Endocrine Abstracts

Introduction: Endogenous cortisol secretion should cease after bilateral adrenalectomy (BAL) and steroid hormone replacement is life sustaining at the regular physiologic dosage. However, there are reports of occurrence of functioning adrenal tissue in the setting of BAL treatment for ACTH dependent Cushing’s syndrome and not the other known indications for BAL.

Case report: A 44-year-old lady with functioning adrenal tissue after BAL for Hereditary pheochromocytoma-paraganglioma (PGL/PCC) syndrome with germline SDHD mutation. She underwent right adrenalectomy in 1984 and left adrenalectomy 20 years later. She currently has bilateral jugular paraganglioma, right vagal paraganglioma and right carotid body tumor. Her other medical history includes familial X-linked hypophosphatemic vitamin D resistant ricket. She has always been on low dose of maintenance hydrocortisone (HC) 5/2.5/2.5 mg daily and fludrocortisone 25 mg every third day. Her surveillance whole body MRI and Gallium Doctatate scans confirmed paraganglioma but did not demonstrate residual adrenal tissue or ectopic adrenal. Biochemistry confirmed residual cortical functions as shown below.

Conclusion: Although, there are reports of increased catecholamines influencing adrenal steroidogenesis in pheochromocytoma but this is not reported in the absence of adrenal tissue. Cortisol production could be from microscopic adrenal remnant, residual or adrenal rest from an accessory adrenal gland which is yet unidentified in this patient. To our knowledge, this is the first case of functioning adrenal tissues after BAL in the setting of PGL/PCC syndrome and highlights the fact that other factors apart from ACTH could stimulate adrenal tissue formation after BAL.