The patient had only mild Cushing’s syndrome, with dynamic tests suggesting, but not proving, Cushing’s disease.
  • Bilateral petrosal sinus sampling showed a rise in the left sinus from 85 ng/L to 1250 ng/L with a simultaneous peripheral ACTH of 240 ng/L prompting a diagnosis of pituitary-dependent Cushing’s Syndrome, or Cushing’s Disease.
  • The patient was started on low-molecular weight heparin and underwent transsphenoidal surgery. Following surgery, the patient showed a 09:00 serum cortisol of 25 nmol/L.
  • She was started on hydrocortisone replacement therapy and all symptoms resolved and her periods returned. The hydrocortisone was tapered off with recovery of her own pituitary-adrenal axis after two years.
  • Having turned 38 years of age, the patient showed signs of clinical and biochemical recurrence of her Cushing’s Disease. An MRI scan showed a probable tumour in her left cavernous sinus.

The patient does not want to have more children. How would you treat her Cushing’s Disease?

  1. Repeat transsphenoidal surgery
  2. Perform a total hypophysectomy
  3. Provide radiosurgery
  • With probable tumour invasion of the cavernous sinus ruling out further surgery, the patient was treated with radiosurgery to the tumour remnant/growth. She was also treated with ketoconazole to normalise her cortisol levels.
  • Two years later, the patient’s tumour remained stable and she was able to discontinue ketoconazole as her serum cortisol levels had normalised. However, she continues to be regularly assessed for other hormone deficiencies, especially growth hormone deficiency.

Conclusion

Patients with ACTH-dependent Cushing’s Syndrome and a normal pituitary MRI should undergo BIPSS to confirm the source of ACTH. If the source is confirmed as the pituitary gland, then transsphenoidal surgery should be performed. However, recurrence may be seen many years later and therapy should be tailored to the patient’s age, sex, lifestyle requirements and disease.

 

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