Endocrine Abstracts

Case summary: A 40 year-old lady, who has a background of asthma, presented to emergency department with back pain radiated down to her left leg with associated foot drop. She also complained of 2 months history of weight gain, hirsutism, and menstrual disturbance. Clinical examination confirmed that she had some cushingoid features. Further assessment and investigations revealed that she had acute disc prolapse on MRI as well as an incidental finding of right adrenal gland and some indeterminant lesions in the iliac bones. Further CT-TAP confirmed that she had a large tumour in the right adrenal gland, 7.4 x 12.2 cm, highly suspicious of malignancy with multiple pleural-based intrathoracic nodules suggesting metastases. Following discussion at the adrenal MDT, further investigations such as PET CT, adrenal biopsy and secretion studies which were arranged on the back of high suspicion for adrenal cortical carcinoma (ACC). Secretion studies confirmed hyperandrogenism with high androstenedione at 25.2 nmol/l(1.0-8.5 nmol/l), testosterone level 4.0 nmol/l(<1.9 nmol/l), DHEAS >27 (0.3-12.0 umol/l). Cortisol was 728nmol/lwith ACTH of 0.7 pmol/l. Rest of the biochemistry, renin and aldosterone ratio and plasma metadrenalines were unremarkable. PET scan showed high uptake in the right adrenal mass with some areas of calcification in keeping with malignancy, multiple pleural nodules bilaterally consistent with metastatic lesions and no evidence of definite FDG avid destructive bone lesion. US guided biopsy confirmed an adrenal cortical origin Systemic chemotherapy was started for ACC under Oncology service and genetic screening was organised.

Conclusion: ACC tumours are rare, but often aggressive in nature. It is important to have high index of suspicion for these lesions whenever it is relevant like in this case. ACC could be functional and cause Cushing’s syndrome and/or virilization, or non-functional and could present with manifestations related to tumour growth (i.e. abdominal or flank pain). It is not uncommon for these lesions to present as incidentaloma especially if the subtle clinical signs are overlooked in an otherwise fit and young patient like ours. This also highlights the importance of having a dedicated adrenal MDT service for these patients through which investigations and referrals to appropriate specialties could be arranged without any undue delay and timely treatment could be initiated.