Ectopic Cushing syndrome in Colombia

ABSTRACT Objective The aim was to describe the clinical features of patients with ectopic Cushing syndrome (ECS) from Colombia and compare these findings with other series to provide the best management for these patients. Materials and methods Records of patients with ECS from 1986 to 2017 were retrospectively reviewed; patients with a diagnosis of adrenal or pituitary Cushing syndrome (CS) were excluded. Results Fourteen patients with ECS were analyzed in this study. The mean age was 54.4 (SD 17.1) years, and the female to male ratio was 1.33:1. Regarding the etiology of ECS, four patients had lung carcinoids (28.6%), three had small-cell lung carcinoma (21.4%), three had pancreatic neuroendocrine tumors (21.4%), one had medullary thyroid cancer (7.1%), one had non-metastatic pheochromocytoma (7.1%), one had metastatic thymoma (7.1%) and one patient had an occult source of ACTH (7.1%). The most common clinical features at presentation were moon-face, muscle weakness, diabetes mellitus and hypertension. Hyperpigmentation was present in 36% of patients, and 12 patients had hypokalemia with a mean value of 2.3 mEq/L (SD 0.71). The median basal cortisol, 24-hour urinary free cortisol (UFC) and ACTH were 30.5 ug/dL (IQR 21-59 ug/dL), 2,600 ug/24 h (IQR 253-6,487 ug/24 h) and 91 pg/mL (IQR 31.9-141.9), respectively. Thirteen patients (92.8%) had the site of the primary lesion identified. Six patients had undergone a surgical intervention to address the primary tumor. Resection was curative in 28.5% of patients. Death occurred in 57.1% of patients, and the median overall survival was 27 months. Intrathoracic tumors had the most aggressive behavior. Conclusions ECS is a rare disease; however, it is associated with high morbidity and mortality. A rapid intervention supported by an interdisciplinary group is required to improve overall survival and quality of life

Severe hypertension and hypokalemia as first clinical manifestations in ectopic Cushing's syndrome / Hipertensão grave e hipocalemia como primeiras manifestações clínicas em casos de síndrome de Cushing ectópica

Ectopic ACTH production occurs in about 10 percent of all cases of Cushing's syndrome, and about 25 percent of cases of ACTH-dependent Cushing's syndrome. Diverse tumor types are able to produce ACTH ectopically, including small cell lung carcinoma. Ectopic ACTH secretion by malignant neoplasm has been reported to have earlier and more aggressive metabolic effects. We report a 59-year-old male patient with severe hypertension, metabolic alkalosis and hypokalemia as the first clinical manifestations of an ACTH-secreting small cell lung carcinoma, although the typical phenotypic features of Cushing's syndrome were not present. Ectopic Cushing's syndrome should always be ruled out in patients with severe hypertension and hypokalemia.

A produção de ACTH ectópico ocorre em aproximadamente 10 por cento dos casos de síndrome de Cushing, e em aproximadamente 25 por cento dos casos de síndrome de Cushing dependentes de ACTH. Diversos tipos de tumores são capazes de produzir ACTH ectopicamente, incluindo carcinoma pulmonar de células pequenas. Relatórios indicam que a secreção de ACTH ectópico por neoplasma maligno causa efeitos metabólicos prematuros e mais agressivos. Apresentamos um paciente, 59 anos, com hipertensão grave, alcalose metabólica e hipocalemia, tendo estas como as primeiras manifestações clínicas de um carcinoma pulmonar de células pequenas com secreção de ACTH, embora as características fenótipas típicas da síndrome de Cushing não estavam presentes. A síndrome de Cushing ectópica deveria ser excluída sempre em pacientes com hipertensão grave e hipocalemia.

A Case of Cushing's Syndrome Associated with Ectopic ACTH Production in Patient with Small: cell Lung Cancer / 대한내과학회지

About 5% of patients with SCLC have the ectopic production of ACTH. Ectopic ACTH production of SCLC is suspected when patients of SCLC have unexplained metabolic alkalosis and hypokalemia. Most patients lack the classic feature of Cushing's syndrome. According to the recent report, they have poor prognosis, which median survival is less than 4 months and associated with a high rate of complication during chemotherapy. Also a case of paraneoplastic CRH production with SCLC can mimic the ectopic ACTH syndrome hut it can be distinguished by immunohistochemistry or direct measurement of serum CRH level. We report here a case of small cell lung cancer associated with Cushing's syndrome of ectopic ACTH production. That is immunohistochemically provened by staining with ACTH.