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1.  Value of anatomical landmarks in single-nostril endonasal transnasal-sphenoidal surgery 
The sphenoid sinus occupies a central location in transsphenoidal surgery (TSS). It is important to identify relevant anatomical landmarks to enter the sphenoid sinus and sellar region properly. The aim of this study was to identify anatomical landmarks and their value in single-nostril endonasal TSS. A retrospective study was performed to review 148 cases of single-nostril endonasal TSS for pituitary lesions. The structure of the nasal cavities and sphenoid sinus, the position of apertures of the sphenoid sinus and relevant arteries and the morphological characteristics of the anterior wall of the sphenoid sinus and sellar floor were observed and recorded. The important anatomical landmarks included the mucosal aperture of the sphenoid sinus, a blunt longitudinal prominence on the posterior nasal septum, the osseocartilaginous junction of the nasal septum, the ‘bow sign’ of the anterior wall of the sphenoid sinus, the osseous aperture and its relationship with the nutrient arteries, the bulge of the sellar floor and the carotid protuberance. These landmarks outlined a clear route to the sella turcica with an optimal view and lesser tissue damage. Although morphological variation may exist, the position of these landmarks was generally consistent. Locating the sphenoid sinus aperture is the gold standard to direct the surgical route of TSS. The ‘bow sign’ and the sellar bulge are critical landmarks for accurate entry into the sphenoid sinus and sella fossa, respectively.
doi:10.3892/etm.2013.936
PMCID: PMC3628397  PMID: 23596471
transsphenoidal approach; nasal cavity; sphenoid sinus; anatomical; landmark; pituitary
2.  Association of Cholesterol Granuloma and Aspergillosis in the Sphenoid Sinus 
Korean Journal of Radiology  2008;9(Suppl):S30-S33.
Cholesterol granuloma (CG) is usually associated with chronic middle ear disease, and is not common in the paranasal sinuses. Additionally, it is very rare for cases of CG to be associated with a fungal infection. However, in this paper, we report a case of sphenoid sinus CG that is associated with aspergilloma in a 78-year-old male patient who presented with right hemifacial pain, headache and toothache. CT revealed the presence of an expansile cystic mass lesion in the sphenoid sinus that showed a high signal intensity on both the T1 and T2 weighted images. This mass was later determined to be CG. The suspected etiologic mechanisms of both CG and aspergilloma of the paranasal sinuses are similar, and impaired drainage and obstruction of the ventilation of the paranasal sinuses are considered to be the causative mechanism of both diseases. Overall, the results of this study indicate that the use of MRI findings could be helpful for differentiating CG from other paranasal sinus mass lesions.
doi:10.3348/kjr.2008.9.s.s30
PMCID: PMC2627201  PMID: 18607122
Cholesterol granuloma; Aspergilloma; Sphenoid sinus
3.  Cavernous sinus thrombosis caused by contralateral sphenoid sinusitis: a case report 
Objective
To report a rare case of unilateral cavernous sinus thrombosis caused by contralateral sphenoid sinusitis.
Case report
A 33-year-old female visited our hospital for severe, right-sided, temporal headache, chemosis, periorbital edema, and proptosis. These signs were associated with congested erythematous nasal mucosa with purulent discharge from the right superior nasal meatus. Contrast enhanced CT showed dilated left superior ophthalmic vein, suggestive of thrombosis, contrast enhancement of the left cavernous sinuses, and dilation of cavernous sinus, indicating cavernous sinus inflammation. The right maxillary, ethmoid and sphenoid sinuses showed mucosal thickening and retention of purulent material. She was diagnosed with cavernous sinus thrombosis caused by contralateral sphenoid sinusitis. All clinical symptoms and signs improved after endoscopic sphenoidotomy and appropriate medical treatment.
Conclusions
Sphenoiditis can cause contralateral cavernous sinus thrombosis. Early surgical sphenoidotomy and aggressive medical treatment are the cornerstones of successful management of this life-threatening complication.
doi:10.1186/1746-160X-9-9
PMCID: PMC3605125  PMID: 23497466
Carvenous sinus thrombosis; Sphenoiditis; Contralaterally; Endoscopy sinus surgery
4.  Visual loss secondary to eosinophilic mucin rhinosinusitis in a woman: a case report 
Introduction
Eosinophilic mucin rhinosinusitis is an inflammatory pathological condition of the nose and paranasal sinuses. It is rare, occurs in immunocompetent patients and is characterised by peripheral eosinophilia and extensive bilateral sinus disease. To the best of our knowledge, visual loss with this condition has not been previously reported.
