ACTH-dependent Cushing's syndrome: a revision of 43 cases.


  • Isabel Paiva Serviço de Endocrinologia, Diabetes e Metabolismo, Hospitais da Universidade de Coimbra, Coimbra.
  • Cristina Ribeiro
  • Leonor Gomes
  • Carla Baptista
  • Fernando Gomes
  • Manuel Rito
  • Olinda Rebelo
  • Dina Marnoto
  • Cristina Moura
  • Fátima Leitão
  • Manuela Carvalheiro



To evaluate the characteristics and outcomes of the patients diagnosed as ACTH-dependent Cushing syndrome, registered in the department.We reviewed the files of forty-three patients followed from 1974 to 2002.A progressive rise in the number of patients diagnosed was found, being 80% females. Clinical suspicion was based mostly on the typical fat distribution; hirsutism and amenorrhoea were important in women. The more reliable diagnostic tests were: 11 pm cortisol, day curve of ACTH and cortisol, and dexamethasone suppression tests. The ACTH response to CRH during inferior petrosal sinus sampling permitted the diagnosis of ectopic source. In thirty-seven patients a pituitary adenoma was diagnosed. The three patients diagnosed before 1985 went for bilateral adrenalectomy (Nelson's syndrome in two); the others were submitted to transsphenoidal pituitary adenomectomy, obtaining remission in twenty six at the first operation and in two others at the second. Three patients had a recidive. Of the six patients with persistent disease (all treated with metyrapone or ketoconazole), three were submitted to radiotherapy, two to bilateral adrenalectomy, and one was waiting for surgery. Four patients had a bronchial carcinoid, successfully removed in three. One patient was lost to follow-up and another was still being evaluated.We found a positive evolution in the capacity to diagnose and treat these patients. Neurosurgical ability to achieve remission was 80% in the operated cases. More effective technical methods and drugs, as well as a multidisciplinary and dedicated medical team, lead to long lasting remissions in most of the patients.


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How to Cite

Paiva I, Ribeiro C, Gomes L, Baptista C, Gomes F, Rito M, Rebelo O, Marnoto D, Moura C, Leitão F, Carvalheiro M. ACTH-dependent Cushing’s syndrome: a revision of 43 cases. Acta Med Port [Internet]. 2004 Oct. 31 [cited 2024 Jul. 25];17(5):367-74. Available from:



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