Endocrine Abstracts

In October 2006 a 53-year-old, non-smoking woman presented with ectopic Cushings from a small cell, undifferentiated carcinoma (Grade 3 neuroendocrine) from a 10 mm tumour at the left lung hilum (Cortisol: 09:00 a.m. 720 nmol/l; 24.00 mm 742 nmol/l. ACTH 114 ng/l). Four years before she had sustained a myocardial infarction, with atrial fibrillation, and was found to have Graves’ disease, being treated initially with carbimazole then radioactive iodine, becoming hypothyroid.

She received four courses chemotherapy (cisplatin and etopiside); radiotherapy (45 Gy to left hilum and 30 Gy to brain) and metyrapone, with both radiological and biochemical remission allowing withdrawal of metyrapone in June 2007. However, she had a biochemical relapse in October 2007 (Cortisol 992; ACTH 142) and restarted metyrapone. Although a CT chest remained unchanged she had a positive pentreotide scan but ACTH levels didn’t suppress with intravenous Octreotide. She declined adrenalectomy.

In August 2008 she presented with right facial numbness, visual field defect and right hemiparesis. MRI brain scan showed three cerebral infarcts and other ischaemic areas. She was commenced on dexamethasone 2 mg daily. Three weeks later she became unrousable and sustained a left hemiparesis. New haemorrhagic basal ganglia infarcts were demonstrated on MRI scan consistent with a paraneoplastic cerebral vasculopathy. She received 16 mg dexamethasone with a rapid recovery. On this therapy she rapidly became severely cushingoid with proximal myopathies. Dexamethasone was stopped 7 weeks later.

A further radiological and biochemical relapse occurred in August 2009, followed by another right occipital cerebral infarct; she has received further chemotherapy.

This case demonstrates iatrogenic Cushings superimposed on ectopic Cushings syndrome, with tumour uptake of pentreotide offering a possible means of pentreotide-linked therapy.