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ACTH-Dependent Cushing Syndrome: Clinical and Diagnostic Aspects, and Treatment Approaches for Ectopic Cushing’s Syndrome

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Cushing's Syndrome

Part of the book series: Contemporary Endocrinology ((COE))

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Summary

Ectopic Cushing’s syndrome (ECS) is represented mainly by the ectopic ACTH syndrome (EAS) and comprises ∼20% of ACTH-dependent CS and ∼10% of all types of CS. Nearly any neuroendocrine or non-endocrine tumour may be associated with EAS but the more prevalent tumours are bronchial carcinoids, small cell lung carcinomas (SCLC), islet cell tumours of the pancreas or pancreatic carcinoids, thymic carcinoids, medullary carcinomas of the thyroid, phaeochromocytomas and gastrinomas. Occult tumours are highly represented in all the series (12–37.5%) and constitute the more challenging cases of EAS, requiring long-term follow-up. Clinical features are often similar in ACTH-dependent CS, but the rapid onset and progress may suggest an ectopic source. A combination of biochemical tests and imaging studies seem the most appropriate approach for the prompt identification of EAS, even if there are several pitfalls to be avoided along the way. The most appropriate management for cure of EAS, when its source is identified, is surgical excision after control of hypercortisolaemia. Inhibitors of cortisol secretion and other newer modalities can be used alone or in combination, and bilateral adrenalectomy remains an alternative option. Tumour histology, the presence of metastases and effective control of hypercortisolaemia affect mortality and morbidity. The identification of a primary source for the EAS represents a considerable challenge for endocrinologists, requiring a careful and logical step-by-step approach of investigational protocols to avoid the calamity of misdiagnosis.

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Authors and Affiliations

  1. Department of Endocrinology, St. Bartholomew’s Hospital, London, UK

    Krystallenia I. Alexandraki MD/PhD/MSC/MSC & Andrea M. Isidori MD

  2. Department of Endocrinology, Barts and the London School of Medicine and Dentistry, John Vane Science Centre, Charterhouse Square, London, UK

    Ashley B. Grossman MD/BA/BSC/FRCP/FMEDSCI

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  2. Andrea M. Isidori MD
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  1. , Division of Endocrinology and Metabolism, Hospital das Clínicas, University of Sao, Sao Paulo, 05403-000, Brazil

    Marcello D. Bronstein

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Alexandraki, K.I., Isidori, A.M., Grossman, A.B. (2010). ACTH-Dependent Cushing Syndrome: Clinical and Diagnostic Aspects, and Treatment Approaches for Ectopic Cushing’s Syndrome. In: Bronstein, M. (eds) Cushing's Syndrome. Contemporary Endocrinology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-60327-449-4_14

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