Endocrine System
Ectopic Cushing’s syndrome is a rare condition caused by an ACTH-secreting tumor
outside the pituitary gland, such as in the lungs or pancreas. These tumors, known
as ectopic ACTH-secreting tumors, disrupt the normal functioning of the endocrine
system and lead to a wide range of physiological disturbances.
In a healthy individual, the hypothalamus secretes corticotropin-releasing hormone
(CRH), which stimulates the anterior pituitary gland to produce adrenocorticotropic
hormone (ACTH). ACTH then acts on the adrenal cortex, signaling the release of
cortisol, a glucocorticoid hormone that regulates various metabolic processes.
Cortisol exerts negative feedback on both the hypothalamus and the anterior
pituitary, maintaining homeostasis.
However, in the case of an ectopic ACTH-secreting tumor, the neoplastic cells
continuously produce ACTH, bypassing the normal regulatory mechanisms. The
excessive ACTH stimulation leads to the overproduction of cortisol by the adrenal
glands, resulting in hypercortisolism. The persistently elevated cortisol levels have
systemic effects, including hyperglycemia, muscle wasting, osteoporosis, and
immunosuppression.
One of the most significant consequences of ectopic Cushing’s syndrome is its
impact on the cardiovascular system. Cortisol enhances the sensitivity of vascular
smooth muscle cells to the effects of catecholamines, leading to increased
peripheral vascular resistance and hypertension. Moreover, cortisol promotes
the retention of sodium and water in the kidneys, further contributing to the
development of hypertension and fluid overload.
The diagnosis of ectopic Cushing’s syndrome involves a combination of biochemical
tests and imaging studies. Dexamethasone suppression tests can help differentiate
between pituitary-dependent and ectopic sources of ACTH. Additionally, imaging
techniques such as CT scans, MRI, and PET scans are used to localize the ACTHsecreting tumor.
Treatment of ectopic Cushing’s syndrome primarily focuses on the surgical removal
of the ACTH-secreting tumor. If the tumor cannot be located or is inoperable,
medications such as ketoconazole or metyrapone may be used to inhibit cortisol
synthesis. In some cases, bilateral adrenalectomy may be necessary to control the
excessive cortisol production.
The excessive ACTH production in ectopic Cushing’s syndrome leads to the
overproduction of cortisol by:
A) The anterior pituitary gland
B) The posterior pituitary gland
C) The adrenal medulla
D) The adrenal cortex
Correct Answer: D
The excessive ACTH production in ectopic Cushing’s syndrome stimulates
the adrenal cortex to overproduce cortisol. The adrenal cortex is the outer
layer of the adrenal gland and is responsible for the synthesis and secretion of
glucocorticoids, such as cortisol.