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Ectopic pituitary adenoma: The lost track of ciurea or the right man at wrong place
Journal Pre-proofs Case Reports & Case Series Ectopic pituitary adenoma: The lost track of Ciurea or the right man at wrong place Suyash Singh, Gagandeep Attri, Kuntal Kanti Das, Sushila Jaiswal, Awadhesh Kumar Jaiswal PII: DOI: Reference:
S2214-7519(19)30004-0 https://doi.org/10.1016/j.inat.2019.100567 INAT 100567
To appear in:
Interdisciplinary Neurosurgery: Advanced Techniques and Case Management Interdisciplinary Neurosurgery: Advanced Techniques and Case Management
Received Date: Revised Date: Accepted Date:
10 December 2018 7 August 2019 14 August 2019
Please cite this article as: S. Singh, G. Attri, K. Kanti Das, S. Jaiswal, A. Kumar Jaiswal, Ectopic pituitary adenoma: The lost track of Ciurea or the right man at wrong place, Interdisciplinary Neurosurgery: Advanced Techniques and Case Management Interdisciplinary Neurosurgery: Advanced Techniques and Case Management (2019), doi: https://doi.org/10.1016/j.inat.2019.100567
This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
© 2019 Published by Elsevier B.V.
Ectopic pituitary adenoma: The lost track of Ciurea or the right man at wrong place Suyash Singha, Gagandeep Attria, Kuntal Kanti Dasa, Sushila Jaiswalb, Awadhesh Kumar Jaiswala,*[email protected] aDepartment
of Neurosurgery, Sanjay Gandhi Post Graduate Institute of Medical Sciences,
Lucknow, 226014, India bDepartment
of Pathology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow,
226014, India *Corresponding
author.
Highlights
Sphenoid sinus is the most common site for ectopic pituitary adenomas
Ectopic sphenoid sinus pituitary adenoma may present with nasal obstruction, headache, decreased vision or cerebrospinal fluid leak
The location per se is favourable for both surgeon and adjuvant radiation
Abstract The aberrant development of pituitary gland may leave functioning or nonfunctioning tissues anywhere along the craniopharyngeal canal. Ectopic pituitary adenoma is extremely rare with a reported incidence of only 0.5% in the literature. The sphenoid sinus is the most common site for ectopic pituitary adenomas. The preoperative diagnosis is often misleading with a normal “sella turcica” or “pituitary gland” with, no evidence of connection between the gland and the ectopic mass, being a pre requisite for diagnosis. Ectopic sphenoid sinus pituitary adenoma may present with nasal obstruction, headache, decreased vision or cerebrospinal fluid leak. The recent understanding in embryogenesis and effect of Wingless integrated (WNT) gene signaling suggest that ectopic glands may have a higher propensity to develop adenomas. We herein report a case of nonfunctional ectopic pituitary adenoma in the sphenoid sinus presenting with nasal stuffiness and highlight the key radiological findings.
