Intrasellar chordoma masquerading as a pituitary neuroendocrine tumor: Illustrative case.

Background: Chordomas are rare, locally aggressive neoplasms recognized as derivatives of the notochord vestiges. These tumors typically involve the midline axial skeleton, and intracranial chordomas exhibit proclivity for the spheno-occipital region. However, purely intrasellar occurrences are extremely rare. We report a case of intrasellar chordoma, which masqueraded as a pituitary neuroendocrine tumor. Case Description: An 87-year-old female presented with an acutely altered mental state after a few-week course of headaches and decreased left vision. Adrenal insufficiency was evident, and magnetic resonance imaging revealed an intrasellar lesion with heterogeneous contrast enhancement and marked T2 hyperintensity. Central adrenal insufficiency due to an intrasellar lesion was suspected. Cortisol replacement was initiated, and transsphenoidal surgery was performed. Anterosuperior displacement of the normal pituitary gland and the absence of the bony dorsum sellae were notable during the procedure. Histological examination led to a diagnosis of conventional chordoma, and upfront adjuvant stereotactic radiosurgery was executed. She has been free from tumor progression for 12 months. Conclusion: This case and literature review suggested that the pathognomonic features of intrasellar chordoma were heterogeneous contrast enhancement, marked T2 hyperintensity, osteolytic destruction of the dorsum sellae, and anterosuperior displacement of the pituitary gland. Clinical outcomes seemed slightly worse than those of all skull base chordomas, which were the rationale for upfront radiosurgery in our case. Neurosurgeons should include intrasellar chordomas in the differential diagnosis of intrasellar lesions, carefully dissect them from the adjacent critical anatomical structures, and consider upfront radiosurgery to achieve optimal patient outcomes.

Extracapsular Dissection With Narrow-Band Imaging Using the Transnasal Endoscopic Tri-port Approach for Extracranial Trigeminal Schwannoma: A Case Report.

The pterygopalatine fossa and infratemporal fossa are often approached through an external incision because of their deep facial location, but this can present problems such as facial scarring and deformity. In schwannoma surgery, intraneural dissection is a useful surgical technique for achieving gross total resection while preserving the capsule, including the nerves. For appropriate enucleation and preservation of the functional nerve, it is indispensable to distinguish between the pseudocapsule and the tumor capsule. This case report presents a case of endonasal surgical intervention for an extracranial trigeminal schwannoma employing the tri-port approach and narrow-band imaging.

Gross-total resection in optic nerve sheath meningiomas: minimally invasive and cosmetic pleasing.

PURPOSE: The optic nerve sheath meningioma (ONSM) is one of the most challenging tumors in orbital surgery. From the perspective of mental health and patient needs, we analyzed the necessity and importance of the endoscopic transnasal approach (ETA) combined with optic nerve transection (ONT) in gross-total resection (GTR) in ONSM patients with residual vision and aim to broaden the use of ONT for specific people. METHODS: The authors included patients with ONSMs who were treated between 2014 and 2022. We divided those cases into two groups named ETA group and lateral orbitotomy approach (LOA) group. We present the application of ETA and analyze the preoperative indication of the ONT and compared the advantages and disadvantages between ETA and LOA. The degree of tumor resection was based on imaging and surgical evaluation. RESULTS: A total of 23 patients with ONSM were included. Sixteen patients underwent ETA, and seven underwent LOA. Among ETA cases, GTR was achieved in 14 patients with ONT and most patients maintained normal eye movement function (75%) and morphology (93.75%). In the ETA group, 14 patients experienced vision loss, while two other patients saw improvements in vision. And proptosis was alleviated (5.20 ± 2.34 vs 0.27 ± 0.46, p < 0.0001). Six patients with blindness and proptosis of the LOA group resulted in GTR with ONT and ophthalmectomy. Although intracranial extension and recurrence included no cases in the two groups, a significant psychological gap was presented due to cosmetic problems. CONCLUSIONS: Under the premise of reducing damage and improving aesthetics, the selection of ETA combined with ONT to gross-total resect ONSMs successfully provides a minimally invasive access with acceptable complications. As an important adjunct to GTR in the surgical treatment of ONSM, the scope of ONT application should be expanded to relieve the patient's psychological burden.

