- Department of Neurosurgery, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina
- Department of Neurosurgery, Hospital Padilla, Tucumán, Argentina
Correspondence Address:
Pablo Ajler
Department of Neurosurgery, Hospital Padilla, Tucumán, Argentina
DOI:10.4103/2152-7806.96066
Copyright: © 2012 Ajler P. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.How to cite this article: Ajler P, Damián Bendersky, Hem S, Campero A. Ectopic prolactinoma within the sphenoidal sinus associated with empty sella. Surg Neurol Int 14-May-2012;3:47
How to cite this URL: Ajler P, Damián Bendersky, Hem S, Campero A. Ectopic prolactinoma within the sphenoidal sinus associated with empty sella. Surg Neurol Int 14-May-2012;3:47. Available from: http://sni.wpengine.com/surgicalint_articles/ectopic-prolactinoma-within-the-sphenoidal-sinus-associated-with-empty-sella/
Abstract
Background:Pituitary adenomas are a common cause of endocrinal dysfunction, which comprise 10–20% of all intracranial tumors. Although almost all of them arise within the sella turcica, there are some rare cases in which a pituitary adenoma is located outside the intrasellar region, so it is defined as an ectopic pituitary adenoma (EPA).
Case Description:We described a case of a 31-year-old male with a serum prolactin (PRL) value of 240 ng/ml Magnetic resonance imaging (MRI) showed a space-occupying mass within the sphenoid sinus (SS) which partially enhanced by gadolinium. MRI did not reveal any sellar floor defect and an empty sella was detected. As dopamine agonist treatment had failed in lowering the serum PRL level, he underwent surgical treatment. A transsphenoidal approach without opening the sellar floor was performed using an operating microscope and the lesion within the SS was completely removed.
Conclusion:Although intrasphenoidal EPAs are rare findings, the presence of an endocrine disorder related to pituitary hormones, and a space-occupying mass within the SS associated with either a normal sellar pituitary gland or an empty sella must lead us to suspect this diagnosis.
Keywords: Ectopic pituitary adenoma, empty sella, prolactinoma, sphenoid sinus
INTRODUCTION
Pituitary adenomas are a common cause of endocrinal dysfunction, which comprise 10–20% of all intracranial tumors.[
CASE REPORT
A 31-year-old male with a 4-month history of sexual dysfunction was referred to the endocrinological department. His past medical history was negative and he was not taking any medication. Psychogenic impotence was ruled out by a psychiatric examination. He had a serum prolactin (PRL) value of 240 ng/ml. Laboratory examinations, including other pituitary hormones, did not reveal other abnormalities. Magnetic resonance imaging (MRI) showed a space-occupying mass within the SS, which partially enhanced by gadolinium. MRI did not reveal any sellar floor defect and an empty sella was detected [Figures
DISCUSSION
Whereas EPAs are not in continuity with an intrasellar normal pituitary gland by definition, they must be distinguished from invasive pituitary adenomas, which are connected with the sellar pituitary gland through a sellar floor defect. The difference between these two types of pituitary adenomas is based on the state, intact or damaged, of the dura of the sellar floor, and it may be seen accurately on MRI. Also, empty sella may be associated with EPAs.[
As we have already referred, EPAs may be intra or extracranial. Intracranial EPAs are found in the hypothalamic region, cavernous sinus, third ventricle, pituitary stalk, and suprasellar region, while extracranial ones are usually located at the SS or the sphenoid bone, and rarely at the nasopharynx or clivus.[
During development, primitive pituitary gland separates into pharyngeal and distal parts in the 8th gestational week. Last ones constitute the Rathke's pouch, which migrates upward to develop the sellar pituitary gland. Its route is known as craniopharyngeal canal.[
In case of an ectopic pituitary mass associated with an empty sella, the speculation is that during development of the anterior pituitary gland, most of the precursors remained in the SS and only a small number of cells constituted the anterior pituitary, leading to the empty sella, which is shown by MRI scans.[
Although between one-third and one-half of EPAs are non-functioning tumors, these may secrete any anterior pituitary hormone, such as sellar pituitary adenomas. Thus, it may cause several endocrine disorders such as Cushing's disease, acromegaly, or hyperprolactinemia.[
Because of its low frequency, there is not any accurate guideline for the treatment of ectopic prolactinomas. Regarding sellar prolactinomas, medical treatment is almost always the first choice and surgical treatment is indicated in those patients who fail to normalize serum PRL level in spite of using dopamine agonists, are intolerant for these medications, or whose tumors compress the optic nerves, as also in the presence of a macroprolactinoma, or in patients who present with neurological deficits, cerebrospinal fluid leakage due to tumor shrinkage after dopamine agonists or pituitary apoplexy.[
CONCLUSION
Although intrasphenoidal EPAs are rare findings, the presence of an endocrine disorder related to pituitary hormones and a space-occupying mass within the SS associated with either a normal sellar pituitary gland or an empty sella must lead us to suspect this diagnosis. It must be distinguished from an invasive pituitary adenoma which is a different entity, and thus it has not the same physiopathology.
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