Wednesday, March 27, 2013

Early diagnosis of pediatric Cushing’s disease improved surgical outcomes


Lonser RR. J Clin Endocrinol Metab. 2013;93:892-901.

  • March 27, 2013
Early postoperative endocrine testing could predict lasting remission among pediatric patients with Cushing’s disease, according to recent study findings published in the Journal of Clinical Endocrinology & Metabolism. Other findings suggest early diagnosis has the potential to improve surgical outcomes due to an association with younger age, smaller adenomas and lack of dural invasion.
Researchers at the NIH included 200 pediatric patients (106 females, 94 males) with Cushing’s disease in the prospective observational study from 1982 to 2010. The mean age was 10.6 years (range; 4 to 19 years) at the time of symptom development and 13.7 years at the time of the NIH study. However, 27 (13%) patients underwent prior surgery at another institution, the researchers reported.
Using MRI, researchers identified adenomas in 97 patients (50%); when positive, the MRI accurately specified discrete adenoma in 96 of the 97 patients (99%). The researchers wrote that this was more accurate compared with adrenocorticotropic hormone (ACTH) ratios during inferior petrosal sinus sampling to influence adenoma lateralization (accurate in 72% of patients without prior surgery).
Moreover, 195 of the 200 patients (98%) demonstrated remission after surgery (189 [97%] were hypocortisolemic; six [3%] were eucortisolemic postoperatively). Factors associated with initial remission (P<.05) included identification of an adenoma at surgery, immunohistochemical ACTH-producing adenoma and noninvasive ACTH adenoma.
According to researchers, younger age, smaller adenoma and absence of cavernous sinus wall or other dural invasion were associated with long-term remission (P<.05). Finally, minimum morning serum cortisol of less than 1 mcg/dL after surgery also demonstrated a positive predictive value for a lasting remission of 96%, the researchers wrote.

Disclosure: The researchers report no relevant financial disclosures.