Endogenous Cushing’s syndrome: The Philippine general hospital experience

Abstract

Background and significance: Endogenous Cushing's syndrome is a rare disease entity approximately 13 cases permillion population. In Asia, and specifically in the Philippines, the exact incidence is unknown. As well, it's 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 Cushing's syndrome. This case 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 Endogenous Cushing’s Syndrome (non steroid-induced) withinthe year 2005-2011 were retrieved using the ICD 10 code for Cushing’s Syndrome (E24). Exogenous Cushing’sSyndrome (steroid-induced) were all excluded from the study. All selected medical records were individually reviewed,assessed and carefully recorded.

Results: The study included 19 cases (8 pituitary adenoma, 7 adrenal adenoma, 4 ectopic adenoma) of endogenous Cushing’s 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 andsevere hypokalemia were observed to be more prominent among cases with ectopic sources. All cases had significantlyelevated midnight serum cortisol level and 24 hour 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: In our series of 19 cases, adrenal, pituitary and ectopic foci were the main sources of endogenous steroid excess. Ectopic ACTH syndrome manifested 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 remained to be the definitive cure for most of the cases, hence prompt diagnosis and localization is necessary.