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SAT-466 Cases of Acromegaly and Presumed Prolactinoma in Setting of Empty Sella Syndrome: Presentation and Management Implications

Introduction; Empty Sella syndrome (ESS) is an uncommon finding on brain imaging whose functional significance remains controversial. While sometimes associated with pituitary hypofunction it is far less commonly associated with pituitary hyperfunction syndromes. We present two cases of this; one wi...

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Autor principal: Uwaifo, Gabriel
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Endocrine Society 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6552471/
http://dx.doi.org/10.1210/js.2019-SAT-466
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author Uwaifo, Gabriel
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description Introduction; Empty Sella syndrome (ESS) is an uncommon finding on brain imaging whose functional significance remains controversial. While sometimes associated with pituitary hypofunction it is far less commonly associated with pituitary hyperfunction syndromes. We present two cases of this; one with acromegaly and one of hyperprolactinemia presumably due to prolactinoma both associated with ESS on brain imaging. Clinical Cases; A 48 yr old man was evaluated on account of reduced libido and chronic fatigue which revealed hypogonadotrophic hypogonadism. Further work up revealed prolactin levels in the 250-300ng/ml range. Brain MRI showed ESS and the rest of the pituitary hormone functional work up was normal. Bone density showed osteopenia and octreotide scan showed no sella enhancement nor other anomalies. When commenced on cabergoline 0.5mg 2 x wkly his prolactin levels normalized over 6 months and the hypogonadism resolved without requiring androgen replacement. A 49 yr old gentleman with long standing hypogonadotrophic hypogonadism on androgen replacement presented with persistent headaches and generalized arthralgias. He had clinical features suggestive of acromegaly including macrocephaly, macroglossia, facial dysmorphia and enlarged hands and feet. Diagnosis was confirmed by elevated IGF-1 (300-400ng/ml range), growth hormone levels and non suppressible growth hormone on Oral glucose tolerance test. No other pituitary functional deficits were found. Brain MRI showed ESS and octreotide scan showed no sella enhancement nor other anomalies. His IGF-1 levels normalized with octreotide LAR 20mg monthly. His androgen repletion also improved with lower testosterone dose requirement along with improvement in headaches and arthralgias though he developed type 2 diabetes. Results and Discussion; Review of our system's clinical records over the prior 5 years revealed 307 cases of ESS. 10 had hypopituitarism, 6 had hypogonadism, 12 had hyperprolactinemia presumed due to prolactinoma, 1 each had central hypothyroidism, acromegaly and diabetes insipidus. 276 (92%) of cases had normal pituitary functional status. Concluding remarks; ESS is an uncommon but important imaging finding on brain imaging. Though most patients with ESS have normal pituitary function it may be associated with pituitary hypofunction or even hyperfunction states. Its finding in pituitary hyperfunction states like hyperprolactinemia and acromegaly make medication therapy the only viable treatment option as surgery and radiotherapy have no visible target to direct therapy.
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spelling pubmed-65524712019-06-13 SAT-466 Cases of Acromegaly and Presumed Prolactinoma in Setting of Empty Sella Syndrome: Presentation and Management Implications Uwaifo, Gabriel J Endocr Soc Neuroendocrinology and Pituitary Introduction; Empty Sella syndrome (ESS) is an uncommon finding on brain imaging whose functional significance remains controversial. While sometimes associated with pituitary hypofunction it is far less commonly associated with pituitary hyperfunction syndromes. We present two cases of this; one with acromegaly and one of hyperprolactinemia presumably due to prolactinoma both associated with ESS on brain imaging. Clinical Cases; A 48 yr old man was evaluated on account of reduced libido and chronic fatigue which revealed hypogonadotrophic hypogonadism. Further work up revealed prolactin levels in the 250-300ng/ml range. Brain MRI showed ESS and the rest of the pituitary hormone functional work up was normal. Bone density showed osteopenia and octreotide scan showed no sella enhancement nor other anomalies. When commenced on cabergoline 0.5mg 2 x wkly his prolactin levels normalized over 6 months and the hypogonadism resolved without requiring androgen replacement. A 49 yr old gentleman with long standing hypogonadotrophic hypogonadism on androgen replacement presented with persistent headaches and generalized arthralgias. He had clinical features suggestive of acromegaly including macrocephaly, macroglossia, facial dysmorphia and enlarged hands and feet. Diagnosis was confirmed by elevated IGF-1 (300-400ng/ml range), growth hormone levels and non suppressible growth hormone on Oral glucose tolerance test. No other pituitary functional deficits were found. Brain MRI showed ESS and octreotide scan showed no sella enhancement nor other anomalies. His IGF-1 levels normalized with octreotide LAR 20mg monthly. His androgen repletion also improved with lower testosterone dose requirement along with improvement in headaches and arthralgias though he developed type 2 diabetes. Results and Discussion; Review of our system's clinical records over the prior 5 years revealed 307 cases of ESS. 10 had hypopituitarism, 6 had hypogonadism, 12 had hyperprolactinemia presumed due to prolactinoma, 1 each had central hypothyroidism, acromegaly and diabetes insipidus. 276 (92%) of cases had normal pituitary functional status. Concluding remarks; ESS is an uncommon but important imaging finding on brain imaging. Though most patients with ESS have normal pituitary function it may be associated with pituitary hypofunction or even hyperfunction states. Its finding in pituitary hyperfunction states like hyperprolactinemia and acromegaly make medication therapy the only viable treatment option as surgery and radiotherapy have no visible target to direct therapy. Endocrine Society 2019-04-30 /pmc/articles/PMC6552471/ http://dx.doi.org/10.1210/js.2019-SAT-466 Text en Copyright © 2019 Endocrine Society https://creativecommons.org/licenses/by-nc-nd/4.0/ This article has been published under the terms of the Creative Commons Attribution Non-Commercial, No-Derivatives License (CC BY-NC-ND; https://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Neuroendocrinology and Pituitary
Uwaifo, Gabriel
SAT-466 Cases of Acromegaly and Presumed Prolactinoma in Setting of Empty Sella Syndrome: Presentation and Management Implications
title SAT-466 Cases of Acromegaly and Presumed Prolactinoma in Setting of Empty Sella Syndrome: Presentation and Management Implications
title_full SAT-466 Cases of Acromegaly and Presumed Prolactinoma in Setting of Empty Sella Syndrome: Presentation and Management Implications
title_fullStr SAT-466 Cases of Acromegaly and Presumed Prolactinoma in Setting of Empty Sella Syndrome: Presentation and Management Implications
title_full_unstemmed SAT-466 Cases of Acromegaly and Presumed Prolactinoma in Setting of Empty Sella Syndrome: Presentation and Management Implications
title_short SAT-466 Cases of Acromegaly and Presumed Prolactinoma in Setting of Empty Sella Syndrome: Presentation and Management Implications
title_sort sat-466 cases of acromegaly and presumed prolactinoma in setting of empty sella syndrome: presentation and management implications
topic Neuroendocrinology and Pituitary
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6552471/
http://dx.doi.org/10.1210/js.2019-SAT-466
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