Abbreviations
MRI, Magnetic resonance imaging; CT, computed tomography; WHO, World Health organization;
Keywords: Ectopic pituitary; Sphenoid sinus; empty sella
Introduction Pituitary adenoma, in the non-sellar location, suggests some underlying developmental anomaly. An associated empty sella, further confuses the treating surgeon about pathological nature of disease and the line of investigation. It is common for a non functional pituitary adenoma to enlarge, erode the sellar floor and reach sphenois sinus. It is further possible that, with progression, sellar part may undergo necrosis and present as an empty sella [1]. However, evidence of normal pituitary gland in the sella and a mass lesion in sphenoid sinus is a different situation. This ectopic pituitary adenoma may be secretory or non secretory. From the management point of view, the sphenoidal location per se is more favourable for both surgeon and the radiation oncologist. We, hereby, report a rare case of sphenoidal non secretory pituitary adenoma, presenting with nasal stuffiness, operated by endoscopic endonasal, approach and discuss the diagnostic dilemma we faced. Case Summary A 42-year old woman was referred to our institute with complaints of right sided nasal stuffiness for 2 months. Her visual acuity was 6/6 bilaterally and the extraocular movements also normal. Nasal examination was normal. On fundoscopy there was no papilloedema. Clinically, there was no endocrinological dysfunction. Computed Tomography (CT) scan of the head and the paranasal sinuses revealed a mass lesion in the sphenoid sinus extending into the upper 1/3 rd of the clivus with erosion of the sellar floor and an open book like sella turcica (Figure 1). Magnetic resonance Imaging (MRI) showed a rounded T2 hyperintense mass lesion present in the sphenoid sinus and upper 1/3 rd of the clivus with sellar extension (Figure 1). The sellar floor dura was apparently intact. Distortion of the pituitary stalk suggested a possible right superolateral displacement of the pituitary gland. Her hormonal profile
was within normal limits [serum prolactin 211.9 miu/l; serum IGF-1 70.8 ug/l; serum FSH 52.06 iu/l; serum 8 a.m. cortisol 408 nmol/l; serum TSH (thyrotropin 1.91 miu/l; and serum T4 (thyroxine) 116.0 nmol/l]. We operated her using an endoscopic endonasal transsphenoidal approach. We encountered a soft, free flowing tumor filling the sphenoid sinus (Figure 2). The mass was entirely extradural, having eroded the sella. The clivus was scalloped from within. After the mass was removed, the sellar floor dura was seen intact. A cruciate incision was made to inspect the sella where the normal pituitary gland was seen separately. The patient recovered uneventfully after surgery. The histopathological examination was suggestive of pituitary adenoma, showing tumor disposed in sheets, trabeculae and nests separated by thin fibrovascular septae, predominantly monomorphic nuclei and immunohistochemistry showed tumor cells show Ki-67 < 1%. (Figure 3) LCA and vimentin were negative and so were ACTH, GH and PRL receptors. Post operative MRI showed complete excision of tumour with sphenoid sinus filled with fat (Figure 2). Follow up hormonal work up was normal. Discussion Ectopic pituitary adenomas are rare entities, often misdiagnosed as neuroendocrine tumors, sinonasal carcinomas or even fungal infections. Other reported sites for ectopic pituitary adenomas are nasal cavity, ethmoid sinus, temporal bone, nasal bridge, meckel’s cave, cavernous sinus and thalamus [2]. The underlying cause for non-sellar location is an aberrant embryological developmental. The anterior lobe of the pituitary arises from the craniopharyngeal canal while the posterior pituitary is actually a part of the hypothalamus, descending in to the sella to combine with its counterpart [3]. In our patient, the pituitary stalk and the compressed posterior pituitary were seen superior to the lesion. The normal sellar structures are difficult to demonstrate in pathological conditions, particularly in pituitary macroadenimas and if it is seen on MRI separately from a well defined mass lesion, then an alternate diagnosis should be considered [7]. The epicenter of the lesion favored clival chordoma, as our first provisional diagnosis, although the MRI intensity patterns favored pituitary adenoma rather than a chordoma.