Case presentation
We present the case of a 26-year-old Asian woman with a background history of chronic sinusitis who presented with acute left-sided visual loss. Imaging showed significant opacification in the frontal, ethmoidal and sphenoidal sinuses as well as evidence of a unilateral optic neuritis. Histological analysis of sinus mucin revealed dense eosinophilic infiltrate and, despite medical and surgical intervention, vision was not restored in her left eye.
Conclusion
We introduce visual loss as a complication of eosinophilic mucin rhinosinusitis. This adds further evidence to previous reports in the literature that optic neuropathy in sinusitis can occur secondary to non-compressive mechanisms. We also describe a rare finding: the vision in this patient did not improve following steroid therapy, antifungal therapy or surgical intervention. There are very few such cases described in the literature. We conclude that chronic sinusitis is an indolent inflammatory process which can cause visual loss and we reiterate the importance of recognizing and considering sinusitis as a cause of visual loss in patients in order that prompt medical and surgical treatment of the underlying disease can be initiated.
doi:10.1186/1752-1947-4-350
PMCID: PMC2978232  PMID: 21034471
5.  Imaging Central Skull Base Neural Tumor Spread from Paranasal Sinus Malignancies 
Skull base surgery  1999;9(1):15-21.
Eight cases of paranasal sinus tumors with central skull base disease are presented. Each patient had coned down noncontrast and contrast-enhanced magnetic resonance imaging (MRI) studies to evaluate the tumor margins and any potential tumor spread into the cavernous sinuses, V2 and the Vidian nerve. Two patients had tumors that extended up to the face of the sphenoid bone and one had a tumor in the ventral cavernous sinus. These three patients had a complete tumor resection with postoperative irradiation. Two more patients had extensive tumor invasion of the central skull base, one was treated with only irradiation, one refused treatment. Three more patients had tumors that involved the ventral portion of the sphenoid bone with limited neural tumor extension. Two of these patients had surgery with cavernous sinus dissections followed by irradiation. One patient delayed treatment for 2 months and when he returned he had a more extensive tumor and was considered incurable by surgery. He refused further treatment. The technique of the focused MR examination and its impact on treatment planning is discussed.
Images
PMCID: PMC1656726  PMID: 17171077
6.  Ectopic acromegaly due to a GH-secreting pituitary adenoma in the sphenoid sinus: a case report and review of the literature 
BMC Research Notes  2013;6:411.
Background
In more than 98% of cases, acromegaly is due to a GH-secreting pituitary adenoma. The term “ectopic acromegaly” includes neuroendocrine tumors secreting GH releasing hormone (GHRH), usually located in the lungs, thymus and endocrine pancreas. Considerably less frequent are cases of ectopic acromegaly due to GH-secreting tumors located out of the pituitary fossa; except for one isolated case of a well-documented GH-secreting lymphoma, the majority of these lesions are located in the sphenoid sinus.
Case presentation
We present the case of a 45 year old woman with acromegaly whose MRI showed an empty sella without evidence of a pituitary adenoma but revealed a large mass within the sphenoid sinus. She underwent transsphenoidal surgery and the excised sphenoid sinus mass, proved to be a GH-secreting adenoma; the sellar floor was intact and no other lesions were found in the pituitary fossa. She required postoperative treatment with somatostatin analogs and cabergoline for clinical and biochemical control.
Conclusions
This case highlights the importance of carefully evaluating the structures surrounding the sellar area when a pituitary adenoma is not found with currently available imaging techniques. The finding of an intact sellar floor and duramater lead us to conclude that the patient’s tumor originated de novo from embryological pituitary remnants. Upon a careful review of the literature and a critical evaluation of our case we found neither clinical nor biochemical features that would distinguish an ectopic from the more common eutopically located somatotrophinoma.
doi:10.1186/1756-0500-6-411
PMCID: PMC3874769  PMID: 24119925
Ectopic acromegaly; Sphenoid sinus; GH-secreting adenoma
7.  Aggressive osteoblastoma of the sphenoid bone 
Oncology Letters  2010;1(2):367-371.
