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[Cushing syndrome with occult ectopic ACTH production]
Insights
A bronchial carcinoid tumor caused ectopic ACTH production, leading to Cushing's syndrome. Surgical removal of the tumor and adrenal glands resolved the patient's symptoms.
Area of Science:
- Endocrinology
- Oncology
- Pulmonology
Background:
- Cushing's syndrome is a hormonal disorder caused by prolonged exposure to high cortisol levels.
- Pituitary adenomas are the most common cause of Cushing's syndrome, but ectopic ACTH production can also occur.
Observation:
- A 19-year-old patient presented with symptoms of prolonged Cushing's syndrome, initially diagnosed as pituitary Cushing's.
- Treatment for pituitary Cushing's and ketoconazole therapy were ineffective.
- Ectopic ACTH production was suspected due to persistent hypercortisolism.
Findings:
- Bilateral adrenalectomy was performed, leading to the resolution of Cushing's syndrome symptoms.
- A CT scan revealed an intrapulmonary mass one year post-adrenalectomy.
- Histological examination confirmed a bronchial carcinoid tumor producing ACTH, with no signs of metastasis.
Implications:
- This case highlights the importance of considering ectopic ACTH production in refractory Cushing's syndrome.
- Bronchial carcinoids can present as a rare cause of ACTH-dependent Cushing's syndrome.
- Early diagnosis and surgical intervention are crucial for managing ectopic ACTH-producing tumors.
Abstract:
A 19-year old patient was admitted to our hospital with the typical stigmata of a prolonged Cushing's syndrome. He presented first at another hospital with elevated ACTH values and the diagnosis of pituitary Cushing's syndrome was made. A partial hypophysectomy was performed, but neither this nor a therapy with ketokonazole could influence the cortisol levels. Because of this background the diagnosis of ectopic ACTH-production was established. Since we were not able to locate a source of ACTH, we recommended a bilateral adrenalectomy, after which the symptoms of Cushing's syndrome disappeared within a few months. About one year after the operation a CT-scan demonstrated an intrapulmonary mass, which was removed surgically. There were neither signs of local infiltration nor of lymphogenic metastasis. The histological and immunhistochemical diagnosis was typical of bronchial carcinoid with ACTH-production. The follow up so far did not reveal a recurrence.