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Ectopic Cushing syndrome: Report of 9 cases
Marta Araujo Castro1, Nuria Palacios García1, Javier Aller Pardo1
1Servicio de Endocrinología y Nutrición, Hospital Universitario Puerta de Hierro, Majadahonda, Madrid, España.
Endocrinologia, Diabetes Y Nutricion
|April 16, 2018
Summary
Ectopic Cushing's syndrome (ECS) is a rare ACTH-secreting tumor condition. Management requires multidisciplinary approaches, often involving surgery, systemic therapy, or chemotherapy, to control hypercortisolism and the tumor.
Area of Science:
- Endocrinology
- Oncology
- Rare Diseases
Background:
- Ectopic Cushing's syndrome (ECS) is a rare endocrine disorder caused by ectopic ACTH secretion from tumors.
- The rarity of ECS presents diagnostic and management challenges due to limited clinical experience.
Purpose of the Study:
- To describe the clinical characteristics, management, and outcomes of patients with ECS.
- To evaluate the diagnostic and therapeutic strategies employed over a 15-year period at a tertiary hospital.
Main Methods:
- Retrospective analysis of clinical, biochemical, and radiographic data.
- Review of treatment modalities and patient follow-up for ECS cases.
Main Results:
- Nine patients (6 female, mean age 47) with rapid symptom onset were identified.
- Elevated urinary free cortisol and ACTH levels were observed; ectopic origin confirmed non-invasively.
- Tumors were identified in 8 cases, with 7 having metastatic disease; treatment involved surgery, systemic therapy, chemotherapy, and bilateral adrenalectomy.
Conclusions:
- ECS encompasses a spectrum of aggressive tumors requiring diverse treatments.
- Diagnosis is often achievable without invasive tests; multidisciplinary management is crucial for optimal outcomes.
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