For osteoblastoma, with its predilection for the spinal column and appendicular skeleton, the skull is an unusual site, and paranasal sinus involvement is very rare. Herein, we report on a case in which the disease was located within the sphenoid bone. To the best of our knowledge, this is the 4th reported case of osteoblastoma with a sphenoid origin (1). We report an osteoblastoma of the sphenoid sinus in a 12-year-old girl who presented with exophthalmos. Computed tomography (CT) demonstrated an expansile lesion of the sphenoid which caused the orbital contents to be compressed and deviated to the right. In the magnetic resonance imaging scan, the lesion was found to invade the cranial base in the frontal and temporal region, approximating to the cavernous sinus and internal carotid artery on the right. Bilateral fronto-orbital craniotomy was performed. Histologically, the lesion was composed of proliferating osteoblasts along with vascular stroma. The tumor was described as an aggressive osteoblastoma. In the follow-up CT four months later, a pathological mass was observed in the area of the nasal septum, and a signal void was present on all sequences in the densely sclerotic areas. A second resection was performed. The patient has been disease-free for 61 months. Herein, we present the diagnosis and management of this unusual lesion. The histopathology and the imaging characteristics are shown.
doi:10.3892/ol_00000065
PMCID: PMC3436399  PMID: 22966310
sphenoid bone; osteoblastoma
8.  Temporary Unilateral Amaurosis with Pneumosinus Dilatans of the Sphenoid Sinus 
Skull base surgery  1994;4(3):169-175.
Pneumosinus dilatans (PSD), first described by Meyes in 1898, is an abnormal dilatation of one or more of the paranasal sinuses without bony erosion. The term sinus pneumocele indicates the presence of bony erosion. Review of the literature from 1968 to 1992 revealed 24 cases of idiopathic PSD and 17 cases of pneumocele of various paranasal sinuses. PSD occurred most commonly in the frontal sinus. Males were more often affected than females. The average age for males ranged from 16.5 for the maxillary sinus to 35.5 years for the sphenoid sinus. Pneumoceles occurred most commonly in the maxillary sinus. The average age for males ranged from 29 years for the frontal sinus and 47 years for the ethmoid sinus. Two patients (1 pneumocele and 1 PSD) had a temporary loss of vision. We report the case of a 37-year-old mountain climber who experienced temporary loss of vision in his left eye above 3000 m. Vision returned below 2000 m. High resolution computed tomography scan revealed pneumosinus dilatans of the sphenoid sinus with dehiscence of the optic canal on the left side. Endonasal microendoscopic resection of the anterior wall of the left sphenoid sinus was performed. The patient has remained symptom-free after 2 years of follow-up. Pneumosinus dilatans should always be considered in the differential diagnosis of sudden visual loss associated with atmospheric pressure changes.
Images
PMCID: PMC1661801  PMID: 17171168
9.  Aggressive juvenile fibromatosis of the paranasal sinuses: case report and brief review 
Desmoid fibromatoses are benign, slow growing fibroblastic neoplasms, arising from musculoaponeurotic stromal elements. Desmoids are characterized by local invasion, with a high rate of local recurrence and a tendency to destroy adjacent structures and organs. Desmoid fibromatoses are rare in children, and though they may occur in the head and neck region, are extremely rare in the paranasal sinuses. Here we report a case of extraabdominal desmoid fibromatosis in a seven-year-old boy involving the sphenoid sinus, one of only six published reports of desmoid fibromatosis of the paranasal sinuses. The expansile soft tissue mass eroded the walls of the sphenoid sinus as well as the posterior ethmoid air cells extending cephalad through the base of the skull. We discuss the clinicopathologic features of this lesion, including structural and ultrastructural characteristics, and we review the literature regarding treatment and outcome.
doi:10.1186/1756-8722-1-3
PMCID: PMC2438440  PMID: 18577255
10.  Sinonasal Hemangiopericytoma 
Skull base surgery  1995;5(3):185-190.
