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A Case-Based Guide to Clinical EndocrinologyPituitary Tumor Behavior and Disease Severity in Patients with Acromegaly

A Case-Based Guide to Clinical Endocrinology: Pituitary Tumor Behavior and Disease Severity in... [In most cases, acromegaly is caused by a GH-secreting pituitary somatotroph cell adenoma. Excess GH secretion subsequently increases IGF-1 levels. The clinical presentation, severity, and prognosis of the disease have been associated to distinctive tumor behavior and pathology characteristics. Some patients may harbor small localized microadenomas, whereas others have invasive macroadenomas causing compression of the optic chiasm, the latter with lower remission rates. Diverse clinical profiles are seen between patients with densely and sparsely granulated adenomas, those with and without p21-mediated cell-cycle arrest, and the degree of type 2 somatostatin receptor (SSTR2) expression on the tumor. Currently, the first-line therapy is transsphenoidal surgery in most patients followed by the somatostatin receptor ligands octreotide LAR and lanreotide autogel (directed mainly to SSTR2) or the multi-receptor SSTR ligand pasireotide LAR. Cabergoline, a dopamine D2 receptor agonist, and pegvisomant, a GH receptor antagonist, are second-line medical choices for disease control. Oral octreotide has been recently approved. Combination therapy is also increasingly employed. Stereotactic radiotherapy is indicated when patients remain active. In this chapter we will review three cases of acromegaly in patients with different clinical presentations, tumor aggressiveness, and outcomes.] http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png

A Case-Based Guide to Clinical EndocrinologyPituitary Tumor Behavior and Disease Severity in Patients with Acromegaly

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Publisher
Springer International Publishing
Copyright
© The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature Switzerland AG 2022
ISBN
978-3-030-84366-3
Pages
13 –25
DOI
10.1007/978-3-030-84367-0_2
Publisher site
See Chapter on Publisher Site

Abstract

[In most cases, acromegaly is caused by a GH-secreting pituitary somatotroph cell adenoma. Excess GH secretion subsequently increases IGF-1 levels. The clinical presentation, severity, and prognosis of the disease have been associated to distinctive tumor behavior and pathology characteristics. Some patients may harbor small localized microadenomas, whereas others have invasive macroadenomas causing compression of the optic chiasm, the latter with lower remission rates. Diverse clinical profiles are seen between patients with densely and sparsely granulated adenomas, those with and without p21-mediated cell-cycle arrest, and the degree of type 2 somatostatin receptor (SSTR2) expression on the tumor. Currently, the first-line therapy is transsphenoidal surgery in most patients followed by the somatostatin receptor ligands octreotide LAR and lanreotide autogel (directed mainly to SSTR2) or the multi-receptor SSTR ligand pasireotide LAR. Cabergoline, a dopamine D2 receptor agonist, and pegvisomant, a GH receptor antagonist, are second-line medical choices for disease control. Oral octreotide has been recently approved. Combination therapy is also increasingly employed. Stereotactic radiotherapy is indicated when patients remain active. In this chapter we will review three cases of acromegaly in patients with different clinical presentations, tumor aggressiveness, and outcomes.]

Published: Jan 4, 2022

Keywords: Acromegaly; Growth hormone; Insulin-like growth factor; Pituitary adenoma; Somatostatin receptor ligand

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