title: Cushing's syndrome due to ectopic ACTH production by a nasal paraganglioma.
abstract: Ectopic secretion of ACTH is an infrequent cause of Cushing's syndrome. We report a case of ectopic ACTH syndrome caused by a nasal paraganglioma, a 68-year-old female with clinical features of Cushing's syndrome, serious hypokalaemia and a right paranasal sinus' lesion. Cranial magnetic resonance image showed a 46-mm mass on the right paranasal sinuses. Endocrinological investigation confirmed the diagnosis of ectopic ACTH production. Resection of the tumour normalised ACTH and cortisol secretion. The tumour was found to be a paraganglioma through microscopic analysis. On follow-up 3 months later, the patient showed nearly complete clinical recovery. Ectopic ACTH syndrome due to nasal paraganglioma is extremely uncommon, as only two other cases have been discussed in the literature.
  
  
  CONCLUSIONS
  Ectopic Cushing's syndrome accounts for 10% of Cushing's syndrome etiologies.Most paraganglioma of the head and neck are not hormonally active.Nasal paraganglioma, especially ACTH producing, is a very rare tumour.
authors.affiliation: Department of Endocrinology Hospital Egas Moniz, Centro Hospitalar Lisboa Ocidental Lisbon Portugal.
authors.forename: F
authors.initials: F
authors.lastname: Serra
authors.affiliation: Department of Endocrinology Hospital Egas Moniz, Centro Hospitalar Lisboa Ocidental Lisbon Portugal.
authors.forename: S
authors.initials: S
authors.lastname: Duarte
authors.affiliation: Department of Endocrinology Hospital Central do Funchal Funchal Portugal.
authors.forename: S
authors.initials: S
authors.lastname: Abreu
authors.affiliation: Department of Neurosurgery Hospital Egas Moniz, Centro Hospitalar Lisboa Ocidental Lisbon Portugal.
authors.forename: C
authors.initials: C
authors.lastname: Marques
authors.affiliation: Department of Pathology Hospital Egas Moniz, Centro Hospitalar Lisboa Ocidental Lisbon Portugal.
authors.forename: J
authors.initials: J
authors.lastname: Cassis
authors.affiliation: Department of Endocrinology Hospital Egas Moniz, Centro Hospitalar Lisboa Ocidental Lisbon Portugal.
authors.forename: M
authors.initials: M
authors.lastname: Saraiva