Incidental diagnosis of Bilateral Adrenal Myelolipomas in a patient with Congential Adrenal Hyperplasia results in enhanced adherence to treatment, with limited evidence to guide management

Rumaisa Banatwalla; Anna Crown

doi:10.1530/endoabs.69.P51

Introduction: Adrenal myelolipomas are rare benign tumours consisting of fat and haematopoietic elements. We present an adult male patient with congenital adrenal hyperplasia (CAH) with an incidental diagnosis of bilateral adrenal myelolipomas, resulting in an improvement in adherence to treatment, but uncertainty regarding his future management.

Case history: 53 year old man with known CAH was referred into the endocrine clinic, historically requiring large doses of fludrocortisone and hydrocortisone. Following an admission with adrenal crisis and acute kidney injury, an abnormal renal tract ultrasound scan (USS) result (Jan 2018) prompted a follow-up CT scan (Mar 2018). This showed incidental findings of bilateral adrenal myelolipomas, 3.6 cm (right) and 9–10 cm (left). It was decided to monitor the myelolipomas annually using ultrasound imaging rather than CT, as the larger tumour could be visualised by USS and this would limit radiation exposure. Compliance with his prescribed medication was encouraged, in the hope that this might reduce the risk of the myelolipomas getting bigger.

Investigation results: The results summarised in the Table (1) evidence enhanced adherence to treatment following the abnormal USS and CT imaging results (January and March 2018). However, his most recent USS (December 2019) suggests a slight increase in the size of the myelolipoma on the left to 12 cm.

Table 1 Evidence of enhanced adherence to treatment following the abnormal USS and CT imaging results (January and March 2018).
Date	Renin (mcg)	Potassium	Fludrocortisone dose	Hydrocortisone dose (mg)
May 2017	3340	4.9	200 increased to 300	20 plus 10
April 2018	445	4.0	300	20 plus 10
Oct 2018	53	3.1	Reduced to 200	Reduced to 15 plus 5
Nov 2019	36.6	3.3	Reduced to100	15 plus 5
(Reference ranges: Renin: Erect 5.4–60 mU/l, Supine 5.4–30 mU/l; Potassium: 3.5–5.1 mmol/l)

Conclusion and points for discussion: The diagnosis of adrenal myelolipomas in this case was based on the characteristic imaging appearance in the context of adult CAH. Since the diagnosis, our patient’s compliance with his medication has improved, but despite this there is evidence of slow growth of the myelolipoma with time. The evidence base to guide the management of adrenal myelolipomas is limited and based on case reports and opinion. Usually adrenal myelolipomas can be followed without surgical excision. There are no reports of malignant change. However there are reports of spontaneous rupture and haemorrhage, and the risks may increase with the size of the tumour.

Endocrine Abstracts

P51