A high index of suspicion is needed in diagnosing these lesions preoperatively. The clues in our patient were the sellar floor destruction and splaying of the sellar boundaries with scalloping of the clivus. This suggested an expanding chronic lesion rather than a destructive malignant lesion. The role of a dynamic MRI focusing on the tumour perfusion, microvascular permeability and volume of the extracellular space is very helpful [4]. Empty sella in such cases acts as a radiological pointer to the diagnosis. The empty sella here represents the Rathke’s pouch that fails to contact it’s anterior counterpart completely. Recent molecular researches highlights the role of (Wingless integrated) WNT signaling in ectopic pituitary adenoma and believe that the ectopic location is an independent risk factor for adenoma formation [2]. The immunohistochemistry may help in concluding the diagnosis as seen in our case. These ectopic adenomas may be functional or non secretory, the latter being more common. The possibility of clival chordoma in our case was kept on the basis of epicentre. The absence of calcification, residual fragments and minimal enhancement favoured the possibility of chordoma [5]. But the intraoperative nature of the tumor made us revisit the radiology. The sphenoidal ectopic adenoma had eroded clivus posteriorly and sellar floor superiorly. Yang BT et al also reported similar findings, where depending on the size of the lesions, they reported adjacent bone remodelling, sclerosis or erosion [5]. Unlike the sellarsuprasellar pituitary adenomas, the ectopic adenomas present unique opportunities of total excision with impunity and application of radiotherapy without much risk to the the hypothalamo-pituitary axis [6]. In conclusion, ectopic pituitary adenoma is a rare entity and high index of suspicion must be kept in the diferrential diagnosis of sphenoid sinus mass with MRI intensity pattern similar to pituitary adenomas. Declarations of interest None
References 1. Chandan
Jyoti
Goswami.American
Das, Ashu Journal
Seith, Shivanand of
Gamanagatti,
Roentgenology 2006;
186
and Ravinder (5):1468-1469.
https://doi.org/10.2214/AJR.05.0329 2. Thompson LD, Seethala RR, Müller S. Ectopic sphenoid sinus pituitary adenoma (ESSPA) with normal anterior pituitary gland: a clinicopathologic and immunophenotypic study of 32 cases with a comprehensive review of the English literature. Head Neck Pathol 2012 Mar;6(1):75-100. https://doi.org/doi:10.1007/s12105-012-0336-9. 3. Hong JF, Ding XH, Wang SS. Coexistence of ectopic pituitary adenoma and empty sella in a patient with acromegaly : a case report and review of literature.Neurol India 2012; 60(3):304-6. doi: https://doi.org/10.4103/0028-3886.98517. Review.PubMed PMID: 22824688. 4. Hori E, Akai T, Kurimoto M, Hirashima Y, Endo S. Growth hormone-secreting pituitary adenoma confined to the sphenoid sinus associated with a normal-sized empty sella. J Clin Neurosci 2002 Mar; 9(2):196-9. PubMed PMID: 11922715. 5. Yang BT, Chong VF, Wang ZC, Xian JF, Chen QH. Sphenoid sinus ectopic pituitary adenomas: CT and MRI findings. Br J Radiol 2010;83(987):218-24. 6. Matsuno A, Katayama H, Okazaki R, Toriumi M, Tanaka H, Akashi M, Tanaka K,Murakami M, Tanaka H, Nagashima T. Ectopic pituitary adenoma in the sphenoid sinus causing acromegaly associated with empty sella. ANZ J Surg. 2001 Aug;71(8):495-8. Review. PubMed PMID: 11504298. 7. Wang F, Zhang J, Wang P, Zhou T, Meng X, Jiang J. Prediction of pituitary stalk position in pituitary adenomas by visualization of the hypothalamo-hypophyseal tract using diffusion tensor imaging tractography. Medicine (Baltimore). 2018;97(10):e0052. doi:10.1097/MD.0000000000010052
Figure 1. T1 weighted post contrast coronal magnetic resonance imaging film of the head showing a homogenous well defined enhancing mass in sphenoid sinus (black arrow). While the right internal carotid artery (ICA) is free of the tumor, the left ICA appears surrounded (white arrow). The tumor is capped by a T1 hyperintensity supero-right laterally probably indicating the compressed clival bone marrow. A faint outline of the distorted pituitary stalk indicates the possible location of the pituitary gland supero-right laterally. The Computed Tomography scan shows eroded sellar sellar floor, anterior clinoids and a partially eroded dorsum sella (green arrow). Figure 2. Intraoperative photograph showing keel of sphenoid bone (black arrow) and tumor filled in sphenoid sinus (white arrow). The post operative T2 weighted sagittal imaging of head showing complete excision of tumor and sphenoid cavity filled with fat (grey arrow). Figure 3. Photomicrograph showing tumor disposed in sheets, trabeculae and nests of round to polygonal cells separated by thin fibrovascular septae. (A:X200) The cells show predominantly monomorphic nuclei with fine nuclear chromatin and scanty to moderate amount of cytoplasm. (B:X400). On immunohistochemistry, tumor cells show cells show Ki-67 < 1%.
