A 32-year-old man was referred to our surgical unit with a left adrenal lesion. He was previously diagnosed with Birt-Hogg-Dube syndrome after presenting with a left pneumothorax and an incidental finding of a right apical lung mass. This syndrome is characterised commonly by benign skin hamartomas, recurrent pneumothoraces and an increased risk of renal tumours. He was unable to tolerate a biopsy of his lung lesion, however, this lung lesion was thought to be benign. Given the size of his adrenal lesion and radiological appearances, we performed a laparoscopic left adrenalectomy. Pathology confirmed that based on morphological appearances and immunohistochemical staining, this may represent an oncocytic tumour of the adrenal gland. This is only the third published case of an oncocytic tumour of the adrenal gland in a patient with Birt-Hogg-Dube syndrome.
- adrenal disorders
- genetic screening / counselling
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Contributors CW involved in the conception of this case report and a subsequent review of the literature concerning Birt-Hogg-Dube (DHB) syndrome. She also assisted in the writing up of the text. JR involved in the writing up of the text, obtaining consent from the patient and the imaging. He also performed a literature review on BHD syndrome.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.