Multi-center study on sellar reconstruction after endoscopic transsphenoidal pituitary surgery.

INTRODUCTION: Surgical techniques for sellar reconstruction include no reconstruction, use of synthetic materials, autologous grafts, and/or vascularized flaps. The aim of this study was to conduct a multi-center study comparing the efficacy and postoperative morbidity associated with different sellar reconstruction techniques. METHODS: A retrospective chart review of patients who underwent endoscopic transsphenoidal surgery for pituitary tumors from five participating sites between January 2021 and March 2023 was performed. The variables included demographics, tumor characteristics, reconstruction technique, postoperative cerebrospinal fluid leak (CSF) leak, and 22-item Sino-Nasal Outcome Test (SNOT-22) scores. Comparisons of postoperative complications, SNOT-22 scores, and duration of surgery by type of onlay reconstruction were evaluated using Fisher's exact test, analysis of variance, and Kruskal‒Wallis test. RESULTS: Five hundred and one patients were identified. The median tumor size was 2.1 cm, and 64% were non-functioning. Intraoperative CSF leak was identified in 38% of patients. A total of 89% of patients underwent onlay reconstruction: 49% were reconstructed with mucosal grafts, 35% with nasoseptal flaps, and 5% with other onlay techniques. Nasoseptal flaps were utilized more frequently in the setting of giant pituitary adenomas (>3 cm), medial cavernous sinus wall resection, and high-flow intraoperative CSF leaks. Cases who utilized mucosal grafts had an overall shorter operating time (median: 183 min vs. 240 min; p < 0.001). Five postoperative CSF leaks were identified, and therefore, statistical analysis could not be performed for this complication. CONCLUSION: The effectiveness and morbidity of different sellar reconstruction techniques are comparable. Vascularized flaps were utilized more frequently in the setting of larger tumors and high-flow intraoperative CSF leaks.

Transpalpebral mini-orbitozygomatic approach for nonvascular skull base lesions: a single neurosurgeon's experience.

OBJECTIVE: The authors aim to describe the advantages, utility, and disadvantages of the transpalpebral mini-orbitozygomatic (MOZ) approach for tumors of the lateral and superior orbit, orbital apex, anterior clinoid, anterior cranial fossa, middle cranial fossa, and parasellar region. METHODS: The surgical approach from skin incision to closure is described while highlighting key technical and anatomical considerations, and cadaveric dissection demonstrates the surgical steps and focuses on important anatomy. Intraoperative images were included to supplement the cadaveric dissection. A retrospective review of adults who had undergone the MOZ approach for nonvascular pathology performed by a single neurosurgeon from 2017 to 2023 was included in this institutional review board-approved study. Descriptive statistics was used to summarize the data. Four representative cases were included to demonstrate the utility of the MOZ approach. RESULTS: The study included 65 patients (46 female, 19 male), average age 54.84 years, who had undergone transpalpebral MOZ surgery. Presenting symptoms included visual changes (53.8% of cases), vision loss (23.1%), diplopia (21.8%), and proptosis (13.8%). The optic nerve and optic chiasm were involved in 32.3% and 10.8% of cases, respectively. The most common pathology was meningioma (81.5% of cases), and gross-total resection was achieved in 50% of all cases. Major complications included an infection and a carotid injury. Improvement of preoperative symptoms was reported in 92.2% of cases. Visual acuity improved in 12 patients. The mean follow-up was 8.57 ± 8.45 months. CONCLUSIONS: The MOZ approach is safe and durable. The transpalpebral incision provides better cosmesis and functional outcomes than those of standard anterolateral approaches to the skull base. Careful consideration of the limits of the approach is paramount to appropriate application on a case-by-case basis. Further quantitative anatomical studies can help to define and compare the utility of the approach to open cranio-orbital and endoscopic transorbital approaches.

Recurrence morbidity of olfactory neuroblastoma.

