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Chordomas of the skull base are rare, slow growing, locally invasive cancers with limited long-term survival analysis reported in the literature. We seek to provide comparative survival analysis of patients on a long-term (20-year) basis using population-level data. The Surveillance, Epidemiology, and End Results (SEER) program was queried for cases of chordoma relegated to the base of the skull, diagnosed between 2000 and 2020. Demographic, disease, and treatment information were analyzed using Cox proportional hazards and log-rank comparisons. 630 patients with chordoma of the skull base were identified. Age ≤ 49 years at diagnosis was associated with increased five-, 10-, and 20-year overall survival (hazard ratio (HR) = 0.39, 0.33, and 0.30, respectively; p < 0.001 for all). Treatment with surgery and adjuvant radiotherapy was associated with increased five-, 10-, and 20-year survival (HR = 0.71, 0.79, and 0.79, respectively; p < 0.001 for all). On univariate analysis, widowed patients had decreased survival (20-year overall survival = 34.8% [15.3%-34.8%] compared to married patients (74.4% [68.1%-80.8%]. Surgery remains the primary treatment associated with increased survival among patients with chordoma of the skull base, with adjuvant radiotherapy serving a complimentary role. Demographic factors such as marital status are also associated with changes in survival., (© 2024. This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply.)

Tumors located in the nasal cavity, paranasal sinuses and the skull base comprise a wide range of histologic subtypes. Among them, neuroendocrine and undifferentiated tumors are rare but noteworthy, because of their distinctive features, aggressive nature, and diagnostic complexities. A literature search was conducted in the PubMed/MEDLINE and the Scopus databases from 2019 until inception. The keywords "neuroendocrine", "undifferentiated", "nose", "sinonasal", "paranasal", "skull base" were used. Thirty-eight articles referring to neuroendocrine and undifferentiated tumors of the nose, paranasal sinuses and the skull base were finally included and analyzed. Neuroendocrine and undifferentiated tumors of the nose, paranasal sinuses and the skull base are infrequent malignancies, most commonly affecting middle-aged men. They usually present with non-specific symptoms, even though ocular or neurologic manifestations may occur. Prognosis is generally poor; however, novel targeted and immunological therapies have shown promising results. Sinonasal Neuroendocrine Carcinomas (SNECs) carry distinct histological and immunohistochemical features. Management consists of surgical resection coupled with systematic therapy. Sinonasal Undifferentiated Carcinomas (SNUCs) lack specific squamous or glandular features. They typically stain positive for pancytokeratin and INI1 antibody. Treatment includes induction chemotherapy, followed by a combination of chemotherapy and radiotherapy. Olfactory neuroblastomas (ONBs) have neuroepithelial or neuroblastic features. They show diffuse positivity for various markers, including synaptophysin, chromogranin, and neuron-specific enolase (NSE). Surgical resection plus radiotherapy is considered the treatment of choice. In conclusion, neuroendocrine and undifferentiated tumors arising from the nose, paranasal sinuses and the skull base represent a unique group of malignancies. A thorough understanding of their clinical features, molecular changes, diagnostic approaches, treatment modalities, and prognostic factors is critical for providing optimal patient care. Still, continued research efforts and multidisciplinary collaboration are warranted, in order to improve outcomes for patients diagnosed with these rare and aggressive tumors., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: The objective of this study was to investigate the role of the foramen of Vesalius (FV) in the pathogenesis of skull base meningioma by analyzing data from various multi-image modalities., Methods: For this single-center retrospective study, 39 consecutive patients with skull base meningioma who underwent tumor resection between January 2020 and March 2023 were enrolled. The anatomical and pathological characteristics of the FV were evaluated using computed tomography and 3-dimensional digital subtraction angiography. The clinical significance of the FV in tumor hemodynamics and treatment, such as preoperative tumor embolization, was investigated using the 3-dimensional digital subtraction angiography/computed tomography fusion images., Results: We identified FV in 52% (17/27) of the finally included patients. In 10 (30%) patients, the FV was found bilaterally with no significant variation in appearance between the healthy and tumor-affected sides (P = 0.786). The mean FV diameter was significantly larger on the tumor-affected side (P = 0.010). No significant anatomical differences, like duplication and partial assimilation with the foramen ovale, were observed between the 2 sides. The FV was involved in venous skull base perfusion around the tumor in 9 cases. In 4 cases where it was the pathway for tumor feeders, preoperative tumor embolization via the FV resulted in disappearance of the tumor stain. No complications associated with endovascular treatment were observed., Conclusions: This study elucidated the anatomical asymmetry of the FV and its role in the hemodynamics of skull base meningioma. Our findings highlight the significance of performing anatomical and pathological evaluations of the FV in determining treatment strategies, including preoperative embolization, for skull base lesions., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Plasmacytoma of the skull base is a rare manifestation of plasma cell neoplasm with only a few cases documented in literature involving young adults. Plasmacytoma can be an isolated solitary lesion or a secondary manifestation of multiple myeloma (MM). In this study, we report the clinical and radiological characteristics, management, and outcomes of patients under the age of 40 who presented with skull base plasmacytoma and associated neurological manifestations. Additionally, we share our experience in treating a rare case of skull base plasmacytoma diagnosed during pregnancy, in which the patient exhibited a favorable response to myeloma treatment initiated after delivery., Case Series: Four patients were identified, comprising one pregnant female and three male patients, with a median age of 36 years (range 33-37 years). The main presenting symptoms were headache, dizziness, and cranial nerve palsy. All patients received underwent systemic myeloma therapy and radiotherapy with three patients also underwent autologous stem cell transplantation (ASCT). Notably, all patients achieved complete remission., Conclusion: Skull base plasmacytoma represents a rare manifestation of plasma cell neoplasms, underscoring the importance of considering it in the differential diagnosis of skull base lesions to ensure early intervention and avoid potential serious complications. Throughout our series, the cornerstone of therapy involved radiotherapy, systemic myeloma therapy, and ASCT, all of which elicited a favorable response in every case., (© 2024 The Author(s). Cancer Reports published by Wiley Periodicals LLC.)

Objective: To summarize and analyze the clinical manifestations, diagnosis and management and prognostic features of solitary fibrous tumor（SFT） in nasal cavity, sinus and skull base. Methods: The clinical data of 12 patients with STF from nasal cavity, sinus and cranial base admitted to the Affiliated Hospital of Qingdao University from April 2014 to January 2022 were retrospectively analyzed, including 4 patients admitted to the department of Otolaryngology head and neck surgery and 8 patients admitted to the department of skull base surgery The clinical characteristics, diagnosis, management and prognosis were analyzed. Results: Twelve patients were included in this research, including 7 males and 5 females. All patients received surgical treatment, and 4 patients also received postoperative adjuvant chemoradiotherapy. After follow-up for 12-60 months, 4 patients with adjuvant radiotherapy and chemotherapy had a good prognosis, and among 8 patients who did not receive radiotherapy and chemotherapy, 6 patients had good prognosis and 2 patients showed relapse. Four patients with a history of recurrence of SFT after surgery were admitted to our hospital for surgical treatment, in which 1 patient had relapse after surgery, and none had metastasis. Nasal cavity and sinus to skull base SFT is rare. The most effective treatment for this disease is surgical resection, and postoperative adjuvant chemoradiation and long-term follow-up can achieve a better prognosis. En bloc resection is the key to treatment success., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Brain metastases (BMs) arising from ovarian cancer remain rare. Spinal cord metastases are even rarer, accounting for just 0.4% of total metastatic spinal cord compressions. In this report, we describe a case of a woman in her 70s who developed sequential brain and spinal cord metastases during her treatment for high-grade serous ovarian cancer, without a germline or somatic BRCA mutation. Following completion of neoadjuvant chemotherapy, interval debulking surgery and adjuvant chemotherapy, relapsed disease was ultimately identified as a single BM, curiously mimicking an acoustic neuroma. Subsequently, spinal cord metastases rapidly developed. Throughout, multidisciplinary team meetings guided decisions on patient management. In this report, we highlight the rarity of such a presentation and discuss the possible role of disease pathophysiology, associated