title: A rare case of ectopic ACTH syndrome originating from malignant renal paraganglioma.
abstract: Ectopic adrenocorticotropic hormone (ACTH) syndrome is characterized by hypercortisolism due to the hypersecretion of a non-pituitary ACTH-secreting tumor leading to Cushing's syndrome. Only a few cases have been reported previously as causing ectopic ACTH related to paraganglioma. Herein, we present a case of Cushing's syndrome, in who was proved to be attributable to an ACTH-secreting renal malignant paraganglioma. A 40-year-old woman presented with a five-month history of newly diagnosed hypertension and diabetes, weakness, hyperpigmentation, oligomenorrhea, hirsutism, and acneiform lesions. She showed cushingoid features, including moon face, facial hirsutism, facial and truncal acne, hyperpigmentation, and severe muscle weakness of the limbs. She did not have other findings such as striae, supraclavicular fat accumulation, and buffalo hump. Laboratory examination showed the presence of hypopotasemia, hyperglycemia, hyperthyroidism, and leukocytosis. The serum levels of ACTH, cortisol, and urine-free cortisol were markedly elevated. Results of an overnight 2-mg dexamethasone suppression test included a basal serum cortisol of 61.1 mcg/dL (normal range: 4.6-22.8 mcg/dL) and a cortisol value of 46.1 mcg/dL after dexamethasone administration. There was no suppression found after 2-day 8-mg dexamethasone administration. Magnetic resonance imaging (MRI) of the pituitary gland indicated two microadenomas. An abdominal MRI scan revealed horseshoe kidney, bilateral adrenal hyperplasia, and masses with dimensions of 35 x 31 mm in the left kidney. Inferior petrosal sinus sampling showed no evidence of a central-to-peripheral gradient of ACTH. A positron emission tomography/computed tomography scan showed intense increased activity in the lower pole of the left kidney. Left adrenalectomy and left partial nephrectomy were performed. The resected tumor was diagnosed as the ACTH-secreting paraganglioma in the pathological examination, which was confirmed by immunohistochemical studies with chromogranin A, synaptophysin, and ACTH. Only a few cases of paragangliomas as a cause of ectopic ACTH syndrome have been reported. To our knowledge, this is the first case of renal paraganglioma resulting in Cushing's syndrome due to ectopic ACTH hypersecretion.
authors.affiliation: Diskapi Yildirim Beyazit Teaching and Research Hospital, Department of Endocrinology and Metabolism, Irfan Bastug Caddesi, Ankara, Turkey.
authors.forename: Esra
authors.initials: E
authors.lastname: Tutal
authors.affiliation: Diskapi Yildirim Beyazit Teaching and Research Hospital, Department of Endocrinology and Metabolism, Irfan Bastug Caddesi, Ankara, Turkey.
authors.forename: Demet
authors.initials: D
authors.lastname: Yılmazer
authors.affiliation: Department of Endocrinology and Metabolism, Diskapi Teaching and Research Hospital, Irfan Bastug Caddesi, Ankara, Turkey.
authors.forename: Taner
authors.initials: T
authors.lastname: Demirci
authors.affiliation: Department of Endocrinology and Metabolism, Diskapi Teaching and Research Hospital, Irfan Bastug Caddesi, Ankara, Turkey.
authors.forename: Evrim
authors.initials: E
authors.lastname: Cakır
authors.affiliation: Department of Nuclear Medicine, Diskapi Teaching and Research Hospital, Irfan Bastug Caddesi, Ankara, Turkey.
authors.forename: Salih Sinan
authors.initials: SS
authors.lastname: Gültekin
authors.affiliation: Department of General Surgery, Diskapi Teaching and Research Hospital, Irfan Bastug Caddesi, Ankara, Turkey.
authors.forename: Bahadır
authors.initials: B
authors.lastname: Celep
authors.affiliation: Department of Endocrinology and Metabolism, Diskapi Teaching and Research Hospital, Irfan Bastug Caddesi, Ankara, Turkey.
authors.forename: Oya
authors.initials: O
authors.lastname: Topaloğlu
authors.affiliation: Department of Endocrinology and Metabolism, Diskapi Teaching and Research Hospital, Irfan Bastug Caddesi, Ankara, Turkey.
authors.forename: Erman
authors.initials: E
authors.lastname: Çakal
mesh_terms: D000182:ACTH Syndrome, Ectopic / Q000209:etiology* / Q000473:pathology; D000328:Adult; D003480:Cushing Syndrome / Q000209:etiology* / Q000473:pathology; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007680:Kidney Neoplasms / Q000150:complications* / Q000378:metabolism* / Q000473:pathology; D008207:Lymphatic Metastasis; D010235:Paraganglioma / Q000150:complications* / Q000378:metabolism* / Q000473:pathology; D010902:Pituitary Gland / Q000473:pathology; D000072078:Positron Emission Tomography Computed Tomography