Abstract
We report a 10 year old female with Cushing's disease with recurrent disease 5 yrs after successful pituitary irradiation. At presentation, bone age, skull films, head CT and visual fields were all normal. Basal serum F, urinary 17-OHCS, free cortisol (F), and 6β-hydrocortisol (6β-OHF) were elevated. Serum F lacked circadian variation and was partially suppressed by high dose dexamethasone (dex). She received pituitary irradiation with a cumulative dose of 4,000 rads over a 1 month period. Serum F, 17-OHCS, 6β-OHF, urinary free F returned to normal, growth velocity improved, and puberty ensued with menarche occurring 3 yrs after irradiation. However, lack of diurnal variation of F persisted. Despite radiotherapy, five years later she developed clinical and biochemical evidence of mild recurrent Cushing's disease. Bone age, head CT, visual fields, and TRH, LHRH and L-DOPA/glucagon testing were all normal. Morning cortisol, 17-OHCS, and 6β-OHF were elevated and were partially suppressed only after high dose dex. The 8:00 PM ACTH level was elevated to 40 pcg/ml. After ovine CRF administration the maximum ACTH response was 46 pcg/ml; the ACTH concentration is increased and the absent ACTH response to CRF is abnormal. In patients with Cushing's disease treated with radiotherapy, the ACTH response to CRF stimulation may not be reliably compared to that of normal controls.
Conclusion: Therefore Cushing's disease may recur despite successful pituitary irradiation. This suggests a hypothalamic CRF producing lesion as the primary lesion in Cushing s disease.
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Dimartino, J., Stoner, E., Cappa, M. et al. 121 RECURRENCE OF CUSHING'S DISEASE AFTER PITUITARY DISEASE. Pediatr Res 19, 623 (1985). https://doi.org/10.1203/00006450-198506000-00141
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DOI: https://doi.org/10.1203/00006450-198506000-00141