Hemangiopericytoma (HPC) is a rare vascular tumor that can arise in any organ system, but occurs most frequently in skeletal muscle. We present a case of a primary sphenoid sinus HPC unusual for its spontaneous remission after biopsy. There have been approximately 55 cases of sinonasal HPC reported in the literature, representing less than 5% of all lesions. In general, HPC behaves aggressively, demonstrating greater than 50% local recurrence and 10% metastatic disease. Although extended surgical resection is traditionally considered the most effective therapy for all HPC, critical literature review does not support this method of treatment for HPC occurring in the paranasal sinuses and skull base. Long-term follow-up indicates that the majority of sinonasal HPC have a benign clinical course regardless of treatment. As a result, we do not recommend extended resection as the initial therapy for sinonasal HPC. Instead, a period of observation coupled with serial MRI or CT scans should be used to detect tumor progression. These indolent tumors should undergo extended resection only after progression has been confirmed. In addition to the case report, a synopsis of the reviewed literature and a summary of treatment recommendations are also presented.
Images
PMCID: PMC1656489  PMID: 17170946
11.  Sphenoid Sinus Basaloid Squamous Cell Carcinoma Presenting as a Sellar Mass: Report a Case with Review of the Literature 
Head and Neck Pathology  2010;5(1):81-85.
Basaloid squamous cell carcinoma (BSCC) is a distinctive variant of squamous cell carcinoma (SCC) with more aggressive behavior. It occurs preferentially in the upper aerodigestive tract. Sinonasal tract BSCC is uncommon, and only limited studies have been reported in literature. In these studies, most BSCCs arose from the nasal mucosa with or without extension to the paranasal sinuses. Rare reported cases of BSCC involved only the paranasal sinus. In this report, we present a case of a female patient with a sphenoid sinus mass. Clinically, the patient had progressively decreasing vision and headache. Magnetic resonance imaging (MRI) and computerized tomographic (CT) scan showed an infiltrating tumor mass involving the sphenoid sinus and the sella with compression of the optic nerve. Pathologic examination revealed an invasive basaloid epithelial neoplasm that was arranged in lobules, nests and cords. The tumor also showed palisading of peripheral cells, focal abrupt squamous differentiation and in situ carcinoma in the surface mucosa. In the immunohistochemical studies, this tumor revealed a strongly positive nuclear staining for p63. The morphologic and ancillary studies indicated a BSCC. To the best of our knowledge, this is the first report of sinonasal tract BSCC that mainly involved the sphenoid bone and sella. In this region, BSCC should be distinguished from benign and malignant neoplasms that more often affect sella and base of skull, such as pituitary adenoma with extensive necrosis, small cell neuroendocrine carcinoma (SCNC), olfactory neuroblastoma, malignant germ cell tumor, paranasal adenoid cystic carcinoma (ACC), and a variety of metastatic malignancies.
doi:10.1007/s12105-010-0214-2
PMCID: PMC3037465  PMID: 20972844
Basaloid squamous cell carcinoma; Sphenoid sinus; Sellar mass
12.  Rapid Development of Bilateral Internal Carotid Artery Aneurysm from Sphenoid Sinus Aspergillosis 
Skull base surgery  1998;8(4):211-214.
The purpose of this case report is to familiarize the sinus surgeon with the possibility of the rapid development of internal carotid artery aneuryams from fungal infections of the sphenoid sinuses. A renal dialysis patient with progressive loss of vision was treated with high doses of steroids for the presumed diagnosis of temporal arteritis. Subsequent work-up included a magnetic resonance arteriogram (MRA) and computed tomography (CT) with contrast that failed to demonstrate aneurysmal changes of the carotid arteries but suggested the presence of a mycotic infection of the sphenoid sinuses. During a sphenoidotomy two days later, in addition to the anticipated aspergillus infection of the sinuses, an aneurysm extending from the left internal carotid artery into the sphenoid sinus was encountered. An emergency arteriogram immediately following the surgery revealed a second newly developed large mycotic aneurysm of the right internal carotid artery filling the right sphenoid sinus as well. This case report documents the rapidity with which mycotic aneurysms can develop from a sphenoid sinus infection secondary to aspergillosis in an immunocompromised host.
