Endocrine Abstracts

30 M referred to the Surgical Same Day Emergency Care (SDEC) clinic with 2-month history of abdominal pain to left loin and RUQ and worsening back pain for 1-week. Abdomen was soft with tenderness to the RIF, LIF and bilateral loin area. Bloods demonstrated a CRP of 210, sodium 145, potassium 3.2, creatinine 63, ALP 572, ALT 73, bilirubin 12 with normal WCC and neutrophils. A CT abdomen pelvis demonstrated a large left adrenal 10 cm × 11 cm × 13 cm solid mass with some central necrosis, which extended to the left renal vein, infrahepatic IVC and L2 lumbar vein, and metastases to the liver, spine, pelvis and sternum. A history of weight gain and perceived facial puffiness was elicited. Further examination demonstrated Cushingoid facies, purple abdominal striae and elevated blood pressure of around 180/100 mmHg. Initial biochemistry demonstrated a random cortisol of 1300 and low potassium. During work up including biochemistry, liver biopsy and MDT, pain was the primary issue for our patient. The palliative care team were involved for analgesia and lead up-titration of opioid, amitriptyline and radiotherapy for bony lesions. CT chest demonstrated a likely metastatic right lung nodule and thoracic spinal metastases. Biochemistry revealed: LH <0.3, FSH 0.5, Testosterone 5.8, TSH 0.69, Free T4 5.8, Cortisol 1388, LDH 968, ACTH <3.8. Plasma testing ruled out a phaeochromocytoma. Urine steroid profile showed increase in metabolites of 11-deoxycortisol, DHEA, cortisol, progesterone, 17-hydroxyprogesterone alongside pregnenetriol and tetrahydro-11-deoxycortisol. Metyrapone 250 mg TDS was commenced and subsequent testing demonstrated a drop in cortisol to 550 (1388) after three days. During admission, this patient became intermittently pyrexic, tachycardic and developed a new oxygen requirement and was diagnosed with a Klebsiella bacteraemia of unclear source. Liver biopsy demonstrated findings consistent with adrenocortical carcinoma, however it was noted that GATA3 was unusually positive and synaptophysin was very weak. Sadly, this died unexpectedly overnight with a cause of death attributed to 1a) Klebsiella Bacteraemia 1b) Metastatic Adrenocortical Carcinoma (ACC). Full work up was not possible (e.g. 24 hour urine free cortisol, dexamethasone suppression test or salivary cortisol), however results including urine steroid profile, supressed ACTH and response to Metyrapone established good proof of cortisol excess and evidence towards a diagnosis of ACC. This case illustrates highly aggressive ACC and highlights a presentation that includes florid Cushing’s and non-specific symptoms secondary to direct organ invasion by tumour growth as well as more distant metastases.