S2214-7519(19)30004-0 https://doi.org/10.1016/j.inat.2019.100567 INAT 100567
To appear in:
Interdisciplinary Neurosurgery: Advanced Techniques and Case Management Interdisciplinary Neurosurgery: Advanced Techniques and Case Management
Received Date: Revised Date: Accepted Date:
10 December 2018 7 August 2019 14 August 2019
Please cite this article as: S. Singh, G. Attri, K. Kanti Das, S. Jaiswal, A. Kumar Jaiswal, Ectopic pituitary adenoma: The lost track of Ciurea or the right man at wrong place, Interdisciplinary Neurosurgery: Advanced Techniques and Case Management Interdisciplinary Neurosurgery: Advanced Techniques and Case Management (2019), doi: https://doi.org/10.1016/j.inat.2019.100567
This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
© 2019 Published by Elsevier B.V.
Ectopic pituitary adenoma: The lost track of Ciurea or the right man at wrong place Suyash Singha, Gagandeep Attria, Kuntal Kanti Dasa, Sushila Jaiswalb, Awadhesh Kumar Jaiswala,*[email protected] aDepartment
of Neurosurgery, Sanjay Gandhi Post Graduate Institute of Medical Sciences,
Lucknow, 226014, India bDepartment
of Pathology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow,
226014, India *Corresponding
author.
Highlights
Sphenoid sinus is the most common site for ectopic pituitary adenomas
Ectopic sphenoid sinus pituitary adenoma may present with nasal obstruction, headache, decreased vision or cerebrospinal fluid leak
The location per se is favourable for both surgeon and adjuvant radiation
Abstract The aberrant development of pituitary gland may leave functioning or nonfunctioning tissues anywhere along the craniopharyngeal canal. Ectopic pituitary adenoma is extremely rare with a reported incidence of only 0.5% in the literature. The sphenoid sinus is the most common site for ectopic pituitary adenomas. The preoperative diagnosis is often misleading with a normal “sella turcica” or “pituitary gland” with, no evidence of connection between the gland and the ectopic mass, being a pre requisite for diagnosis. Ectopic sphenoid sinus pituitary adenoma may present with nasal obstruction, headache, decreased vision or cerebrospinal fluid leak. The recent understanding in embryogenesis and effect of Wingless integrated (WNT) gene signaling suggest that ectopic glands may have a higher propensity to develop adenomas. We herein report a case of nonfunctional ectopic pituitary adenoma in the sphenoid sinus presenting with nasal stuffiness and highlight the key radiological findings.
Abbreviations
MRI, Magnetic resonance imaging; CT, computed tomography; WHO, World Health organization;
Keywords: Ectopic pituitary; Sphenoid sinus; empty sella
Introduction Pituitary adenoma, in the non-sellar location, suggests some underlying developmental anomaly. An associated empty sella, further confuses the treating surgeon about pathological nature of disease and the line of investigation. It is common for a non functional pituitary adenoma to enlarge, erode the sellar floor and reach sphenois sinus. It is further possible that, with progression, sellar part may undergo necrosis and present as an empty sella [1]. However, evidence of normal pituitary gland in the sella and a mass lesion in sphenoid sinus is a different situation. This ectopic pituitary adenoma may be secretory or non secretory. From the management point of view, the sphenoidal location per se is more favourable for both surgeon and the radiation oncologist. We, hereby, report a rare case of sphenoidal non secretory pituitary adenoma, presenting with nasal stuffiness, operated by endoscopic endonasal, approach and discuss the diagnostic dilemma we faced. Case Summary A 42-year old woman was referred to our institute with complaints of right sided nasal stuffiness for 2 months. Her visual acuity was 6/6 bilaterally and the extraocular movements also normal. Nasal examination was normal. On fundoscopy there was no papilloedema. Clinically, there was no endocrinological dysfunction. Computed Tomography (CT) scan of the head and the paranasal sinuses revealed a mass lesion in the sphenoid sinus extending into the upper 1/3 rd of the clivus with erosion of the sellar floor and an open book like sella turcica (Figure 1). Magnetic resonance Imaging (MRI) showed a rounded T2 hyperintense mass lesion present in the sphenoid sinus and upper 1/3 rd of the clivus with sellar extension (Figure 1). The sellar floor dura was apparently intact. Distortion of the pituitary stalk suggested a possible right superolateral displacement of the pituitary gland. Her hormonal profile
