Testicular adrenal rest tumour in an adult patient with congenital adrenal hyperplasia: a case report and review of literature

Late presentations of testicular adrenal rest tumours (TART) are rarely seen in males with congenital adrenal hyperplasia, and are often misdiagnosed as primary testicular germ cell tumours. We report a case of bilateral TART in an adult patient with congenital adrenal hyperplasia who had defaulted treatment. He presented with a gradually increasing swelling of the left testis and genital examination revealed a large hard indurated swelling of both epididymes more prominent on the left side. As imaging was inconclusive, a biopsy was performed which showed features in favour of TART rather than Leydig cell neoplasm and he had good response to steroids. Histopathological evaluation is helpful in cases where there is a strong need for excluding a malignancy. Optimal steroid replacement is the treatment of choice and leads to regression of the lesion in the majority.