Background and significance: Endogenous Cushings syndrome is a rare disease entity approximately 13 cases per million population. In Asia, and specifically in the Philippines incidence is unknown. As well, its clinical presentation among Filipinos is not well described. The local epidemiology of obesity, diabetes and hypertension are different from other countries. These conditions are the usual initial differential diagnosis for Cushings syndrome. This report on a collection is thus important for the awareness of clinicians on the clinical presentation and course of individuals with this condition.
Methodology: Medical records of patients diagnosed with Cushings syndrome within the year 20052011 were retrieved using the ICD-10 code for Cushings syndrome (E24). All selected medical records were individually reviewed, assessed and carefully recorded.
Results: The study included 19 cases (eight pituitary adenoma, seven adrenal adenoma, four ectopic adenoma) of endogenous Cushings syndrome. Female predominance was noted. The most commonly reported clinical symptom and sign were weight gain and moon facies respectively. Short clinical course, hyperpigmentation, ecchymoses, behavioural changes and severe hypokalemia were observed to be more prominent among cases with ectopic sources. All cases had significantly elevated midnight serum cortisol level and 24 h urine cortisol with ectopic sources exhibiting the highest level. There was no observed correlation between the tumour size and level of serum cortisol in all types. Majority underwent tumour resection leading to complete reversal of steroid excess.
Conclusion: Adrenal, pituitary and ectopic foci are the main sources of endogenous steroid excess. Ectopic ACTH syndrome manifests differently with other causes providing clues that might help in rapid clinical differentiation. Imaging studies should only be used to confirm tumour location after rigorous biochemical tests. Surgical management remains to be the definitive cure for most of the cases, hence prompt diagnosis and localization is necessary.
27 Apr - 01 May 2013
European Society of Endocrinology