Ectopic Cushing Syndrome: A Diagnostic and Therapeutic Challenge Síndrome de Cushing Ectópica: Um Desafio Diagnóstico e Terapêutico

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Vera Fernandes - Corresponding Author

Rua das Sete Fontes – São Vítor, 4710-243 Braga, Portugal

Ana Margarida Monteiro
Maria Joana Santos
Maria Lopes Pereira
Olinda Marques


INTRODUCTION: Ectopic Cushing syndrome is a rare disease and the identification of the adrenocorticotropic hormone (ACTH) producing tumour may occur years after Cushing syndrome diagnosis, hampering the therapeutic management of these patients.
CASE REPORT: A 78-year-old woman was referred to the Endocrinology department due to Cushing syndrome. She had signs and symptoms of hypercortisolism and the clinical investigation showed a dependent ACTH Cushing syndrome with a nor-mal pituitary magnetic resonance imaging, negative catheterization of the petrosal sinuses and an 8 mm pulmonary nodule. Scintigraphy with somatostatin receptors, PET-68Ga-SRP, (bronchofibroscopy) and bronchial aspirate cytology were normal. Due to clinical worsening, she started on metyrapone with a very favourable clinical and analytical response.
DISCUSSION: This clinical case was a diagnostic and therapeutic challenge. Although the location of the primary tumour was not found, control of hypercortisolism was achieved, improving the patient’s quality of life.

Keywords: ACTH Syndrome, Ectopic/diagnosis; ACTH Syndrome, Ectopic/therapy; Cushing Syndrome/diagnosis; Cushing Syndrome/therapy

Article Details

Fernandes V, Monteiro AM, Santos MJ, Pereira ML, Marques O. Ectopic Cushing Syndrome: A Diagnostic and Therapeutic Challenge: Síndrome de Cushing Ectópica: Um Desafio Diagnóstico e Terapêutico. Gaz Med [Internet]. 2018 May 25 [cited 2022 Jul. 2];5(1):51-5. Available from:

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