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Ectopic Acromegaly Arising from a Pituitary Adenoma within the Bony Intersphenoid Septum of a Patient with Empty Sella Syndrome

OBJECTIVE:  To describe the work-up and treatment of rare ectopic acromegaly caused by a biopsy-proven somatotroph pituitary adenoma located within the bony intersphenoid septum of a patient with empty sella syndrome (ESS). METHODS:  We report the presentation, clinical course, diagnostic work-up, a...

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Autores principales: Arzamendi, Audrey E., Shahlaie, Kiarash, Latchaw, Richard E., Lechpammer, Mirna, Arzumanyan, Hasmik
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Georg Thieme Verlag KG 2016
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4958022/
https://www.ncbi.nlm.nih.gov/pubmed/27468406
http://dx.doi.org/10.1055/s-0036-1585091
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author Arzamendi, Audrey E.
Shahlaie, Kiarash
Latchaw, Richard E.
Lechpammer, Mirna
Arzumanyan, Hasmik
author_facet Arzamendi, Audrey E.
Shahlaie, Kiarash
Latchaw, Richard E.
Lechpammer, Mirna
Arzumanyan, Hasmik
author_sort Arzamendi, Audrey E.
collection PubMed
description OBJECTIVE:  To describe the work-up and treatment of rare ectopic acromegaly caused by a biopsy-proven somatotroph pituitary adenoma located within the bony intersphenoid septum of a patient with empty sella syndrome (ESS). METHODS:  We report the presentation, clinical course, diagnostic work-up, and lesion localization and treatment challenges encountered in a 55-year-old patient, with a brief review of relevant literature. RESULTS:  A 55-year-old African-American man presented with acromegaly and ESS. Attempts to definitively localize the causative tumor were unsuccessful, though petrosal sinus sampling supported central growth hormone production and imaging suggested bone-enclosed subsellar pituitary tissue. Endoscopic endonasal transphenoidal exploration was undertaken with resection of a somatotroph pituitary microadenoma, and subsequent clinical improvement and biochemical remission. Retrospective review revealed the patient's pituitary to have been located ectopically within a unique bony intersphenoid septum. CONCLUSION:  This report describes the first known case of an ectopic pituitary adenoma located within the midline bony intersphenoid septum, which we postulate to have resulted from anomalous embryological pituitary migration. Intra-intersphenoid septal tumors should be considered in cases of apparent central acromegaly with ESS or absence of tumor tissue within the paranasal sinuses or other peripheral locations. INDEXING:  Acromegaly, ESS, pituitary adenoma, sphenoid sinus septum.
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spelling pubmed-49580222016-07-27 Ectopic Acromegaly Arising from a Pituitary Adenoma within the Bony Intersphenoid Septum of a Patient with Empty Sella Syndrome Arzamendi, Audrey E. Shahlaie, Kiarash Latchaw, Richard E. Lechpammer, Mirna Arzumanyan, Hasmik J Neurol Surg Rep OBJECTIVE:  To describe the work-up and treatment of rare ectopic acromegaly caused by a biopsy-proven somatotroph pituitary adenoma located within the bony intersphenoid septum of a patient with empty sella syndrome (ESS). METHODS:  We report the presentation, clinical course, diagnostic work-up, and lesion localization and treatment challenges encountered in a 55-year-old patient, with a brief review of relevant literature. RESULTS:  A 55-year-old African-American man presented with acromegaly and ESS. Attempts to definitively localize the causative tumor were unsuccessful, though petrosal sinus sampling supported central growth hormone production and imaging suggested bone-enclosed subsellar pituitary tissue. Endoscopic endonasal transphenoidal exploration was undertaken with resection of a somatotroph pituitary microadenoma, and subsequent clinical improvement and biochemical remission. Retrospective review revealed the patient's pituitary to have been located ectopically within a unique bony intersphenoid septum. CONCLUSION:  This report describes the first known case of an ectopic pituitary adenoma located within the midline bony intersphenoid septum, which we postulate to have resulted from anomalous embryological pituitary migration. Intra-intersphenoid septal tumors should be considered in cases of apparent central acromegaly with ESS or absence of tumor tissue within the paranasal sinuses or other peripheral locations. INDEXING:  Acromegaly, ESS, pituitary adenoma, sphenoid sinus septum. Georg Thieme Verlag KG 2016-07 /pmc/articles/PMC4958022/ /pubmed/27468406 http://dx.doi.org/10.1055/s-0036-1585091 Text en © Thieme Medical Publishers
spellingShingle Arzamendi, Audrey E.
Shahlaie, Kiarash
Latchaw, Richard E.
Lechpammer, Mirna
Arzumanyan, Hasmik
Ectopic Acromegaly Arising from a Pituitary Adenoma within the Bony Intersphenoid Septum of a Patient with Empty Sella Syndrome
title Ectopic Acromegaly Arising from a Pituitary Adenoma within the Bony Intersphenoid Septum of a Patient with Empty Sella Syndrome
title_full Ectopic Acromegaly Arising from a Pituitary Adenoma within the Bony Intersphenoid Septum of a Patient with Empty Sella Syndrome
title_fullStr Ectopic Acromegaly Arising from a Pituitary Adenoma within the Bony Intersphenoid Septum of a Patient with Empty Sella Syndrome
title_full_unstemmed Ectopic Acromegaly Arising from a Pituitary Adenoma within the Bony Intersphenoid Septum of a Patient with Empty Sella Syndrome
title_short Ectopic Acromegaly Arising from a Pituitary Adenoma within the Bony Intersphenoid Septum of a Patient with Empty Sella Syndrome
title_sort ectopic acromegaly arising from a pituitary adenoma within the bony intersphenoid septum of a patient with empty sella syndrome
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4958022/
https://www.ncbi.nlm.nih.gov/pubmed/27468406
http://dx.doi.org/10.1055/s-0036-1585091
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