Endocrine Abstracts

Introduction: Adrenocortical carcinoma is a very rare tumor with a heterogenous prognosis.

Case report: Patient R.B is a 58-year-old female patient with no particular familial history. She had a personal history of hypertension, discovered 6 months ago well controlled with beta blockers and amlodipine. Two weeks before her admission to the hospital, she reported having constantly worsening abdominal pain followed by frequent vomiting few days later. The patient consulted a gastroenterologist and an abdominal CT was performed. It revealed the presence of a massive 12×11 cm heterogenous right adrenal mass, invading the IV segment of the liver along with the inferior cavernous vein. She also had bilateral and proximal pulmonary embolism with a right thrombus in her right atrium from tumor extension and suggestive signs of pulmonary metastasis on thoracic CT. Upon her admission in the intensive care unit, clinical examination revealed excessive hirsutism notably in her face and deepening of her voice. Those symptoms were insidious and neglected by the patient for 5 months. She had no evident clinical signs of Cushing syndrome in spite of cutaneous fragility. Laboratory exams showed cytolysis and persistent biological inflammatory syndrome despite the absence of infection. She had no hypokalemia. Hormonal assessment revealed normal Metanephrines and preserved cortisol nychthemeral rhythm as well as urinary free cortisol level. However, she had an extremely high testosterone level: 117 ng/ml, increased dehydroepiandrosterone sulfate: >27 µmol/l (normal level < 6.6) and high 17 alpha hydroxy-progesterone. Few days later, the patient deceased from cardiac arrythmia.

Discussion: Pulmonary embolism in the setting of adrenocortical carcinoma is a rather rare finding. Hypercoagubality associated with Cushing syndrome, cancer thrombi and direct tumor invasion are the pathophysiological mechanisms explaining this finding. The latter hypothesis is the most likely to be the cause of embolism in our case. Albeit, non-secreting adrenal cancer are the most incriminated, our report suggest otherwise.