Vol 59, No 4 (2008)
Case report
Submitted: 2013-02-15
Published online: 2008-07-09
Acromegaly in a patient with normal pituitary gland and somatotropic adenoma located in the sphenoid sinus
Maria Kurowska, Jerzy S. Tarach, Wojciech Zgliczyński, Joanna Malicka, Grzegorz Zieliński, Marzena Janczarek
Endokrynol Pol 2008;59(4):348-351.
Vol 59, No 4 (2008)
Case report
Submitted: 2013-02-15
Published online: 2008-07-09
Abstract
Ectopic acromegaly is a very rare clinical entity occurring in less than 1% of acromegalic patients. In most cases it is caused by GHRH or
rarely GH–secreting neoplasms. Even rarer are ectopic pituitary adenomas located in the sphenoid sinus or nasopharynx that originate
from pituitary remnants in the craniopharyngeal duct. This dissertation presents the difficulties in visualizing GH-secreting adenoma
located in the sphenoid sinus.
A 55-year-old man had somatic features of acromegaly for several years. MRI imaging revealed a slightly asymmetric pituitary gland
(14 ¥ 4 mm) without focal lesions. Simultaneously, a spherical mass, 10 mm in diameter, corresponding with ectopic microadenoma was
demonstrated on the upper wall of the sphenoid sinus. The serum GH level was 4.3 mg/l, IGF-1 = 615 mg/l, and a lack of GH suppression
with oral glucose was proven. After preliminary treatment with a long-acting somatostatin analogue, transsphenoidal pituitary tumour
removal was performed. Histopathological, electron microscopical and immunohistochemical analysis revealed densely granulated somatotropic
pituitary adenoma: GH(+), PRL(-), ACTH(-), TSH(-), FSH(-), LH(-), MIB1 < 1%, SSTR3(+) and SSTR5(+). Post-surgical evaluation
showed normal pituitary MRI scans, GH and IGF-1 levels 0.18 μg/l and 140 mg/l, respectively, as well as normal GH suppression with
oral glucose.
The careful analysis of possible pituitary embryonic malformations points out their significance for proper localization of extrapituitary
adenomas.
Abstract
Ectopic acromegaly is a very rare clinical entity occurring in less than 1% of acromegalic patients. In most cases it is caused by GHRH or
rarely GH–secreting neoplasms. Even rarer are ectopic pituitary adenomas located in the sphenoid sinus or nasopharynx that originate
from pituitary remnants in the craniopharyngeal duct. This dissertation presents the difficulties in visualizing GH-secreting adenoma
located in the sphenoid sinus.
A 55-year-old man had somatic features of acromegaly for several years. MRI imaging revealed a slightly asymmetric pituitary gland
(14 ¥ 4 mm) without focal lesions. Simultaneously, a spherical mass, 10 mm in diameter, corresponding with ectopic microadenoma was
demonstrated on the upper wall of the sphenoid sinus. The serum GH level was 4.3 mg/l, IGF-1 = 615 mg/l, and a lack of GH suppression
with oral glucose was proven. After preliminary treatment with a long-acting somatostatin analogue, transsphenoidal pituitary tumour
removal was performed. Histopathological, electron microscopical and immunohistochemical analysis revealed densely granulated somatotropic
pituitary adenoma: GH(+), PRL(-), ACTH(-), TSH(-), FSH(-), LH(-), MIB1 < 1%, SSTR3(+) and SSTR5(+). Post-surgical evaluation
showed normal pituitary MRI scans, GH and IGF-1 levels 0.18 μg/l and 140 mg/l, respectively, as well as normal GH suppression with
oral glucose.
The careful analysis of possible pituitary embryonic malformations points out their significance for proper localization of extrapituitary
adenomas.
Keywords
acromegaly; ectopic somatotropic adenoma; sphenoid sinus
Title
Acromegaly in a patient with normal pituitary gland and somatotropic adenoma located in the sphenoid sinus
Journal
Endokrynologia Polska
Issue
Vol 59, No 4 (2008)
Article type
Case report
Pages
348-351
Published online
2008-07-09
Page views
708
Article views/downloads
5490
Bibliographic record
Endokrynol Pol 2008;59(4):348-351.
Keywords
acromegaly
ectopic somatotropic adenoma
sphenoid sinus
Authors
Maria Kurowska
Jerzy S. Tarach
Wojciech Zgliczyński
Joanna Malicka
Grzegorz Zieliński
Marzena Janczarek