EU2019 Clinical Update Workshop D: Disorders of the adrenal gland (16 abstracts)
Good Hope Hospital, Birmingham, UK.
: 39Y old male who was originally treated as child with CAH secondary to 21-Hydroxylase deficiency and was put on glucocorticoids and mineralocorticoids from first week of life due to salt wasting crises. He has had very high 17-OH progesterone and adrenal androgen due to noncompliance. He developed polycythemia secondary to androgen excess, resulted in venesection. Haematologist have investigated the mutation and EPO measurement and he felt that this was probably secondary polycythemia. The main issue was that he has number of admissions with breathless and flank pain. He underwent abdominal ultrasound, which was suggestive of mass in left adrenal area and finding of bilateral multi-focal adrenal nodules with central low density changes likely to represent multiple adrenal myelipomas. The was concern about alternative diagnosis of liposarcoma. A CT scan reported of bilateral adrenal lesions and there were concerns as to liver metastases. Repeat scan did not show any liver lesion. He also has ultrasound of testes, which showed heterogenous masses in both testes and their appearance suggestive of testicular adrenal rests. Case was discussed in adrenal MDT and the consensus was that bilateral adrenalectomy in this scenario would not be advisable given his poor compliance on steroids and associated risk of life threatening adrenal crises. Patient was referred to surgical colleagues to discuss the possibility of a unilateral adrenalectomy in first instance, to determine a response in the symptoms. Patient underwent laproscopic left adrenalectomy and recuperating very well. Histopathology report awaited. He was back on his usual pre-surgery steroids dosage and conversant of sick day rules. He is due to follow up in adrenal clinic next month.