Images
PMCID: PMC1656709  PMID: 17171068
13.  Isolated Sphenoid Pyocele with Thornwaldt’s Cyst of Nasopharynx 
Isolated sphenoid sinusitis (ISS) is a rare entity. ISS accounts for about 1–2% of all sinus infections. Isolated sphenoid sinus involvement may include mucoceles, pyoceles and isolated mycotic infections. We report a case of isolated sphenoid pyocele in a 35 year-old female who presented in August 2007 with frontal and occipital headache, post nasal discharge and bilateral nasal obstruction for 6 years. CT scan showed isolated right sphenoid sinusitis. Diagnostic nasal endoscopy showed a streak of post nasal discharge on right side above the torus tubaris confirming right sphenoiditis. A cyst in the nasopharynx was incidentally detected which was confirmed by fluid aspiration to be Thornwaldt’s cyst (TC). TCs have reported prevalence of 0.2–5% and though most are asymptomatic their location renders them vulnerable to infection. Sphenoidotomy was done and the cyst in the nasopharynx was removed and marsupialised. Postoperative course was uneventful.
doi:10.1007/s12070-011-0256-0
PMCID: PMC3146695  PMID: 22754866
Sphenoid pyocele; Thornwaldt’s cyst; Nasopharyngeal bursa
14.  New Landmark for the Endoscopic Endonasal Transsphenoidal Approach of Pituitary Surgery 
Objective
To clarify the anatomical correlations of the sphenoid sinus with surrounding structures in the normal Korean population, and to identify surgical landmarks for safe sellar floor dissection in the anterior skull base by endoscopy and microscopy.
Methods
We reviewed the 196 brain magnetic resonance imaging findings showing a normal appearance, and measured the distances between anatomical landmarks.
Results
The mean distances from the base of the columella to the anterior wall of the sphenoid sinus and the sellar floor were 69.71±4.25 mm and 86.26±4.57 mm, respectively in the over 15 age group, and showed the smallest degree of variation among the measurements. The mean angles between the floor of the nasal cavity and the straight line connecting the base of the columella and the sellar floor were 29.45±3.25° and 24.75±4.00° in the over 15 and under 15 age groups, respectively. The mean values of both distances and angles increased with age until 15 years after which no further increases were evident. There were no significant differences in the measurements between males and females or among subjects with different degrees of pneumatization in the over 15 age group.
Conclusion
The distances from the base of the columella to the sellar floor and the anterior wall of the sphenoid sinus, which were consistent among individuals, could be used as a surgical indicator to investigate the sellar floor in endoscopic or microscopic transsphenoidal approaches.
doi:10.3340/jkns.2013.53.4.218
PMCID: PMC3698231  PMID: 23826477
Pituitary gland; Neuroendoscopy; Sphenoid sinus; Skull base neoplasm
15.  Sphenoid Cranial Base Defects in Siblings Presenting with Cerebrospinal Fluid Leak 
Skull base surgery  1997;7(4):193-197.
Two sisters presented to our medical center with nontraumatic cerebrospinal fluid (CSF) fistulas from left sphenoid sinocranial junction defects. One sister had recurrent meningitis over a 20-year period that prompted a skull base evaluation. Four years later, her younger sister presented with profuse CSF rhinorrhea. Transethmoid sphenoidotomy with sinus obliteration and lumbar-subarachnoid temporary CSF diversion successfully treated one sister, while the other required reoperation and permanent lumbar-peritoneal shunting. In both cases the skull base defect was identically located in the posterolateral left sphenoid sinus. Embryological considerations, evaluation and management are presented.
Images
PMCID: PMC1656647  PMID: 17171030
16.  An unusual case of a pituitary fossa aspergilloma in an immunocompetent patient mimicking infiltrative tumour 
Journal of Surgical Case Reports  2013;2013(4):rjt018.