systemic anticancer therapy resistance, and treatment possibilities for both cerebral and spinal metastases., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Background and Purpose: Tumor embolization through the meningohypophyseal trunk and inferolateral trunk is known to be effective in skull base tumors; however, microcatheter cannulation into these arteries is difficult, and the number of cases that can be safely embolized is limited. In this study, we present a novel embolization procedure for the meningohypophyseal trunk and inferolateral trunk using the distal balloon protection technique and detail its clinical efficacy and complication risks. We developed this procedure to allow safe embolization in patients who cannot be adequately cannulated with microcatheters into these arteries., Materials and Methods: Patients who underwent meningohypophyseal trunk or inferolateral trunk embolization using the distal balloon protection technique for skull base tumors at our institution between 2010 and 2023 were included. In this procedure, the ICA was temporarily occluded with a balloon at the ophthalmic artery bifurcation, the microcatheter was guided to the meningohypophyseal trunk or inferolateral trunk vicinity, and embolic particles were injected into the arteries. The balloon was deflated after the embolic particles that had refluxed into the ICA were aspirated., Results: A total of 25 meningohypophyseal trunks and inferolateral trunks were embolized during 21 operations. Of these 25 arteries, only 9 (36.0%) were successfully cannulated with microcatheters. Nevertheless, effective embolization was achieved in all cases. Permanent complications occurred in only 1 case (4.8%) in which the central retinal artery was occluded during inferolateral trunk embolization, resulting in a visual field defect. No permanent complications resulting from the embolic cerebral infarction were observed. Of 16 cases that underwent MR imaging within a week after embolization, however, 11 (68.8%) demonstrated embolic cerebral infarctions., Conclusions: In patients with skull base tumors with meningohypophyseal trunk or inferolateral trunk feeders that cannot be catheterized directly, embolization using the distal balloon protection technique for tumor supply can be considered as a salvage technique., (© 2024 by American Journal of Neuroradiology.)

The role of systemic therapy in primary or advanced and metastatic chordoma has been traditionally limited because of the inherent resistance to cytotoxic therapies and lack of specific or effective therapeutic targets. Despite resection and adjuvant radiation therapy, local recurrence rates in clival chordoma remain high and the risk of systemic metastases is not trivial, leading to significant morbidity and mortality. Recently, molecular targeted therapies (MTTs) and immune checkpoint inhibitors (ICIs) have emerged as promising therapeutic avenues in chordoma. In recent years, preclinical studies have identified potential targets based on intrinsic genetic dependencies, epigenetic modulators, or newly identified tumor-associated cell populations driving treatment resistance and recurrence. Nonetheless, the role of systemic therapies in the neoadjuvant or adjuvant setting for primary, locally progressive, and distant metastatic chordomas is still being investigated. Herein, an overview of current and emerging systemic treatment strategies in advanced clival chordoma is provided. Furthermore, several molecular biomarkers have been recently uncovered as potential predictors of the response to specific molecular therapeutics. The authors describe the recently discovered role of 1p36 and 9p21 deletions as biomarkers capable of guiding drug selection. Then they discuss completed and ongoing clinical trials of MTTs, including several tyrosine kinase inhibitors used as monotherapy or in combination, such as imatinib, sorafenib, dasatinib, and lapatinib, among others, as well as mammalian target of rapamycin inhibitors such as everolimus and rapamycin. They present their experience and other recent studies demonstrating vast benefits in advanced chordoma from ICIs. Additionally, they provide a brief overview of novel systemic strategies such as adoptive cell transfer (CAR-T and NK cells), oncolytic viruses, epigenetic targeting (KDM6, HDAC, and EZH2 inhibitors), and several promising preclinical studies with high translational potential. Finally, the authors present their institutional multidisciplinary protocol for the incorporation of systemic therapy for both newly diagnosed and recurrent chordomas based on molecular studies including upfront enrollment in MTT trials in patients with epidermal growth factor receptor upregulation or INI-1 deficiency or enrollment in ICI clinical trials for patients with high tumor mutational burden or high PD-L1 expression on tumor cells or in the tumor microenvironment.

