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An infant with Cushing's disease due to an adrenocorticotropin-producing pituitary adenoma
Insights
This study presents an infant with Cushing's disease caused by an ACTH-producing pituitary adenoma. The tumor led to elevated hormone levels and was confirmed through pathology and cell culture.
Area of Science:
- Pediatric Endocrinology
- Neuroendocrinology
- Oncology
Background:
- Cushing's disease in infants is rare and characterized by glucocorticoid excess.
- Early diagnosis and understanding of the underlying cause are crucial for management.
Observation:
- An 8-month-old male infant presented with clinical signs of glucocorticoid excess.
- Elevated levels of cortisol, 17-hydroxyprogesterone, and adrenal androgens were observed.
- Dexamethasone suppression tests and ACTH stimulation tests showed abnormal responses.
Findings:
- Plasma ACTH levels were significantly elevated (700 pg/ml).
- Imaging revealed an intracranial mass superior and anterior to the sella turcica.
- A craniotomy confirmed an inoperable ACTH-producing pituitary adenoma with a significant fibroblastic reaction.
Implications:
- Confirms the diagnosis of an ACTH-producing pituitary adenoma in an infant via immunohistochemistry and in vitro cell culture.
- Highlights the importance of advanced imaging and pathological confirmation in diagnosing pediatric Cushing's disease.
- Underscores the challenges in managing inoperable pituitary adenomas in infants.
Abstract:
An 8-month old male with Cushing's disease is presented; his clinical presentation and appearance were typical of infants with glucocorticoid excess. Concentrations of cortisol, 17-hydroxyprogesterone, and adrenal androgens were strikingly elevated. High doses of dexamethasone did not suppress the excretion of urinary free cortisol or 17-hydroxycorticoids, and administration of ACTH elicited no further rise in plasma cortisol. Responses of LH, FSH, and PRL to iv LRF and TRF were appropriate for age, but neither TSH nor ACTH rose significantly. Plasma ACTH values were elevated to 700 pg/ml. An intracranial mass lesion superior and anterior to the sella turcica was demonstrated by computerized axial tomography and angiography. An inoperable pituitary adenoma was a massive surrounding fibroblastic reaction was found at craniotomy. The pathological diagnosis of an ACTH-producing pituitary adenoma was confirmed by immunohistochemistry and by the in vitro secretion of ACTH by cells cultured from the tumor.