Sellar aspergillosis is a rare infection commonly mistaken for a pituitary tumour. We present a rare case of pituitary fossa Aspergillus fumigatus mycetoma in an immunocompetent 90-year-old female, who presented with headaches. Magnetic resonance imaging scans demonstrated an enhancing pituitary fossa mass that appeared to infiltrate the sphenoid sinus, suggestive of an invasive tumour. Stereotactic trans-sphenoidal resection confirmed localized A. fumigatus infection. The abscess was debrided and the dura was left intact. Her headaches resolved post-operatively and she was treated with voriconazole. This indicates that aspergilloma should be considered as a differential for an unexplained pituitary lesion even in elderly immunocompetent patients.
doi:10.1093/jscr/rjt018
PMCID: PMC3635217
17.  Pituitary infiltration by non-Hodgkin's lymphoma: a case report 
Introduction
Pituitary adenomas represent the most frequently observed type of sellar masses; however, the presence of a rapidly growing sellar tumor, diabetes insipidus, ophthalmoplegia and headaches in an older patient strongly suggests metastasis to the pituitary. Since the anterior pituitary has a great reserve capacity, metastasis to the pituitary and pituitary involvement in lymphoma are usually asymptomatic. Whereas diabetes insipidus is the most frequent symptom, patients can present with headaches, ophthalmoplegia and bilateral hemianopsia.
Case presentation
A 70-year-old woman with no previous history of malignancy presented with headaches, right oculomotor nerve palsy and diabetes insipidus. As magnetic resonance imaging revealed a sellar mass involving the pituitary gland and infundibular stalk, which also extended into the right cavernous sinus and sphenoid sinus, the patient underwent an immediate transsphenoidal decompression surgery. Her prolactin was 102.4 ng/ml, whereas her gonadotropic hormone levels were low. A low level of urine osmolality after overnight water deprivation, along with normal plasma osmolality suggested diabetes insipidus. Histological examination revealed that the mass had been the infiltration of a high grade B-cell non-Hodgkin's lymphoma involving respiratory system epithelial cells. Paranasal sinus computed tomography scanning and magnetic resonance imaging of the thorax and abdomen were performed. Since magnetic resonance imaging did not reveal any abnormality, after paranasal sinus computed tomography was performed, we concluded that the primary lymphoma originated from the sphenoid sinus and infiltrated the pituitary. Chemotherapy and radiotherapy to the sellar area were planned, but the patient died and her family did not permit an autopsy.
Conclusion
Lymphoma infiltration to the pituitary is difficult to differentiate from pituitary adenoma, meningioma and other sellar lesions. To plan the treatment of lymphoma infiltration of the pituitary gland, it must be differentiated from other sellar lesions.
doi:10.1186/1752-1947-3-9293
PMCID: PMC2803816  PMID: 20062782
18.  Anatomic Variations of the Sphenoid Sinus and Their Impact on Trans-sphenoid Pituitary Surgery 
Skull Base  2008;18(1):9-15.
ABSTRACT
Introduction: The trans-sphenoid access to the pituitary gland is becoming the most common approach for pituitary adenomas. Preoperative evaluation of the anatomy of the sphenoid sinus by computed tomography (CT) scan and magnetic resonance imaging (MRI) is a routine procedure and can direct the surgical decision. Purpose: This work determines the incidence of the different anatomical variations of the sphenoid sinus as detected by MRI and CT scan and their impact on the approach. Methods: The CT scan and MRI of 296 patients operated for pituitary adenomas via a trans-sphenoid approach were retrospectively reviewed regarding the different anatomical variations of the sphenoid sinus: degree of pneumatization, sellar configuration, septation pattern, and the intercarotid distance. Results: There were 6 cases with conchal pneumatization, 62 patients with presellar, 162 patients with sellar, and 66 patients with postsellar pneumatization. There was sellar bulge in 232 patients, whereas this bulge was absent in 64 patients. There was no intersphenoid sinus septum in 32 patients, a single intersphenoid septum in 212 patients, and an accessory septum in 32 patients. Intraoperatively, the sellar bulge was marked in 189 cases and was mild in 43 cases. Discussion: The pattern of pneumatization of the sphenoid sinus significantly affects the safe access to the sella. A highly pneumatized sphenoid sinus may distort the anatomic configuration, so in these cases it is extremely important to be aware of the midline when opening the sella to avoid accidental injury to the carotid and optic nerves. The sellar bulge is considered one of the most important surgical landmarks, facilitating the access to the sella. The surgical position of the patient is also a crucial point to avoid superior or posterior misdirection with resultant complications. It is wise to use extreme caution while removing the terminal septum. Conclusion: Different anatomical configurations of the sphenoid sinus can seriously affect the access to the sella via the nose. The surgeon should be aware of these findings preoperatively to reach the sella safely and effectively.