was within normal limits [serum prolactin 211.9 miu/l; serum IGF-1 70.8 ug/l; serum FSH 52.06 iu/l; serum 8 a.m. cortisol 408 nmol/l; serum TSH (thyrotropin 1.91 miu/l; and serum T4 (thyroxine) 116.0 nmol/l]. We operated her using an endoscopic endonasal transsphenoidal approach. We encountered a soft, free flowing tumor filling the sphenoid sinus (Figure 2). The mass was entirely extradural, having eroded the sella. The clivus was scalloped from within. After the mass was removed, the sellar floor dura was seen intact. A cruciate incision was made to inspect the sella where the normal pituitary gland was seen separately. The patient recovered uneventfully after surgery. The histopathological examination was suggestive of pituitary adenoma, showing tumor disposed in sheets, trabeculae and nests separated by thin fibrovascular septae, predominantly monomorphic nuclei and immunohistochemistry showed tumor cells show Ki-67 < 1%. (Figure 3) LCA and vimentin were negative and so were ACTH, GH and PRL receptors. Post operative MRI showed complete excision of tumour with sphenoid sinus filled with fat (Figure 2). Follow up hormonal work up was normal. Discussion Ectopic pituitary adenomas are rare entities, often misdiagnosed as neuroendocrine tumors, sinonasal carcinomas or even fungal infections. Other reported sites for ectopic pituitary adenomas are nasal cavity, ethmoid sinus, temporal bone, nasal bridge, meckel’s cave, cavernous sinus and thalamus [2]. The underlying cause for non-sellar location is an aberrant embryological developmental. The anterior lobe of the pituitary arises from the craniopharyngeal canal while the posterior pituitary is actually a part of the hypothalamus, descending in to the sella to combine with its counterpart [3]. In our patient, the pituitary stalk and the compressed posterior pituitary were seen superior to the lesion. The normal sellar structures are difficult to demonstrate in pathological conditions, particularly in pituitary macroadenimas and if it is seen on MRI separately from a well defined mass lesion, then an alternate diagnosis should be considered [7]. The epicenter of the lesion favored clival chordoma, as our first provisional diagnosis, although the MRI intensity patterns favored pituitary adenoma rather than a chordoma.
A high index of suspicion is needed in diagnosing these lesions preoperatively. The clues in our patient were the sellar floor destruction and splaying of the sellar boundaries with scalloping of the clivus. This suggested an expanding chronic lesion rather than a destructive malignant lesion. The role of a dynamic MRI focusing on the tumour perfusion, microvascular permeability and volume of the extracellular space is very helpful [4]. Empty sella in such cases acts as a radiological pointer to the diagnosis. The empty sella here represents the Rathke’s pouch that fails to contact it’s anterior counterpart completely. Recent molecular researches highlights the role of (Wingless integrated) WNT signaling in ectopic pituitary adenoma and believe that the ectopic location is an independent risk factor for adenoma formation [2]. The immunohistochemistry may help in concluding the diagnosis as seen in our case. These ectopic adenomas may be functional or non secretory, the latter being more common. The possibility of clival chordoma in our case was kept on the basis of epicentre. The absence of calcification, residual fragments and minimal enhancement favoured the possibility of chordoma [5]. But the intraoperative nature of the tumor made us revisit the radiology. The sphenoidal ectopic adenoma had eroded clivus posteriorly and sellar floor superiorly. Yang BT et al also reported similar findings, where depending on the size of the lesions, they reported adjacent bone remodelling, sclerosis or erosion [5]. Unlike the sellarsuprasellar pituitary adenomas, the ectopic adenomas present unique opportunities of total excision with impunity and application of radiotherapy without much risk to the the hypothalamo-pituitary axis [6]. In conclusion, ectopic pituitary adenoma is a rare entity and high index of suspicion must be kept in the diferrential diagnosis of sphenoid sinus mass with MRI intensity pattern similar to pituitary adenomas. Declarations of interest None
References 1. Chandan
Jyoti
Goswami.American
Das, Ashu Journal
Seith, Shivanand of
Gamanagatti,
Roentgenology 2006;
186
and Ravinder (5):1468-1469.