Chordomas are tumors thought to originate from notochordal remnants that occur in midline structures from the cloves of the skull base to the sacrum. In adults, the most common location is the sacrum, followed by the clivus and then mobile spine, while in children a clival origin is most common. Most chordomas are slow growing. Clinical presentation of chordomas tend to occur late, with local invasion and large size often complicating surgical intervention. Radiation therapy with protons has been proven to be an effective adjuvant therapy. Unfortunately, few adjuvant systemic treatments have demonstrated significant effectiveness, and chordomas tend to recur despite intensive multimodal care. However, insight into the molecular underpinnings of chordomas may guide novel therapeutic approaches including selection for immune and molecular therapies, individualized prognostication of outcomes, and real-time noninvasive assessment of disease burden and evolution. At the genomic level, elevated levels of brachyury stemming from duplications and mutations resulting in altered transcriptional regulation may introduce druggable targets for new surgical adjuncts. Transcriptome and epigenome profiling have revealed promoter- and enhancer-dependent mechanisms of protein regulation, which may influence therapeutic response and long-term disease history. Continued scientific and clinical advancements may offer further opportunities for treatment of chordomas. Single-cell transcriptome profiling has further provided insight into the heterogeneous molecular pathways contributing to chordoma propagation. New technologies such as spatial transcriptomics and emerging biochemical analytes such as cell-free DNA have further augmented the surgeon-clinician's armamentarium by facilitating detailed characterization of intra- and intertumoral biology while also demonstrating promise for point-of-care tumor quantitation and assessment. Recent and ongoing clinical trials highlight accelerating interest to translate laboratory breakthroughs in chordoma biology and immunology into clinical care. In this review, the authors dissect the landmark studies exploring the molecular pathogenesis of chordoma. Incorporating this into an outline of ongoing clinical trials and discussion of emerging technologies, the authors aimed to summarize recent advancements in understanding chordoma pathogenesis and how neurosurgical care of chordomas may be augmented by improvements in adjunctive treatments.

Hemangiopericytoma (HPC) is a rare tumor originating from Zimmerman pericytes. It constitutes less than 1% of all intracranial tumors. Because of the high recurrence rates, radiotherapy (RT) is a vital step in the treatment, but the timing and dose remain uncertain. We presented a 39-year-old patient with a high-grade hemangiopericytoma located at the skull base. The patient presented with a severe headache. Cranial magnetic resonance imaging (MRI) revealed a mass of size of 60 × 50 mm. A subtotal resection was performed that confirmed the diagnosis of HPC. The patient received 66 Gy postoperative RT. Adjuvant sunitinib treatment was initiated after RT. The complete regression was achieved in the third month after RT. The patient has a complete response for 25 months. To our knowledge, this case is one of the rare cases in the literature in which immediate RT and adjuvant chemotherapy were used in combination with surgery. Although HPC is a radio- and chemo-resistant disease, a trimodality treatment approach can provide the patients with a complete response. In particular, patients with high-grade and inoperable tumors and patients who undergo subtotal resection should be evaluated for trimodal therapy., (Copyright © 2023 Copyright: © 2023 Indian Journal of Cancer.)

Cranial chordoma is a rare neoplasm that is challenging to both diagnose and manage. We present our experience in treating a case of clival chordoma mimicking a nasopharyngeal mass without any signs of cranial deficits or intracranial insult. Management was comprised of endoscopic transnasal debulking surgery followed by radiotherapy. However, we failed to achieve an oncologic outcome due to the development of lethal central nervous system and respiratory infections 1 month after the surgery., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: This article reviews the presenting features, molecular characteristics, diagnosis, and management of selected skull base tumors, including meningiomas, vestibular schwannomas, pituitary neuroendocrine tumors, craniopharyngiomas, chordomas, ecchordosis physaliphora, chondrosarcomas, esthesioneuroblastomas, and paragangliomas., Latest Developments: Skull base tumors pose a management challenge given their complex location and, as a result, the tumors and treatment can result in significant morbidity. In most cases, surgery, radiation therapy, or both yield high rates of disease control, but the use of these therapies may be limited by the surgical accessibility of these tumors and their proximity to critical structures. The World Health Organization classification of pituitary neuroendocrine tumors was updated in 2022. Scientific advances have led to an enhanced understanding of the genetic drivers of many types of skull base tumors and have revealed several potentially targetable genetic alterations. This information is being leveraged in the design of ongoing clinical trials, with the hope of rendering these challenging tumors treatable through less invasive and morbid measures., Essential Points: Tumors involving the skull base are heterogeneous and may arise from bony structures, cranial nerves, the meninges, the sinonasal tract, the pituitary gland, or embryonic tissues. Treatment often requires a multidisciplinary approach, with participation from radiation oncologists, medical oncologists, neuro-oncologists, and surgical specialists, including neurosurgeons, otolaryngologists, and head and neck surgeons. Treatment has largely centered around surgical resection, when feasible, and the use of first-line or salvage radiation therapy, with chemotherapy, targeted therapy, or both considered in selected settings. Our growing understanding of the molecular drivers of these diseases may facilitate future expansion of pharmacologic options to treat skull base tumors., (Copyright © 2023 American Academy of Neurology.)