doi:10.1055/s-2007-992764
PMCID: PMC2435468  PMID: 18592020
Sphenoid sinus; pneumatization; pituitary adenoma; trans-sphenoid approach
19.  Paranasal Sinus Neuroendocrine Carcinoma: A Case Report and Review of the Literature 
Neuroendocrine neoplasms are defined as epithelial neoplasms with predominant neuroendocrine differentiation. They can arise in almost every organ of the body although they are most commonly found in the gastrointestinal tract and respiratory system. Nasal cavity and paranasal sinuses are a rare site for neuroendocrine carcinoma. In contrast to the other regions, neuroendocrine tumours of the sinuses have been reported to be recurrent and locally destructive. Very few cases of paranasal sinus neuroendocrine carcinoma have been reported till date. Difficulty in pathologic diagnosis and rarity of this malignancy have hindered the progress in understanding the clinical course and improving outcomes. We herein report a case of poorly differentiated neuroendocrine tumour of ethmoid and sphenoid sinus with invasion of orbit and intracranial extension. The patient had complete response at the end of chemoradiation and he was disease-free for 9 months duration after which he developed bone metastasis without regional recurrence.
doi:10.1155/2013/728479
PMCID: PMC3586447  PMID: 23476846
20.  Obstructive Hydrocephalus, Fifth Nerve and Hypothalamus Involvement: Acute Presentation of a Giant Prolactinoma 
Introduction
Pituitary tumors from lactotrope cells account for about 40% of all functioning pituitary cancers. Men tend to present with a larger, more invasive and rapid growth prolactinomas than women, possibly because hypogonadism features are less evident.
Case report
A 27-year-old, previously asymptomatic Saudi man presented with a 3-day history of emesis with severe left-sided frontal headache, left face and right upper limb numbness, with signs of obstructive hydrocephalus. Brain Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) revealed a giant pituitary mass occupying several regions (sellar, infra-sellar, and supra-sellar) measuring 6.5 × 5.7 × 5.9 cm, and invading the sphenoid sinus as well as the cavernous sinuses bilaterally, with intra-pituitary hemorrhage compressing the third ventricle causing obstructive hydrocephalus. Prolactin levels were >200,000 mIU/L, consistent with invasive giant prolactinoma (IGP). He was treated with Cabergoline which eventually normalized the prolactin level and significantly reduced the size of IGP.
Conclusion
This is a rare case of obstructive hydrocephalus with super-imposed intra-pituitary hemorrhage secondary to IGP, highlighting the importance of a full hormonal assessment for proper diagnosis and management.
doi:10.4137/CCRep.S9675
PMCID: PMC3408871  PMID: 22855635
(IGP) invasive giant prolactinoma; obstructive hydrocephalus
21.  Extracranial Infrasellar Ectopic Craniopharyngioma: A Case Report and Review of the Literature 
Skull Base  2010;20(6):475-480.
We present a case of a purely infrasellar craniopharyngioma that initially presented as a sphenoid sinus mass. Craniopharyngiomas are usually located within the sella. Purely infrasellar craniopharyngiomas have only rarely been reported in the literature. A 25-year-old woman presented with 6-month history of progressive headaches. Initial neuroimaging revealed the presence of a sphenoid sinus mass. Initially, she underwent an endoscopic biopsy of the mass by our ENT service. Pathology was consistent with craniopharyngioma and she was referred to neurosurgery for further surgical management. She then underwent an endoscopic transsphenoidal approach for complete resection of the purely extracranial, infrasellar craniopharyngioma. The Rathke pouch arises from the roof of the primitive mouth and grows toward the brain at the fourth week of gestation. Normally, it loses its attachment with the stomadeum completely by the eighth week of gestation. The craniopharyngeal canal (CPC) extends from the floor of the sella to the vomer and may rarely give rise to ectopic craniopharyngiomas. This case shows that such ectopic tumors may arise anywhere along the CPC. Endoscopic endonasal approach provides an excellent route for the resection of these tumors.
doi:10.1055/s-0030-1261269
PMCID: PMC3134813  PMID: 21772808
Craniopharyngioma; ectopic; Rathke pouch; infrasellar
22.  A patient presenting with concha bullosa in another concha bullosa: a case report 
Introduction
Anatomic variations of the paranasal sinuses are very common. The paranasal sinus anatomy should be carefully examined prior to performing endoscopic sinus surgery in terms of both existent pathologies and anatomic variations. The anatomy of the paranasal sinuses and its variations have gained importance, along with advances in coronal paranasal sinus computed tomography and extensive use of endoscopic sinus surgery.