BACKGROUND: With modern treatment paradigms, olfactory neuroblastoma (ONB) has favorable overall survival (OS); however, the incidence of recurrence remains high. The primary aims of this study were to delineate the prognosis of recurrence of ONB and explore how recurrence subsites are associated with OS, disease-specific survival (DSS), and further recurrence. METHODS: A retrospective chart review of ONB cases from nine academic centers between 2005 and 2021 was completed. Tumor characteristics, recurrence subsites, timelines to recurrence, additional recurrences, and survival estimates were determined using descriptive and time-to-event analyses. RESULTS: A final cohort of 233 patients was identified, with 70 (30.0%) patients recurring within 50.4 (standard deviation ±40.9) months of diagnosis on average, consisting of local (50%), neck (36%), intracranial (9%), and distant (6%) recurrence. Compared with subjects without recurrence, patients with recurrence had significantly different primary American Joint Committee on Cancer T stage (p < 0.001), overall stage (p < 0.001), and modified Kadish scores (p < 0.001). Histopathology identified that dural involvement and positive margins were significantly greater in recurrent cases. First recurrence was significantly associated with worse 5-year DSS (hazard ratio = 5.62; p = 0.003), and subjects with neck or local recurrence had a significantly better DSS compared to intracranial or distant recurrence. CONCLUSIONS: Recurrent cases of ONB have significantly different stages and preoperative imaging factors. Patients with local or neck recurrence, however, have better DSS than those with intracranial or distant recurrence, independent of initial tumor stage or Hyams grade. Identifying specific factors that confer an increased risk of recurrence and DSS is important for patient counseling in addition to surveillance planning.

Predictors and Complications of Cerebrospinal Fluid Leak after Endoscopic Endonasal Surgery: A Single Institution Retrospective Review.

Background The endoscopic endonasal approach (EEA) is a commonly used technique for resection of sellar, suprasellar, and anterior fossa masses. One of the most troublesome complications of this technique is cerebrospinal fluid (CSF) leak. In this study, we evaluate the risk factors and consequences of CSF leak on surgical outcomes. Methods The current study is a retrospective single-institution cohort study evaluating patients who underwent EEA for sellar and/or suprasellar masses from July 2017 to March of 2020. Risk factors for intraoperative and postoperative CSF leak were evaluated, including sellar defect size, tumor volume and pathology, age, body mass index, prior endoscopic endonasal surgery, lumbar drain placement, nasoseptal and mucosal graft use, year of surgery, and cavernous sinus invasion. Postoperative infection, perioperative antibiotic use, and length of stay were also evaluated. Results Our study included 175 patients. Sellar defect size ( p = 0.015) and intraoperative CSF leak ( p < 0.001) were significantly associated with an increased risk of postoperative CSF leak. Patients with nasoseptal flaps were more likely to have a postoperative CSF leak than those with free mucosal grafts ( p = 0.025). Intraoperative CSF leak, Cushing's disease, and lumbar drain placement were associated with an increased length of stay. Conclusion Sellar defect size, intraoperative CSF leak, and nasoseptal flap use were associated with an increased risk of postoperative CSF leak. Intraoperative CSF leak, Cushing's disease, and lumbar drain placement are all associated with an increased length of stay.

Uninterrupted use of continuous positive airway pressure after endoscopic endonasal approach for pituitary tumors.

KEY POINTS: Resumption of continuous positive airway pressure (CPAP) in the immediate postoperative period after endoscopic endonasal approaches (EEA) for pituitary adenomas can be safe.

Consecutive resections of double pituitary adenoma for resolution of Cushing disease: illustrative case.

