A 39 year old female patient presented with one week history of Shortness of breathing. Over the last four months she noted bilateral leg swelling, which failed to improve on diuretic. Examination revealed no other evidence of fluid overload or heart failure. An element of right heart strain rather than failure was raised on cardiologists review. Subsequently, an echo showed a friable mass extending form inferior vena cava to right atrium into proximal left ventricle. The nature of which was revealed to be a tumor thombus extending from the upper pole of right kidney with resultant pulmonary emboli. To this end, an impression of invasive renal carcinoma was made. Until endocrinology review for erratic hyperglycemia, when the patient reported irritability, 6 weeks amenorrhea, change in weight distribution and facial hirsutism in addition to abdominal striae on examination. Hence,the diagnosis shifted to Adrenocortical carcinoma with Cushing. Tertiary center was consulted. In the meantime, Mitotane was started gradually, alongside Hydrocortison. It was a difficult time for the patient who preferred palliative support at home. Unfortunately, few days after discharge she was readmitted with hypoglycemiia, metabolic acidosis and liver impairment. She was treated as Addison crises, though cortisol levels came normal later. Liver involvement was not confirmed to be Mitotane related toxicity or ischemic injury with progression of malignancy.
Unfortunately, she did not survive his well-known aggressive tumor. ACC are tumors are rare tumors and presentation with such vascular invasion is an additional rarity. As clinician on medical take, there is tendency to overclaim heart failure. Having high index of suspicion for patients with Cushing syndrome is central, although not all tumors are functional. The definitive management of ACC is surgical which is not always feasible, particularly with such vascular extension. Wider scale clinical studies pave the management of ACC and optimize Mitotatne therapy.
20 - 23 May 2017
European Society of Endocrinology