Functioning Adrenocortical Carcinoma with Extension upto the Right Atrium Producing Cushing's Syndrome
A 40-year-old female presented with a 7-month history of intermittent right flank pain in the loin region and features of Cushing's syndrome such as weight gain, central obesity, bipedal edema, moon facies, hirsutism, oligomenorrhea, recent onset of hypertension and diabetes mellitus (treated with insulin). There was no history of persistent headache, vomiting, and focal neurological symptoms. Her electrolytes, renal and liver function tests were within normal limits. Hormonal profile testing revealed markedly elevated serum 8 AM cortisol (1177 nmol/L) with loss of diurnal variation (11 PM cortisol - 800 nmol/L) which was not suppressible by Dexamethasone (LDDST {low dose dexamethasone suppression test} - 917.1 nmol/L; HDDST {high dose dexamethasone suppression test}- 812.9 nmol/L). Adrenocorticotropic hormone (ACTH) was suppressed (1.89 pg/mL), serum testosterone was elevated (6.3 nmol/L) with suppressed luteinizing hormone (LH) 1.14 mIU/mL (normal in follicular phase 2.4-12.6mIU/L) and follicle-stimulating hormone (FSH) 1.15 mIU/mL (normal in follicular phase 3.3-12.5 mIU/L). Ultrasound abdomen revealed a hypoechoic lesion in the right lobe of liver of size 10.1 × 8.2 cm. On contrast enhanced computed tomography (CECT) abdomen there was a well-defined heterogeneously enhancing mass lesion 10 × 9 cm with necrotic areas in right suprarenal location with infiltration into the segment VI and V of the liver and tumor thrombus in the IVC reaching up to the right atrium []. Left adrenal gland was normal. positron emission tomography (PET CT) with computed tomography scan showed intense FDG uptake {standardized uptake values} (SUV) max 8.6) [].\nThe patient underwent a bilateral subcostsal incision with midline sternotomy for excision of tumor mass with removal of intracaval and right atrial extension for which she required a cardiopulmonary bypass. Tumor planes with the inferior surface of the liver were lost and during resection there was liver parenchymal injury from where she had severe bleeding. Packing of the abdominal cavity and abdomen closure had to be done with plans for pack removal 48 hours later, by which time bleeding had subsided. Specimen excised revealed complete removal of tumor with the thrombus []. Post-operation the patient was kept on a ventilator and inotropic support, which was gradually withdrawn. Histopathology of the specimen confirmed the diagnosis of adrenocortical carcinoma with tumor thrombosis [].