BACKGROUND: Double pituitary adenomas are rare presentations of two distinct adenohypophyseal lesions seen in <1% of surgical cases. Increased rates of recurrence or persistence are reported in the resection of Cushing microadenomas and are attributed to the small tumor size and localization difficulties. The authors report a case of surgical treatment failure of Cushing disease because of the presence of a secondary pituitary adenoma. OBSERVATIONS: A 32-year-old woman with a history of prolactin excess and pituitary lesion presented with oligomenorrhea, weight gain, facial fullness, and hirsutism. Urinary and nighttime salivary cortisol elevation were elevated. Magnetic resonance imaging confirmed a 4-mm3 pituitary lesion. Inferior petrosal sinus sampling was diagnostic for Cushing disease. Primary endoscopic endonasal transsphenoidal resection was performed to remove what was determined to be a lactotroph-secreting tumor on immunohistochemistry with persistent hypercortisolism. Repeat resection yielded a corticotroph-secreting tumor and postoperative hypoadrenalism followed by long-term normalization of the hypothalamic-pituitary-adrenal axis. LESSONS: This case demonstrates the importance of multidisciplinary management and postoperative hormonal follow-up in patients with Cushing disease. Improved strategies for localization of the active tumor in double pituitary adenomas are essential for primary surgical success and resolution of endocrinopathies.

Multicenter Survival Analysis and Application of an Olfactory Neuroblastoma Staging Modification Incorporating Hyams Grade.

Importance: Current olfactory neuroblastoma (ONB) staging systems inadequately delineate locally advanced tumors, do not incorporate tumor grade, and poorly estimate survival and recurrence. Objective: The primary aims of this study were to (1) examine the clinical covariates associated with survival and recurrence of ONB in a modern-era multicenter cohort and (2) incorporate Hyams tumor grade into existing staging systems to assess its ability to estimate survival and recurrence. Design, Setting, and Participants: This retrospective, multicenter, case-control study included patients with ONB who underwent treatment between January 1, 2005, and December 31, 2021, at 9 North American academic medical centers. Intervention: Standard-of-care ONB treatment. Main Outcome and Measures: The main outcomes were overall survival (OS), disease-free survival (DFS), and disease-specific survival (DSS) as C statistics for model prediction. Results: A total of 256 patients with ONB (mean [SD] age, 52.0 [15.6] years; 115 female [44.9%]; 141 male [55.1%]) were included. The 5-year rate for OS was 83.5% (95% CI, 78.3%-89.1%); for DFS, 70.8% (95% CI, 64.3%-78.0%); and for DSS, 94.1% (95% CI, 90.5%-97.8%). On multivariable analysis, age, American Joint Committee on Cancer (AJCC) stage, involvement of bilateral maxillary sinuses, and positive margins were associated with OS. Only AJCC stage was associated with DFS. Only N stage was associated with DSS. When assessing the ability of staging systems to estimate OS, the best-performing model was the novel modification of the Dulguerov system (C statistic, 0.66; 95% CI, 0.59-0.76), and the Kadish system performed most poorly (C statistic, 0.57; 95% CI, 0.50-0.63). Regarding estimation of DFS, the modified Kadish system performed most poorly (C statistic, 0.55; 95% CI, 0.51-0.66), while the novel modification of the AJCC system performed the best (C statistic, 0.70; 95% CI, 0.66-0.80). Regarding estimation of DSS, the modified Kadish system was the best-performing model (C statistic, 0.79; 95% CI, 0.70-0.94), and the unmodified Kadish performed the worst (C statistic, 0.56; 95% CI, 0.51-0.68). The ability for novel ONB staging systems to estimate disease progression across stages was also assessed. In the novel Kadish staging system, patients with stage VI disease were approximately 7 times as likely to experience disease progression as patients with stage I disease (hazard ratio [HR], 6.84; 95% CI, 1.60-29.20). Results were similar for the novel modified Kadish system (HR, 8.99; 95% CI, 1.62-49.85) and the novel Dulguerov system (HR, 6.86; 95% CI, 2.74-17.18). Conclusions and Relevance: The study findings indicate that 5-year OS for ONB is favorable and that incorporation of Hyams grade into traditional ONB staging systems is associated with improved estimation of disease progression.

Clival intraosseous myxoma treated with an endoscopic endonasal approach: illustrative case.