Endoscopic Transnasal Management of Meningoceles and Encephaloceles in Children: A Systematic Review.

OBJECTIVE: To review surgical techniques used in the endoscopic transnasal repair of pediatric basal meningoencephaloceles and compare perioperative outcomes in children <2 and ≥2 years old. DATA SOURCES: MEDLINE, EMBASE, and CENTRAL. REVIEW METHODS: Data sources were searched from inception to August 22, 2022, using search terms relevant to endoscopic transnasal meningoencephalocele repair in children. Reviews and Meta-analyses were excluded. Primary outcomes were the incidence of intraoperative and postoperative complications, including cerebrospinal fluid leak, recurrence, and reintervention. Quality assessments were performed using Newcastle-Ottawa Scale, ROBIN-I, and NIH. RESULTS: Overall, 217 patients across 61 studies were identified. The median age at surgery was 4 years (0-18 years). Fifty percent were female; 31% were <2 years. Most defects were meningoencephaloceles (56%), located transethmoidal (80%), and of congenital origin (83%). Seventy-five percent of repairs were multilayered. Children ≥2 years underwent multilayer repairs more frequently than those <2 years (P = 0.004). Children <2 years more frequently experienced postoperative cerebrospinal fluid leaks (P = 0.02), meningoencephalocele recurrence (P < 0.0001), and surgical reintervention (P = 0.005). Following multilayer repair, children <2 years were more likely to experience recurrence (P = 0.0001) and reintervention (P = 0.006). CONCLUSION: Younger children with basal meningoencephaloceles appear to be at greater risk of postoperative complications following endoscopic endonasal repair, although the quality of available evidence is weakened by incomplete reporting. In the absence of preoperative cerebrospinal fluid leak or meningitis, it may be preferable to delay surgery as access is more conducive to successful repair in older children.

Profiles of central nervous system surgical site infections in endoscopic transnasal surgery exposing the intradural space.

OBJECTIVE: Despite its efficacy and minimal invasiveness, the clean-contaminated nature of endoscopic transnasal surgery (ETS) may be susceptible to central nervous system surgical site infections (CNS-SSIs), especially when involving intradural exposure. However, the profiles of ETS-associated CNS-SSIs are not fully elucidated. METHODS: The institutional ETS cases performed between May 2017 and March 2023 were retrospectively analysed. The incidences of CNS-SSIs were calculated, and their risk factors examined. RESULTS: The incidence of CNS-SSIs was 2.3% (7/305) in the entire cohort and 5.0% (7/140) in ETSs with intradural exposure. All the CNS-SSIs were meningitis and developed following ETS with intradural exposure. The incidences were 0%, 5.6% and 5.8% in ETSs with Esposito grade 1, 2 and 3 intraoperative cerebrospinal fluid leakage, respectively. Among the pre- and intra-operative factors, body mass index (unit odds ratio (OR), 0.62; 95% confidence interval (CI), 0.44-0.89; P<0.01), serum albumin (unit OR, 0.03; 95% CI, 0.0007-0.92; P=0.02), and American Society of Anesthesiologists physical status score (unit OR, 20.7; 95% CI, 1.65-259; P<0.01) were significantly associated with CNS-SSIs. Moreover, postoperative cerebrospinal fluid leakage was also significantly associated with CNS-SSIs (OR, 18.4; 95% CI, 3.55-95.0; P<0.01). CONCLUSIONS: The incidence of ETS-associated CNS-SSIs is acceptably low. Intradural exposure was a prerequisite for CNS-SSIs. Malnutrition and poor comorbidity status should be recognized as important risks for CNS-SSIs in ETS.

Primary Intracranial Adenoid Cystic Carcinoma: Report of Three Cases.