https://doi.org/10.2214/AJR.05.0329 2. Thompson LD, Seethala RR, Müller S. Ectopic sphenoid sinus pituitary adenoma (ESSPA) with normal anterior pituitary gland: a clinicopathologic and immunophenotypic study of 32 cases with a comprehensive review of the English literature. Head Neck Pathol 2012 Mar;6(1):75-100. https://doi.org/doi:10.1007/s12105-012-0336-9. 3. Hong JF, Ding XH, Wang SS. Coexistence of ectopic pituitary adenoma and empty sella in a patient with acromegaly : a case report and review of literature.Neurol India 2012; 60(3):304-6. doi: https://doi.org/10.4103/0028-3886.98517. Review.PubMed PMID: 22824688. 4. Hori E, Akai T, Kurimoto M, Hirashima Y, Endo S. Growth hormone-secreting pituitary adenoma confined to the sphenoid sinus associated with a normal-sized empty sella. J Clin Neurosci 2002 Mar; 9(2):196-9. PubMed PMID: 11922715. 5. Yang BT, Chong VF, Wang ZC, Xian JF, Chen QH. Sphenoid sinus ectopic pituitary adenomas: CT and MRI findings. Br J Radiol 2010;83(987):218-24. 6. Matsuno A, Katayama H, Okazaki R, Toriumi M, Tanaka H, Akashi M, Tanaka K,Murakami M, Tanaka H, Nagashima T. Ectopic pituitary adenoma in the sphenoid sinus causing acromegaly associated with empty sella. ANZ J Surg. 2001 Aug;71(8):495-8. Review. PubMed PMID: 11504298. 7. Wang F, Zhang J, Wang P, Zhou T, Meng X, Jiang J. Prediction of pituitary stalk position in pituitary adenomas by visualization of the hypothalamo-hypophyseal tract using diffusion tensor imaging tractography. Medicine (Baltimore). 2018;97(10):e0052. doi:10.1097/MD.0000000000010052
Figure 1. T1 weighted post contrast coronal magnetic resonance imaging film of the head showing a homogenous well defined enhancing mass in sphenoid sinus (black arrow). While the right internal carotid artery (ICA) is free of the tumor, the left ICA appears surrounded (white arrow). The tumor is capped by a T1 hyperintensity supero-right laterally probably indicating the compressed clival bone marrow. A faint outline of the distorted pituitary stalk indicates the possible location of the pituitary gland supero-right laterally. The Computed Tomography scan shows eroded sellar sellar floor, anterior clinoids and a partially eroded dorsum sella (green arrow). Figure 2. Intraoperative photograph showing keel of sphenoid bone (black arrow) and tumor filled in sphenoid sinus (white arrow). The post operative T2 weighted sagittal imaging of head showing complete excision of tumor and sphenoid cavity filled with fat (grey arrow). Figure 3. Photomicrograph showing tumor disposed in sheets, trabeculae and nests of round to polygonal cells separated by thin fibrovascular septae. (A:X200) The cells show predominantly monomorphic nuclei with fine nuclear chromatin and scanty to moderate amount of cytoplasm. (B:X400). On immunohistochemistry, tumor cells show cells show Ki-67 < 1%.