BACKGROUND: Solitary lesions of the clivus, especially nonchordomatous ones, are exceptionally rare pathologies representing only 0.1%-0.2% of intracranial tumors that may present diagnostic and therapeutic challenges. Intraosseous myxomas are benign tumors arising from mesenchymal cells with an unknown pathophysiology. OBSERVATIONS: In this article, a 15-year-old male with clival intraosseous myxoma who was treated via an endoscopic endonasal surgical approach is presented. The patient became symptomatic after a baseball struck his head, leading to throbbing headaches and visual difficulties for tracking objects. Examination noted diplopia, cranial nerve VI palsy, and ptosis. After imaging revealed a tumor extending to the left cavernous sinus with bulging of the dorsal clivus against the basilar artery/ventral pons, resection was performed. Postoperatively, the patient noted ocular motility and alignment improvement without further complications. LESSONS: Clival masses present with symptoms from compromised neurovascular structures including visual disturbances and trigeminal sensory deficits. Given the rarity of these entities, patients may postpone further treatment until workup; this patient was misdiagnosed for possible concussion until several weeks passed. A PubMed database review of cranial myxoma cases was conducted to identify solitary clival intraosseous myxoma cases. This case is one of the few in which this pathology was treated through an endoscopic endonasal operative approach without complications, demonstrating its safety and effectiveness.

Radiographic predictors of occult intracranial involvement in olfactory neuroblastoma patients.

BACKGROUND: Traditional management of olfactory neuroblastoma (ONB) includes margin-negative resection with removal of cribriform plate, dura, and olfactory bulb, regardless of intracranial disease. This approach may be overtreating certain patients. Our investigation examines risk factors associated with occult intracranial disease to optimize therapeutic outcomes. METHODS: This retrospective, multi-institutional cohort study examined clinical covariates associated with occult intracranial involvement. Patient demographics, staging, Hyam's grade, and pathologic involvement of dura, olfactory bulb/tract, and brain were collected. Diagnostic imaging was reviewed. Positive and negative predictive value (NPV) were estimated along with effect size estimates. Cox hazard regression examined associations with overall survival (OS) and disease-free survival (DFS). RESULTS: A total of 224 subjects with new diagnoses of ONB (2005-2021) were identified. Skull base bone involvement on computed tomography (CT) had the highest NPV for pathologic dura (88.0%), olfactory bulb (88%), and brain involvement (97.3%). Hyam's grade category was significantly associated with dural involvement (φC  = 0.26; 95% confidence interval [CI]: 0.16, 0.42). Subjects without radiologic skull base involvement (n = 66) had pathologic positivity of 12.1%. Within this subgroup, Hyam's grade was clinically significant for dural positivity (φ = 0.34; 95% CI: -0.12, 0.71) with 28.6% involvement in high grade tumors. Neither clinical nor pathologic positivity of intracranial structures were associated with significantly different OS or DFS. CONCLUSIONS: Both CT and magnetic resonance imaging (MRI) had reasonably good NPV for involvement of dura and olfactory bulb. Higher Hyam's grade was associated with dural involvement. Patients with low-grade tumors not involving the skull base may be suitable for avoiding skull base resection; however, further investigation is warranted.

Endoscopic endonasal resection of a primary intraosseous clival myxoma: illustrative case.

BACKGROUND: An intraosseous myxoma is a rare, benign mesenchymal tumor that penetrates the bone. The occurrence of an intraosseous myxoma in the clivus is a unique presentation of the disease. OBSERVATIONS: The authors discuss the case of a 15-year-old male with a new diagnosis of a primary clival intraosseous myxoma presenting with cranial nerve VI palsy. This is the third documented case of this pathology occurring in the clivus. This patient was successfully treated with endoscopic endonasal resection of the tumor. LESSONS: Primary clival intraosseous myxomas are extremely rare, but nonetheless it is important to add it to the differential diagnosis of clival masses. This mass has a high risk of recurrence, and prior literature suggests gross total resection may improve chances of progression-free survival. However, further larger studies are needed to provide guidelines regarding proper management of this pathology.

A Rare Case of Neck Sarcomatoid Squamous Cell Carcinoma With Brain Metastases.

Squamous cell carcinoma (SCC) is the second most prominent form of skin malignancy. It occurs most frequently in older males with fair skin complexion that have extensive sun exposure most commonly in their childhood. The metastatic presentation of SCC is rare and is most common in the lung. In this paper, we present the unique case of a 73-year-old patient with sarcomatoid squamous cell carcinoma in their posterior neck that metastasized to the brain.

