ECE2017 Eposter Presentations: Pituitary and Neuroendocrinology Clinical case reports - Pituitary/Adrenal (41 abstracts)
Ectopic ACTH syndrome presenting with partial loss of conciousness and nonconvulsive status epilepticus in a patient with no signs of Cushing syndrome
Eray Onur Mert 1 , Samet Yaman 1 , Didem Ozdemir 2 , Nagihan Bestepe 3 , Dursun Ali Saglam 4 , Osman Ersoy 5 , Reyhan Ersoy 2 & Bekir Cakir 2
1Ankara Yildirim Beyazit University, School of Medicine, Department of Internal Medicine, Ankara, Turkey; 2Ankara Yildirim Beyazit University, School of Medicine, Department of Endocrinology and Metabolism, Ankara, Turkey; 3Ankara Ataturk Education and Research Hospital, Department of Endocrinology and Metabolism, Ankara, Turkey; 4Ankara Ataturk Education and Research Hospital, Department of Internal Medicine, Ankara, Turkey; 5Ankara Yildirim Beyazit University, School of Medicine, Department of Gastroenterology, Ankara, Turkey.
Introduction: Ectopic adrenocorticotrophic hormone (ACTH) syndrome is associated with variable tumor groups most commonly originating from neuroendocrine cells. Here, we reported a patient considered to have nonconvulsive status epilepticus initially due to neurological symptoms and diagnosed as neuroendocrine tumor with ectopic ACTH syndrome.
Case: A 85 years old woman with regulated hypertension admitted with partial loss of conciousness. She had cachectia and body mass index was 16.7 kg/m2. There was no acute pathology in cranial imaging. Because electroencephalography revealed findings compatible with nonconvulsive status epilepticus, levatiresatam was started. In laboratory examination, she had hypokalemia (2.5 mg/dl) resistant to i.v. replacement. Her serum cortisol was 126 μg /ml (5–20 μg/ml) and ACTH was 331.7 pg/ml (0–60 pg/ml). Hypophysial MRI showed a 6×3 mm lesion in right hypopysis. She did not any physical signs of Cushing syndrome. In thoracal CT, a 16 mm solid irregular lesion extending to the parencyma and costal pleura in right lung was observed. Abdominal CT revealed a 125 mm heterogenous mass and satellite lesions in the liver and hypertrophic adrenal glands. A tru-cut biopsy from the lesion was reported as metastasis of neuroendocrine tumor. The lesion was diffusely positive for TTF-1, cytokeratin 7, synaptophysin and chromagranin. Ki67 proliferation index was 10–15% and primary tumor was suggested to be atypical carcinoid tumor of lung according to the immunohistopathological findings. The patient died at the 20th day of intensive care unit due to sepsis.
Conclusion: Ectopic ACTH syndrome is a rare cause of ACTH dependent Cushing syndrome. It can be easily included in the differential diagnosis in a patient with classical signs of Cushing syndrome and without adenoma in hypophysis. However, like ours, well-known physical appearance and signs of Cushing syndrome might not be seen in all patients, and the only clinical presentation might be neurocognitive dysfunctions.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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