Objective Nasal cavity and paranasal sinuses host a variety of malignant tumors with adenoid cystic carcinoma (ACC) being the most frequent cancer of salivary gland origin. The histological origin of such tumors virtually precludes primarily intracranial localization. The aim of this study is to report cases of primarily intracranial ACC without evidence of other primary lesions at the end of an exhaustive diagnostic workup. Methods An electronic medical record search complemented by manual searching was conducted to identify prospective and retrospective cases of intracranial ACCs treated in Endoscopic Skull Base Centre Athens at the Hygeia Hospital, Athens from 2010 until 2021 with a mean follow-up time of at least 3 years. Patients were included if after complete diagnostic workup there was no evidence of a nasal or paranasal sinus primary lesion and extension of the ACC. All patients were treated with a combination of endoscopic surgeries performed by the senior author followed by radiotherapy (RT) and/or chemotherapy. Results Three unique illustrative cases (ACC involving the clivus, cavernous sinus and pterygopalatine fossa, one orbital ACC with pterygopalatine fossa and cavernous sinus involvement and one involving cavernous sinus, and Meckel's cave with extension to the foramen rotundum) were identified. All patients underwent subsequently proton or carbon-ion beam radiation therapy. Conclusions Primary intracranial ACCs constitute an extremely rare clinical entity with atypical presentation, challenging diagnostic workup and management. The design of an international web-based database with a detailed report of these tumors would be extremely helpful.

COVID-19 nasopharyngeal swab and cribriform fracture.

Since the start of the pandemic, over 400 million COVID-19 swab tests have been conducted in the UK with a non-trivial number associated with skull base injury. Given the continuing use of nasopharyngeal swabs, further cases of swab-associated skull base injury are anticipated. We describe a 54-year-old woman presenting with persistent colourless nasal discharge for 2 weeks following a traumatic COVID-19 nasopharyngeal swab. A ß2-transferrin test confirmed cerebrospinal fluid (CSF) rhinorrhoea and a high-resolution sinus computed tomography (CT) scan demonstrated a cribriform plate defect. Magnetic resonance imaging showed radiological features of idiopathic intracranial hypertension (IIH): a Yuh grade V empty sella and thinned anterior skull base. Twenty-four hour intracranial pressure (ICP) monitoring confirmed raised pressures, prompting insertion of a ventriculoperitoneal shunt. The patient underwent CT cisternography and endoscopic transnasal repair of the skull base defect using a fluorescein adjuvant, without complications. A systematic search was performed to identify cases of COVID-19 swab-related injury. Eight cases were obtained, of which three presented with a history of IIH. Two cases were complicated by meningitis and were managed conservatively, whereas six required endoscopic skull base repair and one had a ventriculoperitoneal shunt inserted. A low threshold for high-resolution CT scanning is suggested for patients presenting with rhinorrhoea following a nasopharyngeal swab. The literature review suggests an underlying association between IIH, CSF rhinorrhoea and swab-related skull base injury. We highlight a comprehensive management pathway for these patients, including high-resolution CT with cisternography, ICP monitoring, shunt and fluorescein-based endoscopic repair to achieve the best standard of care.

Long-term Outcomes of Non-vascularized Multilayer Fascial Closure Technique for Dural Repair in Endoscopic Transnasal Surgery: Efficacy, Durability, and Limitations.

OBJECTIVE: Non-vascularized multilayer fascial closure technique (NMFCT) can be used instead of nasoseptal flap reconstruction for dural repair in endoscopic transnasal surgery (ETS); however, due to the lack of blood supply, its long-term durability and possible limitations need to be clarified. METHODS: This was a retrospective study on patients who underwent ETS with intraoperative cerebrospinal fluid (CSF) leakage. We assessed the postoperative and delayed CSF leakage rates and the associated risk factors. RESULTS: Among 200 ETSs with intraoperative CSF leakage, 148 (74.0%) ETSs were performed for skull base pathologies other than pituitary neuroendocrine tumor. The mean follow-up period was 34.4 months. Esposito grade 3 leakage was confirmed in 148 (74.0%) cases. NMFCT was used either with (67 [33.5%]) or without (133 [66.5%]) lumbar drainage. There were 10 cases (5.0%) of postoperative CSF leakage that necessitated reoperation. In 4 other cases (2.0%), CSF leakage was suspected but lumbar drainage alone successfully restored the condition. Multivariate logistic regression analyses revealed that posterior skull base location (P < 0.01, odds ratio 11.5, 95% CI 1.99-2.17 × 102) and craniopharyngioma pathology (P = 0.03, odds ratio 9.4, 95% CI 1.25-1.92 × 102) were significantly associated with postoperative CSF leakage. No delayed leakage occurred during the observation period except for 2 patients who underwent multiple radiotherapies. CONCLUSIONS: NMFCT is a reasonable alternative with long-term durability, though vascularized flap may be a better choice for cases in which vascularity of the surrounding tissues is significantly impaired due to interventions including multiple radiotherapies.