Background and Purpose: Skull base metastases are a severe complication of various malignant tumors. If cranial nerves are involved even small lesions can cause significant symptoms. Specific clinical characteristics like neurological symptoms, associated primary tumors, prognosis and optimal treatment are poorly defined and are systematically described in this article., Methods: In a monocentric retrospective study patients with skull base metastases and cranial nerve deficits who received treatment between 2006 and 2018 were analyzed concerning clinical characteristics at initial diagnosis, treatment and course of the disease., Results: In this study 45 patients with skull base metastases and cranial nerve deficits were included. The most frequent primary tumors were prostate cancer (27%), breast cancer (22%) and multiple myeloma (16%). The most involved cranial nerves were trigeminal nerve (42%), oculomomotor nerve (33%) and facial nerve (27%). Of the patients 84% had additional bone metastases outside the skull base. Dural infiltration or meningeal carcinomatosis were each observed in 13% of the patients. After radiotherapy cranial nerve deficits remained stable in 61% of all cases and in 22% symptoms improved. Median overall survival from treatment was 8 months (range 0.4-51 months). Patients with dose-escalated radiotherapy appeared to live longer (16.4 months vs. 4.7 months). This effect persisted in a multivariate analysis including the Karnofsky index, number of metastases, primary tumor and radiation dose (HR 0.37, p = 0.02)., Conclusion: Skull base metastases with cranial nerve deficits are complex diseases with poor prognosis. Precise diagnosis and treatment are required. Further research is needed to improve treatment., (© 2022. The Author(s).)

Background and Purpose: Skull base tumors are commonly supplied by dural branches of the meningohypophyseal and inferolateral trunks. Embolization through these arteries is often avoided due to technical challenges and inherent risks; however, successful embolization can be a valuable surgical adjunct. We aimed to review the success and complications in our series of tumor embolizations through the meningohypophyseal and inferolateral trunks., Materials and Methods: We performed a retrospective review of patients with tumor treated with preoperative embolization at our institution between 2010 and 2020. We reviewed the following data: patients' demographics, tumor characteristics, endovascular embolization variables, and surgical results including estimated blood loss, the need for transfusion, and operative time., Results: Among 155 tumor embolization cases, we identified 14 patients in whom tumor embolization was performed using the meningohypophyseal ( n = 13) or inferolateral ( n = 4) trunk. In this group of patients, on average, 79% of tumors were embolized. No mortality or morbidity from the embolization procedure was observed in this subgroup of patients. The average estimated blood loss in the operation was 395 mL (range, 200-750 mL). None of the patients required a transfusion, and the average operative time was 7.3 hours., Conclusions: Some skull base tumors necessitate embolization through ICA branches such as the meningohypophyseal and inferolateral trunks. Our series demonstrates that an effective and safe embolization may be performed through these routes., (© 2022 by American Journal of Neuroradiology.)