Case presentation
A 53-year-old Caucasian woman was admitted to our clinic with complaints of nasal breathing difficulties and headache persisting for a long time. Another concha bullosa was detected in the middle concha bullosa on preoperative paranasal computed tomography examination. It is known that the paranasal sinuses have a number of anatomical variations.
Conclusion
Herein we report a rare case, along with a review of the literature, to emphasize that a concha bullosa inside another concha bullosa should not be ignored.
doi:10.1186/1752-1947-6-87
PMCID: PMC3338398  PMID: 22448660
23.  Sphenoid Sinus Myxoma: Case Report and Literature Review 
The Ochsner Journal  2008;8(4):166-171.
Objectives: We present the first known case in the English-language literature of a myxoma arising in the sphenoid sinus. By describing the patient's clinical course and the salient features of this rare neoplasm, we seek to increase the awareness of the presentation, histological features, and treatment considerations for myxomas of the head and neck. In the process, we intend to describe the work-up of isolated sphenoid sinus lesions and focus on the varying and evolving techniques for surgical access to the sphenoid sinus.
Study Design and Methods: Case report and literature review.
Results: We describe the clinical course of a patient with a myxoma of the sphenoid sinus. The patient underwent an external sphenoethmoidectomy through a lateral rhinotomy approach with medial maxillectomy under MRI-guidance. He remains without evidence of recurrent disease after 8 months.
Conclusions: Myxomas of the head and neck are rare neoplasms. Their infiltrative nature and tendency to recur demand an aggressive surgical approach that may be accomplished with minimal morbidity using currently available image-guided techniques.
PMCID: PMC3096368  PMID: 21603497
Myxoma; neoplasms; sphenoid sinus
24.  Sinusitis: A Rare Cause for Galactorrhoea 
Case Reports in Otolaryngology  2012;2012:981375.
A 32-year-old woman presented to the endocrinology clinic with recent onset galactorrhoea. Investigations revealed raised prolactin levels. An MRI scan demonstrated a normal pituitary gland, and an incidental finding of sphenoid sinusitis with expansion of the sphenoid sinus was thought to be due to a mucocele. It is postulated that either the direct local pressure by the mucocele or localised inflammation secondary to sinusitis might cause hyperprolactinaemia. The patient underwent endoscopic surgery to drain the mucocele, after which her galactorrhoea resolved. A review of the literature reveals only one previously documented case of sinusitis causing hyperprolactinaemia and galactorrhoea.
doi:10.1155/2012/981375
PMCID: PMC3504374  PMID: 23198229
25.  Non-Hodgkin's lymphoma of the sphenoid sinus presenting as isolated oculomotor nerve palsy 
Background
Solitary involvement of the sphenoid sinus has rarely been reported in non-Hodgkin's lymphoma. Isolated oculomotor nerve palsy is uncommon as an initial presentation of malignant tumors of the sphenoid sinus.
Case presentation
A 53-year-old woman presented with a three-month history of headache and diplopia. Neurological examination revealed complete left oculomotor nerve palsy. Magnetic Resonance Imaging (MRI) demonstrated a homogenous soft-tissue lesion occupying the left sphenoid sinus and invading the left cavernous sinus. The patient underwent transsphenoidal biopsy and the lesion was histologically diagnosed as non-Hodgkin's lymphoma, diffuse large B-cell type. Tumor cells were positive for CD20 and negative for CD3. Following six cycles of chemotherapy, the left oculomotor nerve palsy that had been previously observed was completely resolved. There was no enhancing lesion noted on follow-up MRI.
Conclusion
It is important to recognize that non-Hodgkin's lymphoma of the sphenoid sinus can present with isolated oculomotor nerve palsy, although it is extremely rare. The cranial nerve deficits can resolve dramatically after chemotherapy.
doi:10.1186/1477-7819-5-86
PMCID: PMC1950883  PMID: 17683562

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