A modified microscopic-endoscopic bilateral transseptal approach for pituitary adenoma.

BACKGROUND: Endoscopic transnasal surgery for tumors in the sellar region has become popular across the globe. However, postoperative nasal outcomes related to endoscopic transnasal surgery have yet to be described in detail. METHOD: Here, we introduce a modified microscopic-endoscopic bilateral transseptal approach for the surgical treatment of tumors in the sellar region. This technique yielded satisfactory postoperative nasal outcomes without any loss of the visual advantages associated with endoscopy. CONCLUSION: The modified microscopic-endoscopic bilateral transseptal approach described herein is a minimally invasive endoscopic transnasal approach and results in satisfactory postoperative outcomes in terms of nasal structure and olfactory function.

Endoscopic Transnasal Resection of Trigeminal Schwannoma.

Trigeminal schwannoma is a rare skull base tumor that can be managed in a variety of treatments including image observation, surgery, stereotactic radiosurgery, such as gamma knife radiosurgery (GKS), and combination of these. Endoscopic transnasal resection is very effective when the tumor is not invading far laterally, or the risk of cerebrospinal fluid (CSF) leak is estimated to be low. A 74-year-old man with a history of prostate cancer and diabetes presented with left oculomotor nerve palsy over a month. Magnetic resonance images (MRI) demonstrated a 25-mm mass in the left cavernous sinus protruding to the left orbit via the superior orbital fissure ( Fig. 1 ). The patient underwent endoscopic transnasal surgery to decompress the mass. The surgery was uneventful, and postoperative MRI demonstrated satisfactory subtotal resection of the mass ( Fig. 2 ). The final pathology returned as schwannoma. At 1-year follow-up, the tumor slowly enlarged, and the patient underwent GKS with a marginal dose of 14 Gy. At the last follow-up, 4 months after GKS, the tumor was stable. Unfortunately the patient deceased from the known prostate cancer. Endoscopic transnasal surgery was especially useful in this case, considering the preoperative known cancer state that management of this benign tumor did not ruin the quality of life of this patient while minimizing hospitalization, as achieving satisfactory tumor control with aid from postoperative GKS, minimizing complications. The link to the video can be found at: https://youtu.be/Q0Ugc2VFV4w .

Combined endoscopic endonasal transtubercular and transclival approaches for large neurenteric cyst in posterior cranial fossa: A case report and literature review.

BACKGROUND: Intracranial neurenteric cysts (NCs) are extremely rare tumors that more commonly involve the posterior fossa than any other cranial part. While transcranial skull base surgery has been the mainstay of treatment, the utility of endoscopic transnasal surgery (ETS) remains to be established. CASE DESCRIPTION: We report a case of a large posterior fossa NC extensively involving the suprasellar region, cerebellopontine angle, and prepontine cistern, which we successfully resected with ETS through a combination of transtubercular and transclival routes. Before surgery, the patient presented with abducens nerve and pseudobulbar palsies, which resolved within 2 weeks postoperatively. The patient remained free from recurrence for 3 years postoperatively. CONCLUSION: Extended ETS may offer a minimally invasive option for the posterior fossa NC, extensively occupying the ventral space of the brainstem.

A single-center experience with giant cell tumors of sphenoid bone and clivus.

OBJECTIVE: To present pathologic, clinical, and treatment findings for giant cell tumors (GCTs) of sphenoid bone and clivus. METHODS: We describe the optimal treatment algorithm in patients with a histopathologic diagnosis of bone GCT by presenting the effects of denosumab treatment in both pediatric and adult patients with GCT undergoing endoscopic transnasal surgery. Clinicopathologic correlation is crucial for the differential diagnosis of GCT and the choice of treatment modality. CONCLUSION: GCT of bone is a local aggressive tumor that accounts for about 3%-7% of all bone tumors. GCTs located in the cranium are extremely uncommon neoplasms. There are no defined guidelines for the treatment of GCTs in skull base. Following surgical resection of the tumor, the addition of denosumab treatments to radiotherapy has a significant role in preventing the recurrence of GCT and in promoting regression of residual tumor size.

