Ectopic cushing's syndrome caused by a pulmonary acth-secreting tumor in a patient treated with octreotide [Síndrome de Cushing ectópico causado por um tumor pulmonar secretor de ACTH em tratamento com octreotide]

• Type: Article in International Scientific Journal
• Date: 2012

Authors

Rodrigues, P (Author) Other; Castedo, JL (Author) Other; Damasceno, M (Author) Other; Carvalho, Davide (Author) FMUP

Journal

Title: Arquivos Brasileiros de Endocrinologia e Metabologia

Vol. 56

Pages: 461-464

ISSN: 0004-2730

Publisher: Universidade de São Paulo

Indexing

ISI Web of Knowledge - 5 Citations

Scopus - 7 Citations

Other information

Authenticus ID: P-008-7B3

DOI: 10.1590/s0004-27302012000700009

Abstract (EN): Ectopic ACTH syndrome is a rare disease often associated with severe hypercortisolism. When feasible, optimal management is surgical excision of the tumor. A 33-year-old male patient was admitted to the hospital in 1993 with clinical manifestations suggestive of Cushing's syndrome. He presented high plasma ACTH and markedly elevated urinary free cortisol excretion that was not suppressed with high-dose dexamethasone administration. Pituitary MRI scan was normal. No central-to-peripheral ACTH gradient was present in bilateral inferior petrosal sinus sampling. Thoracic CT scan showed a 1.7 cm nodule at the left lung. Pulmonary fine needle cytology and immunocytochemical and ultrastructural studies, together with the presence of bone metastases, led to the diagnosis of an ACTH-producing neuroendocrine carcinoma. He was initially submitted to chemotherapy and has been on treatment with octreotide LAR since 1998, having shown a favorable clinical, biochemical and imaging response. We highlight the excellent longterm response to medical therapy with octreotide LAR, without tachyphylaxis, probably due to its antiproliferative effect.© ABE&M todos os direitos reservados.

Language: English

Type (Professor's evaluation): Scientific

No. of pages: 4