Spontaneous remission in Cushing’s disease: A case report

Abstract

Spontaneous remission of Cushing disease might be a phase of cyclic disease, but could also be explained by an ACTH-microadenoma infarction or hemorrhage. True remission must be differentiated from cyclic Cushing’s disease with prolonged follow up. 63-years-old female patient was referred to endocrinologist’s and presented with uncontrolled hypertension, type 2 diabetes, central obesity, hirsutism, swelling of the face and legs and a ‘buffalo hump’. After additional investigation elevated ACTH (24.1pmol/l (n1.63-14.15)), basal cortisol (1187nmol/l (n147-726)) and high 24-hours urinary free cortisol 2236.4nmol/l (n138- 524) were determined. Cortisol was not suppressed after low-dose dexamethasone suppression test. Pituitary MRI revealed 0.5x0.2x0.3cm hypointense zone in posterior pituitary. Petrosal sinus sampling was planned, but the patient was admitted to the emergency department with panniculitis in her limbs. After chest and abdominal CT scan 3.1x2.4cm tumor in left ventricle and 0.6cm hypointense tumor in the liver was detected. Patient case was discussed at multidisciplinary team and it was concluded that at that moment biopsy benefit would not outweigh the risks. Patient was treated with iv a/b. When inflammation markers decreased petrosal sinus sampling was performed. It confirmed pituitary source of ACTH production and right side lateralization which coincided with MRI data. During two months period from the first suspicion of pituitary microadenoma till the petrosal sinus sampling was performed, clinical symptoms of Cushing syndrome significantly decreased as well as hormone levels: basal cortisol level 1187->403.16nmol/l, ACTH 24.1->7.1pmol/l. Diabetes control significantly improved as well. The situation was interpreted as spontaneous remission and it was decided to continue active observation of the patient. However, 10 months later worsening of patient’s status was observed. Basal cortis[...].