Endoscopic Transnasal Odontoidectomy.

Background Odontoidectomy may represent the treatment of choice for symptomatic ventral craniovertebral junction stenosis in selected cases. An endoscopic transnasal approach has been proposed as an alternative to a classic transoral approach. Method We report a case of a patient with a craniovertebral junction stenosis due to the ossification of the posterior ligament. The clinical and radiological records of the patient and a step-by-step description of the surgical technique are presented ( Fig. 1 ). Conclusion Endoscopic transnasal odontoidectomy provides a direct access to the dens and adjacent structures of the anterior upper cervical spine with a less invasive burden on the oropharingeal structures. The link to the video can be found at: https://youtu.be/Ofrk1sFTV9I .

Pituitary stone resulting in visual dysfunction and spontaneous rhinorrhea in nonfunctioning pituitary adenoma: illustrative case.

BACKGROUND: Calcifications in pituitary adenomas are rare, being found in only 5.4%-25% of reported cases. These are divided into eggshell-like calcifications around the tumor and nodular calcifications at the center of the tumor, the latter of which are called "pituitary stones" (PSs). OBSERVATIONS: The authors report the case of a 60-year-old male with a nonfunctional pituitary adenoma with PSs and asymptomatic ventricular dilatation who presented with spontaneous cerebrospinal fluid (CSF) rhinorrhea and rapid visual aggravation without an increase in tumor size over the course of 4 years. After endoscopic transnasal surgery, his visual acuity immediately improved temporarily. It was believed that the increased intracranial pressure due to secondary hydrocephalus resulted in visual aggravation; thus, a ventriculoperitoneal (VP) shunt was created. After creation of the VP shunt, the patient's visual acuity improved gradually and completely. Histological findings showed that adenoma cells were observed among the lamellar bone trabeculae. To the best of the authors knowledge, this is the first report of osteoid metaplasia-type PSs in nonfunctioning pituitary adenoma. LESSONS: PSs formed near the sellar floor and caused spontaneous CSF rhinorrhea due to direct mechanical stress on the dura mater and optic nerves, which may have caused meningitis and secondary hydrocephalus that resulted in visual impairment independent of tumor size.

Diagnostic Value of Non-Contrast CT in Cerebrospinal Fluid Leakage After Endoscopic Transnasal Surgery for Sellar and Suprasellar Tumors.

We aimed to study the relationship between pneumocephalus on non-contrast CT (NCCT) and post-operative cerebrospinal fluid leakage (p-CFL) after endoscopic transsphenoidal sellar and suprasellar tumor surgeries. Data from patients who underwent endoscopic treatment for sellar or suprasellar tumors from January 2018 to March 2020 were consecutively collected and reviewed. The NCCT pneumocephalus (NP) was measured the first day after operation and the first day after the expansive sponge was extracted. p-CFL was determined according to post-operative clinical symptoms, high resolution CT and glucose test, and expert consensus. Of the 253 patients enrolled in this study, 32 (12.6%) had p-CFL. Compared with patients without p-CFL, patients with p-CFL had a higher occurrence of intra-operative CFL, a longer operation time, a higher rate of pneumocephalus on first-day NCCT after operation (i.e., first-day NP), and a higher rate of NP volume change between two NCCT measurements (referred to as the NP change) (all p < 0.05). In multivariate regression analysis, first-day NP was independently associated with p-CFL occurrence [odds ratio (OR)=6.395, 95% confidence interval (CI)=2.236-18.290, p=0.001). After adding the NP change into the regression model, first-day NP was no longer independently associated with p-CFL, and NP change (OR = 19.457, 95% CI = 6.095-62.107, p<0.001) was independently associated with p-CFL. The receiver operating characteristic curve comparison analysis showed that NP change had a significantly better predicting value than first-day NP (area under the curve: 0.988 vs. 0.642, Z=6.451, p=0.001). NP is an effective imaging marker for predicting p-CFL after endoscopic sellar and suprasellar tumors operation, and the NP change has a better predicting value.