Delayed Postoperative Tension Pneumocephalus Treated With a Subdural Evacuating Port System: A Case Report and Review of the Literature.

Tension pneumocephalus (TP) is a rare neurosurgical emergency due to the rise of intracranial pressure from air in the cranial cavity. Tension pneumocephalus' clinical presentation ranges from headache, visual alterations, altered mental status, and death. Given its nonspecific clinical presentation, tension pneumocephalus is usually diagnosed via computed tomography (CT) imaging. Open burr hole craniotomy is the preferred treatment method for tension pneumocephalus. Subdural evacuating port system (SEPS) drains have, however, seen increased utilization in neurosurgery due to decreased possibilities for infections, reduced seizure probability, and better outcomes post-surgery, especially for elderly patients. In this article, we present the case of a 67-year-old female with postoperative tension pneumocephalus after the evacuation of an acute subdural hematoma. The patient became symptomatic from tension pneumocephalus, which was evacuated using a subdural evacuating port system drain. Post-drain placement, the patient had a radiographic and clinical resolution of her tension pneumocephalus. Thesubdural evacuating port system is a useful adjunctive tool for treating tension pneumocephalus.Given the favorable characteristic profile of subdural evacuating port system drains compared to open surgical modalities, further inquiry should be pursued to analyze the feasibility of establishing subdural evacuating port systems as a less invasive treatment alternative.

Large Middle Cranial Fossa Schwannoma: A Rare Presentation of Vestibular Schwannoma.

Schwannomas are benign tumors composed of neoplastic Schwann cells and rarely occur in the central nervous system. Schwannomas account for approximately 8% of intracranial tumors and most commonly originate from cranial nerve VIII at the cerebellopontine angle in the posterior fossa. Herein, we report two cases of vestibular schwannomas extending in the middle fossa. The first case shows a 51-year-old male who presented with a history of mild headaches for one year associated with acute nausea, vomiting, and word-finding difficulties. Imaging revealed a large multicystic contrast-enhancing lesion in the left middle cranial fossa. The middle fossa lesion was resected with pathology indicating a schwannoma. The second case shows a 63-year-old woman who presented with seizures, right-sided hearing loss, and right-sided facial weakness. On MRI, she is found to have a large right middle fossa lesion originating from the right internal auditory canal and consistent with vestibular schwannoma with a 9 mm leftward midline shift. The histopathologic examination of the excised tumor indicated a schwannoma. Schwannomas most commonly occur in the posterior fossa when they present intracranially. However, in rare occurrences, they may present as middle fossa masses with significant intracranial compression.

Intraosseous Meningioma Along the Left Petrous Bone: A Rare Cause of Trigeminal Neuralgia.

Trigeminal neuralgia (TN) presents with symptoms of intense recurrent shock-like brief pain localized to specific areas of the face innervated by the fifth cranial nerve. The pathology of trigeminal neuralgia is attributed to the fifth cranial nerve compression or demyelination. Most cases of this diagnosis are not due to bony structures, making this case an uncommon presentation of trigeminal neuralgia. Herein, we present a case of trigeminal neuralgia due to an intraosseous meningioma that formed along the left petrous bone, resulting in trigeminal nerve compression. On head computed tomography (CT), osteomatous growths along the left petrous bone were noticed compressing the trigeminal nerve. After trigeminal nerve decompression and drilling out the protruding part of the petrous bone through middle cranial fossa craniotomy, the patient's symptoms were completely improved postoperatively and at the two-month follow-up. To our knowledge, there are only four reported cases of trigeminal neuralgia caused by petrous bone compression in the literature. We emphasize the significance of considering petrous bone lesions as a cause of trigeminal neuralgia.

O-GlcNAcylation Is Essential for Rapid Pomc Expression and Cell Proliferation in Corticotropic Tumor Cells.

