1. The clinical and biochemical spectrum of ectopic acromegaly.
- Author
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Fainstein-Day P, Ullmann TE, Dalurzo MCL, Sevlever GE, and Smith DE
- Subjects
- Humans, Neuroendocrine Tumors diagnosis, Neuroendocrine Tumors metabolism, Neuroendocrine Tumors complications, Human Growth Hormone blood, Human Growth Hormone metabolism, Pituitary Neoplasms diagnosis, Pituitary Neoplasms surgery, Pituitary Neoplasms complications, Pituitary Neoplasms metabolism, Acromegaly diagnosis, Acromegaly etiology, Acromegaly blood, Growth Hormone-Releasing Hormone metabolism
- Abstract
Ectopic acromegaly is a rare condition caused by extrapituitary central or peripheral neuroendocrine tumours (NET) that hypersecrete GH or, more commonly, GHRH. It affects less than 1% of acromegaly patients and a misdiagnosis of classic acromegaly can lead to an inappropriate pituitary surgery. Four types of ectopic acromegaly have been described: 1) Central ectopic GH-secretion: Careful cross-sectional imaging is required to exclude ectopic pituitary adenomas. 2) Peripheral GH secretion: Extremely rare. 3) Central ectopic GHRH secretion: Sellar gangliocytomas immunohistochemically positive for GHRH are found after pituitary surgery. 4) Peripheral GHRH secretion: The most common type of ectopic acromegaly is due to peripheral GHRH-secreting NETs. Tumours are large and usually located in the lungs or pancreas. Pituitary hyperplasia resulting from chronic GHRH stimulation is difficult to detect or can be misinterpreted as pituitary adenoma in the MRI. Measurement of serum GHRH levels is a specific and useful diagnostic tool. Surgery of GHRH-secreting NETs is often curative., (Copyright © 2024 Elsevier Ltd. All rights reserved.)
- Published
- 2024
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