Transventricular Preforniceal Approach Combined with Endoscopic Transnasal Surgery for a Giant Pituitary Adenoma: A Case Report and Literature Review.

Giant pituitary adenomas carry significant surgical risks when treated with transsphenoidal approaches or the transcranial approach alone. Combined transsphenoidal and transcranial approaches have been reported; however, removing adenomas extending into the third ventricle may still be challenging. We report a case of giant pituitary adenoma expanding into the third ventricle, which was removed using a combined transventricular preforniceal approach and an endoscopic endonasal transsphenoidal surgery (ETSS). A 41-year-old man with headache, nausea, and a 1-week history of a visual field defect was transferred to our hospital. He had a disturbed left visual acuity, right homonymous hemianopia, and choked disc in both eyes. Preoperative magnetic resonance imaging revealed a giant pituitary adenoma with a maximum diameter of 55 mm extending from the intrasellar to the suprasellar region, thus occupying the entire third ventricle and causing hydrocephalus. The space between the anterior commissure and the fornix was expanded. The foramen of Monro was shifted backward due to compression by the tumor. He underwent maximum surgical resection using a combined transventricular preforniceal approach and ETSS. Considering technical demands and reliability, the intra- to suprasellar parts were removed by ETSS while the intraventricular part was removed through the preforniceal approach. The residual tumor in the right cavernous sinus and behind the anterior communicating artery was treated with stereotactic radiotherapy. One year after the operation, the patient leads an independent life. The combined technique of the preforniceal approach and ETSS provided a direct view of the entire third ventricle and hemostasis in the present case.

[Anatomy of anterior craniovertebral junction in endoscopic transnasal approach]. / Anatomiya perednikh otdelov kraniovertebral'nogo sochleneniya pri endoskopicheskom transnazal'nom dostupe.

Modern achievements in endoscopic technologies ensure extending the indications for endoscopic transnasal approach in skull base surgery. Knowledge on topographic anatomy of craniovertebral junction is a prerequisite for surgical interventions in this area. Transnasal endoscopic surgery of craniovertebral junction is a relatively new field. Therefore, this manuscript and similar anatomical studies are extremely important for neurosurgeons.

Mycotic Aneurysm of the Extracranial Internal Carotid Artery Following Otitis Media.

Mycotic aneurysm of the internal carotid artery following otomastoiditis can manifest as a nasopharyngeal mass. This is a very rarely described condition. The case of a patient diagnosed with a retropharyngeal mycotic aneurysm of the left internal carotid artery following otitis media, treated with exclusion of the mycotic aneurysm from the circulation and transnasal drainage. Infection of the mastoid can spread to the parapharyngeal space of the nasopharynx and damage the carotid artery wall, resulting in an infected aneurysm. There are no guidelines on how to surgically approach those aneurysms. An endoscopic transnasal approach can be beneficial.

Nasal Functions in Three-Dimensional Endoscopic Skull Base Surgery.

OBJECTIVES: Endoscopic transnasal transsphenoidal surgery has become the standard procedure for the majority of skull base diseases, including sellar, parasellar, and clival pathologies. The aim of this study was the objective evaluation of nasal airflow resistances and olfactory function in 3-dimensional (3D) endoscopic transnasal transsphenoidal surgery. METHODS: One hundred patients who underwent 3D transnasal endoscopic surgery for sellar, parasellar, and clival diseases were enrolled. Active anterior rhinomanometry and Sniffin' Sticks tests were performed before endoscopic surgery and at 3 and 6 months postoperatively. RESULTS: No significant difference about nasal airflow resistance and olfactory function was observed between preoperative and postoperative subjective and objective scores. In the group of patients with sellar and parasellar diseases, a worst nasal respiratory function was seen when crusting was present, and a worst olfactory function was observed in patients with synechiae. Nasal functions returned to previous levels when crusting or synechiae solved. No statistically significant correlation was observed between the evaluated nasal functions and the reconstruction with flaps. CONCLUSIONS: The 3D endoscopic transnasal transsphenoidal surgery represents a more and more important tool in skull base surgery. It does not determine nasal respiratory and olfactory alterations after the treatment, without an increase in nasal complaints that could worsen quality of life.