Pituitary adenomas have a staggering 16.7% lifetime prevalence and can be devastating in many patients because of profound endocrine and neurologic dysfunction. To date, no clear genomic or epigenomic markers correlate with their onset or severity. Herein, we investigate the impact of the O-GlcNAc posttranslational modification in their etiology. Found in more than 7000 human proteins to date, O-GlcNAcylation dynamically regulates proteins in critical signaling pathways, and its deregulation is involved in cancer progression and endocrine diseases such as diabetes. In this study, we demonstrated that O-GlcNAc enzymes were upregulated, particularly in aggressive adrenocorticotropin (ACTH)-secreting tumors, suggesting a role for O-GlcNAcylation in pituitary adenoma etiology. In addition to the demonstration that O-GlcNAcylation was essential for their proliferation, we showed that the endocrine function of pituitary adenoma is also dependent on O-GlcNAcylation. In corticotropic tumors, hypersecretion of the proopiomelanocortin (POMC)-derived hormone ACTH leads to Cushing disease, materialized by severe endocrine disruption and increased mortality. We demonstrated that Pomc messenger RNA is stabilized in an O-GlcNAc-dependent manner in response to corticotrophin-releasing hormone (CRH). By affecting Pomc mRNA splicing and stability, O-GlcNAcylation contributes to this new mechanism of fast hormonal response in corticotropes. Thus, this study stresses the essential role of O-GlcNAcylation in ACTH-secreting adenomas' pathophysiology, including cellular proliferation and hypersecretion.

COVID-19 Testing in the Era of Modern Neurosurgery: Mitigating Risk in Our Vulnerable Patient Populations.

BACKGROUND: The respiratory illness identified as coronavirus disease 2019 (COVID-19) has resulted in a pandemic illness that has changed the face of healthcare. As the COVID-19 pandemic continues, patients have continued to require neurosurgical interventions, and the endoscopic endonasal approach for surgery has continued to be a mainstay treatment of pituitary tumors and anterior skull base lesions. METHODS: We sought to highlight the current lack of recommendations regarding testing protocols for neurosurgical patients. RESULTS: We implemented a novel testing protocol for our patient populations at increased risk and have proposed a model that can be used at other institutions to mitigate the risk of complications associated with some forms of COVID-19 testing. CONCLUSION: Patients with anterior skull base defects may be at risk with current COVID-19 testing protocols, and may benefit from alternative specimen collection strategies.

Comparison of Follow-Up Length-Matched Single-Center Myelomeningocele Postnatal Closure Cohort to the Management of Myelomeningocele Study (MOMS) Trial Results.

OBJECTIVE: We sought to compare our large single-institution cohort of postnatal myelomeningocele closure to the 2 arms of the Management of Myelomeningocele Study (MOMS) trial at the designated trial time points, as well as assess outcomes at long-term follow-up among our postnatal cohort. METHODS: A single-institutional retrospective review of myelomeningocele cases presenting from 1995 to 2015 at Children's Hospital of Pittsburgh was performed. We compared outcomes at 12 and 30 months to both arms of the MOMS trial and compared our cohort's outcomes at those designated time points to our long-term outcomes. Univariate statistical analysis was performed as appropriate. RESULTS: One-hundred sixty-three patients were included in this study. All patients had at least 2-year follow-up, with a mean follow-up of 10 years (range 2-20 years). There was no difference in the overall distribution of anatomic level of defect. Compared to our cohort, the prenatal cohort had a higher rate of tethering at 12 months of age, 8 versus 1.8%. Conversely, the Chiari II decompression rate was higher in our cohort (10.4 vs. 1.0%). At 30 months, the prenatal cohort had a higher rate of independent ambulation, but our cohort demonstrated the highest rate of ambulation with or without assistive devices among the 3 groups. When comparing our cohort at these early time points to our long-term follow-up data, our cohort's ambulatory function decreased from 84 to 66%, and the rate of detethering surgery increased almost 10-fold. CONCLUSIONS: This study demonstrated that overall ambulation and anatomic-functional level were significantly better among our large postnatal cohort, as well as having significantly fewer complications to both fetus and mother, when compared to the postnatal cohort of the MOMS trial. Our finding that ambulatory ability declined significantly with age in this patient population is worrisome for the long-term outcomes of the MOMS cohorts, especially given the high rates of cord tethering at early ages within the prenatal cohort. These findings suggest that the perceived benefits of prenatal closure over postnatal closure may not be as substantial as presented in the original trial, with the durability of results still remaining a concern.

