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Diagnosis and Treatment > Signs and Symptoms

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F Serra Department of Endocrinology, Hospital Egas Moniz, Centro Hospitalar Lisboa Ocidental, Lisbon, Portugal

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S Duarte Department of Endocrinology, Hospital Egas Moniz, Centro Hospitalar Lisboa Ocidental, Lisbon, Portugal

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S Abreu Department of Endocrinology, Hospital Central do Funchal, Funchal, Portugal

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C Marques Departments of Neurosurgery

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J Cassis Pathology, Hospital Egas Moniz, Centro Hospitalar Lisboa Ocidental, Lisbon, Portugal

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M Saraiva Department of Endocrinology, Hospital Egas Moniz, Centro Hospitalar Lisboa Ocidental, Lisbon, Portugal

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Summary

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.

Learning points

  • 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.

Taxonomy:
Clinical Overview, Gland/Organ, Adrenal, Topic, Endocrine-related cancer, Hormone, ACTH, Condition/ Syndrome, Cushing's syndrome, Ectopic ACTH syndrome, Hyperglycaemia, Hypokalaemia, Hypothyroidism, Paraganglioma, Paranasal sinus lesion, Patient Demographics, Age, Adult, Gender, Female, Ethnicity, White, Country of Treatment, Portugal, Diagnosis and Treatment, Signs and Symptoms, Hearing loss, Hyperglycaemia, Immunodeficiency, Oedema, Polydipsia, Polyuria, Investigation, ACTH, Cortisol (serum), MRI, Medication, Ketoconazole, Octreotide, Potassium chloride, Spironolactone, Related Disciplines, Oncology, Otolaryngology, Pathology, Publication Details, Case Report Type, Unique/unexpected symptoms or presentations of a disease
DOI:
https://doi.org/10.1530/EDM-13-0038
Volume/Issue:
Volume 2013: Issue 1
Open access