Cushing's disease due to an unusually large adenoma of the pituitary gland in infancy

We report an 18-month-old girl with Cushing's disease caused by a large adenoma of the pituitary gland. Tumour size and extension were determined by X-ray, CT-scan and angiographic studies. The endocrinological findings were typical for this disease: elevated plasma levels of ACTH, cortisol, 17...

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Veröffentlicht in:European journal of pediatrics 1985, Vol.143 (3), p.221-223
Hauptverfasser: STEGNER, H, LÜDECKE, D. K, KADRNKA-LOVRENCIC, M, STAHNKE, N, WILLIG, R. P
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container_end_page 223
container_issue 3
container_start_page 221
container_title European journal of pediatrics
container_volume 143
creator STEGNER, H
LÜDECKE, D. K
KADRNKA-LOVRENCIC, M
STAHNKE, N
WILLIG, R. P
description We report an 18-month-old girl with Cushing's disease caused by a large adenoma of the pituitary gland. Tumour size and extension were determined by X-ray, CT-scan and angiographic studies. The endocrinological findings were typical for this disease: elevated plasma levels of ACTH, cortisol, 17-Hydroxyprogesterone (17-OHP) and testosterone, elevated urinary excretion of 17-Ketosteroids (17-KS) and 17-Hydroxycorticoids (17-OHCS). Dexamethasone failed to suppress ACTH and cortisol plasma levels. TRH induced only a minimal TSH increase. Following LH-RH injection gonadotropin levels rose to pubertal values. The hGH response to insulin-induced hypoglycaemia was subnormal. After resection of the tumour the infant died because of non-treatable arrhythmia. Histological findings showed a non-differentiated adenoma with extension into the subarachnoid space and into nerve tissues. In vitro lysine-vasopressin (LVP) and arginine-vasopressin (AVP) exhibited only weak stimulatory effects on the ACTH secretion of the tumour cells.
doi_str_mv 10.1007/BF00442145
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subjects Adenoma - complications
Adenoma - pathology
Adenoma - surgery
Biological and medical sciences
Cushing Syndrome - etiology
Endocrinopathies
Female
Humans
Hypothalamus. Hypophysis. Epiphysis (diseases)
Infant
Malignant tumors
Medical sciences
Pituitary Neoplasms - complications
Pituitary Neoplasms - pathology
Pituitary Neoplasms - surgery
title Cushing's disease due to an unusually large adenoma of the pituitary gland in infancy
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