Intracranial Capillary Hemangiomas: A Peripartum Presentation and Review of the Literature.

BACKGROUND: Intracranial capillary hemangiomas (ICHs) are rare vascular tumors composed of a bed of many narrow thin-walled vessels. Within the confines of the skull, these tumors can lead to serious neurologic deficits including cranial nerve dysfunction, mood/personality disturbances, and signs of intracranial mass effect. METHODS: We report the case of a 23-year-old, 5-week postpartum woman with a history of progressive painful ophthalmalgia of the right eye presenting with rapid onset of ptosis, diplopia, and right-sided facial pain and hypesthesia. Imaging demonstrated a small extraaxial mass within the right cavernous sinus. She underwent 2 operations via an endoscopic endonasal approach for biopsy followed by complete resection. Histology showed a highly mitotic capillary hemangioma, which was negative for both estrogen and progesterone receptors. RESULTS: We review cases of ICH reported in the literature and provide an updated summary of the presentation, diagnosis, and treatment of ICH. We then present a brief analysis of the reported cases with respect to age and sex. CONCLUSIONS: We conclude that, in experienced hands, the endoscopic endonasal approach can be used to access the cavernous sinus for complete resection of ICHs of the cavernous sinus. We also suggest that further attention be paid to such cases in pregnant and peripartum women as these tumors may progress more quickly in this subpopulation.

Long-term outcomes in the treatment of pediatric skull base chordomas in the endoscopic endonasal era.

OBJECTIVE: Pediatric skull base chordoma is a rare entity that is traditionally considered to display aggressive behavior with an increased risk of recurrence. There is an absence of literature examining outcomes in the pediatric population in general and using the endoscopic endonasal approach (EEA). METHODS: The authors retrospectively reviewed all patients with skull base chordomas presenting by the age of 18 years to the Children's Hospital of Pittsburgh or the University of Pittsburgh Medical Center from 2004 to 2019. Clinical outcomes, the number and location of recurrences, and progression-free survival time were determined. RESULTS: Twenty patients met the study criteria. The most common presenting complaints were diplopia (n = 7), headache (n = 6), and swallowing difficulty (n = 4). Three cases were incidentally discovered. Twelve patients underwent single-stage EEA alone, 2 patients had two-stage EEA, and 6 patients had combined EEA with open far-lateral or extreme-lateral approaches. Fourteen patients underwent gross-total resection (GTR), and 6 patients had near-total resection. Larger tumors were more likely to require staging or a combined approach (86% vs 7%) and were less likely to receive GTR (33% vs 86%) but had comparable recurrence and mortality rates. Five patients developed CSF leaks requiring reoperation, 2 patients developed a permanent abducens nerve palsy, 1 patient suffered an internal carotid artery injury, 1 patient developed an epidural hematoma, and 1 patient developed a subdural empyema. Four (20%) patients had recurrence during follow-up (mean radiographic follow-up 59 months and mean time to local recurrence 19 months). Two patients with recurrence underwent further resection, and 1 patient elected to stop treatment. Both patients who underwent repeat resection experienced a second recurrence, one of whom elected to stop treatment. Both patients who died had an elevated Ki-67 (p = 0.039), one of whom developed de-differentiated histology. A third patient died of progressive spinal metastases without local recurrence and is one of 2 patients who developed postoperative spinal metastases. Both patients whose tumors became de-differentiated progressed from tumors with an initial Ki-67 of 15 or greater (p = 0.035) and received prior radiotherapy to the bulk tumor (p = 0.03). CONCLUSIONS: The majority of pediatric skull base chordomas, when managed at a specialized center with a goal of GTR, may have a better outcome than traditionally believed. Elevated Ki-67 rates may predict poor outcome and progression to de-differentiation.