Endocrine Abstracts

Introduction: Cushing’s disease (CD) is a potentially fatal disease caused by corticotrophic adenoma of the pituitary gland. Crooke Cell Adenomas (CCA) are a rare variant characterized by cytoplasmic ring deposits of cytokeratin filaments. They are usually aggressive macroadenomas with a higher rate of recurrence and malignancy.

Clinical case: A 36 year old man, with 34 kg weight gain and uncontrolled hypertension for 4 years presented at the Emergency Department In September 2018 with new onset dyspnea for small exertion, orthopnea and paroxysmal nocturnal dyspnea, BP 201/152 mmHg, heart rate 135 bpm, normal cardiopulmonary auscultation and hepatomegaly. Laboratory workup: metabolic alkalosis, hypoxemia, normocapnia and hypokalemia, serum creatinine 3.9 mg/dl and a troponin rise from 262 ng/dl to 624 ng/dl (VR <34.2 ng/dl). ECG without changes. Acute heart failure was admitted with an AMI without ST elevation and acute kidney injury. Coronary angiography was postponed. Echocardiogram: concentric ventricular hypertrophy, LVEF 40% with lateral segmental hypokinesis. Fundoscopy: severe hypertensive retinopathy. In the ward, hypertension persisted despite 5 antihypertensive drugs and also refractory hypokalemia. When observed by the Endocrinologist it was noted his central obesity (IMC 36 kg/m2), moon face, thin skin, violaceous striae, and decreased proximal muscular strength. Laboratory tests: baseline cortisol and ACTH, 13.2 mg/dl and 57.7 pg/ml, respectively; Cortisol after DXM low dose–7.2 mg/dl and after high dose–7.2 mg/dl. Pituitary MRI: 7 mm posterior intrasellar lesion with slight lateralization to the right, probably cystic”. Inferior petrosus sinus catheterization: basal ACTH central/ peripheral gradient of 33.2 and after CRH stimulation 40. Metyrapone 750mg/day was instituted, with progressive improvement in blood pressure control.

Submitted to endoscopic transphenoidal surgery with removal of the pituitary lesion without complications. Serum cortisol 17 h after intervention–0.7 mg/dl. Discharged under hydrocortisone 40 mg, amlodipine 10 mg, carvedilol 25 mg and enoxaparin 40 mg/day, with controlled arterial tension and stable renal dysfunction. Histology: solid large cell tumor with abundant cytoplasm, without atypia. ACTH + on the surface and ring cytokeratin in the cytoplasm; Ki67 <3% - Crooke Cell Adenoma. Over the next 15 months, progressive improvement in blood pressure control and kidney function. Hidrocortisone was stopped on the 13 month, and he remains without biochemical evidence of hypercortisolism

Discussion: This case illustrates the serious consequences of prolonged endogenous hypercortisolism. The posterior location of the pituitary lesion raised doubts about its causal relationship with CD and the best neurosurgical approach. Albeit in remission, a careful follow up is indicated, namely considering the histologic type of the corticotrophic adenoma.