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ACTH-producing pheochromocytoma.

M R Berenyi, G Singh, E S Gloster

    Archives of Pathology & Laboratory Medicine
    |January 1, 1977
    PubMed
    Summary
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    A rare benign adrenal tumor secreted adrenocorticotropic hormone (ACTH), causing Cushing syndrome. This tumor mimicked pituitary Cushing disease, with 70% biologically active ACTH found only in pituitary tissue.

    Area of Science:

    • Endocrinology
    • Oncology

    Background:

    • Cushing syndrome is typically caused by pituitary adenomas or adrenal tumors.
    • Adrenal medullary tumors rarely secrete hormones like ACTH.

    Observation:

    • A benign adrenal medullary tumor was identified as the source of ACTH secretion.
    • The patient presented with clinical and biochemical features of Cushing syndrome.

    Findings:

    • The tumor secreted ACTH, leading to bilateral adrenocortical hyperplasia.
    • Tumor ACTH fractionation revealed a high concentration (70%) of biologically active "little" ACTH, characteristic of pituitary tissue.
    • The patient showed partial suppression of urinary hydroxycorticosteroids after high-dose dexamethasone administration, similar to pituitary Cushing disease.

    Implications:

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    • This case highlights a rare cause of Cushing syndrome originating from the adrenal medulla.
    • The findings suggest that adrenal medullary tumors can ectopically produce ACTH with pituitary-like characteristics.
    • Further research is needed to understand the mechanisms behind ectopic ACTH production by adrenal tumors.