SFEEU2022 Society for Endocrinology Clinical Update 2022 Workshop D: Disorders of the adrenal gland (17 abstracts)
64 y/o male was being investigated for raised PSA. Following an MRI and biopsy, he was diagnosed with high grade prostatic intraepithelial neoplasia requiring close PSA surveillance. Incidentally, he was found to have bilateral adrenal lesions, measuring 6.4 cm on the left and 4 cm on the right. Prior to review in endocrine clinic, adrenal workup and CT adrenals were performed. CT showed both adrenal masses contain fat and calcification and are thought to be bilateral adrenal myelolipomas. Interestingly, adrenal workup, showed significantly raised l 17OH-progesterone (17OHP) at >300 nmol/l (1.2-5), raised renin level at 175.6mU/l (5.4-30), and ACTH of 140ng/l (0.0-5.0). From the history, he has been married twice though has never had any children, mainly due to personal circumstances rather than the inability to father a child. He has always been sexually active. He is totally asymptomatic from endocrine point of view and he is not on any medication. On examination he had normal secondary sexual characteristics. His right testis was significantly enlarged and apparently this has always been the case. It is non-tender. Short synacthen test revealed exaggerated 17OHP response (364 nmol/l at 0min and 718 nmol/l at 30min) with blunted cortisol response (110 nmol/l at 0 min and 104 nmol/l at 30 mins), confirming congenital adrenal hyperplasia (CAH) with glucocorticoid deficiency. Repeat renin level remains significantly elevated at 213.5mU/l suggesting mineralocorticoid deficiency. USS testes showed large lobulated right testes with an irregular complex calcific mass (31 x 38 x 38mm) with smaller lobulated left testes with diffusely heterogenous echotexture at the lower pole raising suspicion of whether these are testicular adrenal test tumors (TART). He has been commenced on glucocorticoid and mineralocorticoid replacements. Multidisciplinary team decided on conservative management for the adrenal myelolipomas, with yearly CT surveillance. In summary, this is an interesting case of middle-age male who was incidentally found to have large bilateral adrenal myelolipomas, a rare benign adrenal tumor. The association of adrenal myelolipomas with late-diagnosed and poorly managed CAH has been documented in number of studies and case reports. Despite being asymptomatic, biochemical work-up confirmed diagnosis of CAH with glucocorticoid and mineralocorticoid deficiency necessitating treatment which is uncommonly seen in non-classical CAH, as most of them does not require treatment. The US findings also raised suspicion of TART, which rarely reported in non-classical type.