SFEBES2021 Poster Presentations Adrenal and Cardiovascular (45 abstracts)
Giant bilateral adrenal mylelolipoma in a patient with congenital adrenal hyperplasia
Sajnin Zaman 1 , Masato Ahsan 1 , David Lloyd 1,2 , Neil Bharadwaj 1,2 , Emma Bremner 1 , Mary Barrowcliffe 1 , Ragini C Bhake 1 , Manohara Kenchaiah 3 , Shailesh Gohil 1,2 , Miles J Levy 1,2 & Narendra L Reddy 1,2
1University Hospitals of Leicester NHS trust, Leicester, United Kingdom; 2University of Leicester, Leicester, United Kingdom; 3Northampton General Hospital, Northampton, United Kingdom
Introduction: Congenital adrenal hyperplasia (CAH) is an inherited autosomal recessive disorder characterized by insufficient production of cortisol and high androgens. Myelolipoma is a benign neoplasm of adrenal gland composing mature adipose tissue and scattered islands of hematopoietic elements. We report a case of rare association of giant bilateral adrenal mylelolipoma in a congenital adrenal hyperplasia patient who was non-compliant with medication.
Case report: A 45-year-old male with congenital adrenal hyperplasia (21-hydroxylase deficiency) diagnosed at birth presented with dyspnoea and severe abdominal discomfort from gross abdominal distension. No hypo-adrenal crises reported despite non-compliance. Past history included hypertension; no other family members have CAH. Computed tomography of abdomen showed hypo-enhancing fat containing large bilateral adrenal masses (Right 26.5x15.1 cm; Left 21.2 cm x 16.1 cm)) occupying most of the abdomen. Investigations: 17-OH-progesterone 172.48 nmol/l (1.8-6.65), Androstenedione 52.38 nmol/l (2.1-10.48), DHEAS 1.28 μmol/l (1.2-8.98), Testosterone 12.2 nmol/l (8.6-29), Plasma Renin level 230.58, Plasma Aldosterone 941 pmol/l (117-580).
Progress: Abdominal discomfort and dyspnoea resolved following open bilateral adrenalectomy, right adrenal weighed 5.8kg; left weighed 5.4kg. Histology revealed mature adipose tissue with scattered islands of haematopoietic tissue consistent with myelolipoma. Commenced on Prednisolone and Fludrocortisone and compliance reiterated.
Discussion: Most myelolipomas are unilateral, small and asymptomatic. Giant bilateral myelolipomas are rare especially in the context of CAH. Literature review reveals over 30 patients with this association. Whether non-compliance to CAH treatment has a role in aetiology of giant myelolipomas is not fully established. Cases of giant bilateral myelolipomas as initial presentation of CAH have been reported, thereby indicating non-compliance leading to chronic corticotropin stimulation as a potential risk factor.
Learning point: 1. Please consider CAH as a differential diagnosis in incidental bilateral adrenal masses. 2. Non-compliance to CAH treatment can potentially lead to abnormally huge bilateral adrenal masses necessitating bilateral adrenalectomy.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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