Objective: Preoperative embolization for intracranial meningiomas can cause tumor necrosis, reduce intraoperative blood loss, and facilitate surgery. This study aimed to evaluate the efficacy of tumor embolization using Embosphere microspheres for skull base meningiomas and analyze postembolization plain computed tomography (CT) and magnetic resonance imaging (MRI) scans to identify findings that could potentially predict treatment response., Methods: Between April 2014 and April 2020, 80 patients with skull base meningiomas presenting at our medical center underwent embolization with Embosphere microspheres. The effects of tumor embolization were evaluated through a comparison of postembolization plain CT and contrast-enhanced MRI., Results: A total of 143 vessels (102 of 108 external carotid artery branches and 41 of 65 internal carotid artery branches) from 80 skull base meningiomas were embolized with Embosphere microspheres. Microspheres 100-300 μm in size were used in 2 cases, microspheres 300-500 μm in size were used in 12 cases, and microspheres 500-700 μm in size were used in 66 cases. Postembolization contrast-enhanced MRI showed reductions in enhancing lesions within the tumor in 55 of 80 cases. Postembolization plain CT scans showed high-density lesions within the tumor in 41 of 55 cases. Thus, reductions in enhancing lesions on postembolization contrast-enhanced MRI were statistically significantly associated with the presence of high-density lesions on postembolization plain CT (P < 0.001). Embolization-related neurological complications occurred in 3 cases., Conclusions: Embosphere microspheres are user friendly and effective embolic materials for the embolization of skull base meningiomas. Postembolization contrast-enhanced MRI and plain CT findings may be useful for evaluating the effects of tumor embolization., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Benign bony tumors of the skull base and paranasal sinuses are uncommon entities, with an overall higher incidence in males. Benign bony tumors may lead to local expansion with resultant mass effect of potentially critical structures. Some benign bony tumors may undergo malignant transformation. This article reviews the presentation and management of benign bone tumors of the skull base and paranasal sinuses with special consideration to involvement of the adjacent orbit, intracranial and critical neurovascular structures. This review covers tumor incidence, location, gross and histologic appearance as well as radiographic findings, treatment, and recurrence rates. Tumors discussed in this article include osteochondromas, osteomas, osteoid osteomas, aneurysmal bone cysts, fibrous dysplasia, giant cell tumors, cemento-ossifying fibroma, ameloblastic fibro-odontoma, ecchordosis physaliphora, chondromyxoid fibroma, primary chronic osteomyelitis, primary chronic osteomyelitis, osteochondromyxoma, and dense bone islands., (Copyright © 2022. Published by Elsevier Inc.)

Tumors that arise in and around the skull base comprise a wide range of common and rare entities. Recent studies have advanced our understanding of their pathogenesis, which in some cases, have significantly influenced clinical practice. The genotype of meningiomas is strongly associated with their phenotype, including histologic subtype and tumor location, and clinical outcome. A single molecular alteration, NAB2-STAT6 fusion, has redefined the category of solitary fibrous tumors to include the previous entity hemangiopericytomas. Schwannomas, both sporadic and familial, are characterized by near ubiquitous alterations in NF2 , with additional mutations in SMARCB1 or LZTR1 in schwannomatosis. In pituitary adenohypophyseal tumors, cell lineage transcription factors such as SF-1, T-PIT, and PIT-1 are now essential for classification, providing a more rigorous taxonomy for tumors that were previously considered null cell adenomas. The pituicyte lineage transcription factor TTF-1 defines neurohypophyseal tumors, which may represent a single nosological entity with a spectrum of morphologic manifestations (ie, granular cell tumor, pituicytoma, and spindle cell oncocytoma). Likewise, the notochord cell lineage transcription factor brachyury defines chordoma, discriminating them from chondrosarcomas. The identification of nonoverlapping genetic drivers of adamantinomatous craniopharyngiomas and papillary craniopharyngiomas indicates that these are distinct tumor entities and has led to successful targeted treatment of papillary craniopharyngiomas using BRAF and/or mitogen-activated protein kinase inhibitors. Similarly, dramatic therapeutic responses have been achieved in patients with Langerhans cell histiocytosis, both with BRAF -mutant and BRAF -wildtype tumors. Familiarity with the pathology of skull base tumors, their natural history, and molecular features is essential for optimizing patient care., (Copyright © Congress of Neurological Surgeons 2021. All rights reserved.)

Objective: Skull base osteosarcoma is a rare and aggressive tumor that is most commonly treated with primary surgical resection and adjuvant chemoradiation. Using the National Cancer Database, we analyzed demographic and clinical prognosticators for overall survival (OS)., Methods: The National Cancer Database was queried for cases of histologically confirmed skull base osteosarcoma treated between 2004 and 2015, excluding patients receiving palliation or having <1 month of follow-up. A total of 314 patients treated with surgery alone (n = 82), surgery with adjuvant radiotherapy (n = 35), surgery with chemotherapy (n = 114), or trimodality therapy (n = 56) were identified. The χ 2 test for categorical variables, Cox proportional hazards models, and Kaplan-Meier log-rank analysis were used to test associations with treatment, OS, and survival time., Results: None of the studied demographic characteristics (age, sex, race, overall health) and socioeconomic factors (income and average regional education) were associated with OS (none P < 0.05). Treatment modalities also did not show a significant association with OS (none P < 0.05). Certain tumor characteristics showed an association with OS, with fibroblastic and Paget histologic subtypes (each P = 0.003), poorly differentiated tumor grade (P = 0.03), and tumor size >5 cm (P = 0.045) associated with poorer OS., Conclusions: Tumor histologic subtype, advanced tumor grade, and greater tumor size are predictors of worse OS in skull base osteosarcoma. No significant differences in OS were identified based on treatment modality, which warrants further investigation., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Summary: Traditionally, surgical access for extirpation and reconstruction of midfacial tumors requires external incisions that can cause a myriad of complications, especially in irradiated patients. The modern midfacial degloving approach involves hidden, sublabial and intranasal incisions that provide reliable access for free flap reconstruction of nasal and maxillofacial tumors. Seven patients with a history of radiation therapy underwent free flap reconstruction of the midface. Five patients underwent the technique in a delayed manner, and the remaining two underwent reconstruction immediately at the time of resection. Five patients underwent free radial forearm fasciocutaneous free flaps and two underwent reconstruction with anterolateral thigh perforator flaps. Bone and/or rib cartilage grafting was used in all patients. All patients underwent successful free flap reconstruction of the midface without external incisions. The most common complication was postoperative infection requiring oral or intravenous antibiotics. No patients sustained loss of their grafts or hardware in the postoperative period. The midfacial degloving technique provides satisfactory exposure to the nasal cavity, midface, orbits, and skull base for free flap reconstruction, without disrupting the external soft tissue. The authors describe a novel use of the midfacial degloving technique to provide safe and reliable results with improved cosmetic outcome., (Copyright © 2021 by the American Society of Plastic Surgeons.)

Background: Chondrosarcomas of the skull base are rare intracranial tumors of chondroid origin. The rarity of these lesions has made it difficult to form a consensus on optimal treatment regimens. The aim of this study was to provide a comprehensive analysis of prognostic factors, treatment modalities, and survival outcomes in patients with chondrosarcoma of the skull base., Methods: Patients with diagnosis codes for chondrosarcoma of the skull base were queried from the National Cancer Database for the years 2004-2016. Outcomes were investigated using Cox univariate and multivariate regression analyses, and survival curves were generated for comparative visualization., Results: A total of 718 patients with chondrosarcoma of the skull base were identified. Mean overall survival (OS) in these patients was 10.7 years. Older age and presence of metastases were associated with worsened OS. Of patients, 83.3% received surgical intervention, and both partial resection and radical resection were associated with significantly improved OS (P < 0.001). Neither radiotherapy nor chemotherapy improved OS; however, patients who received proton-based radiation and patients who received high-dose radiation (≥6000 cGy) had significantly improved OS compared with patients who received traditional radiation., Conclusions: In the largest study to our knowledge of skull base chondrosarcoma to date, both partial resection and radical resection significantly improved OS, thus supporting the goal of maximal safe resection to preserve vital neurovascular structures without sacrificing outcome. In patients who received radiotherapy, proton-based modalities and high-dose radiation were associated with increased OS., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Skull base primary malignancies represent a heterogeneous group of histologic diagnoses and sarcomas of the skull base are specific malignant tumors that arise from mesenchymal cells and can be classified by site of origin into bony and soft tissue sarcomas. The most common bony sarcomas include: chondrosarcoma, osteosarcoma, chordoma, Ewing's sarcoma. Given the relative rarity of each histologic diagnosis, especially in the skull base, there is limited published data to guide the management of patients with skull base sarcomas. An electronic search of the literature was performed to obtain key publications in the management of bony sarcomas of the skull base published within the last decade. This article is thus a review of the multi-disciplinary management principles of primary bony sarcomas of the skull base. Of note, there have been several recent advancements in the realm of skull base sarcoma management that have resulted in improved survival. These include advances in: imaging and diagnostic techniques, surgical techniques that incorporate oncologic surgical principles, conformal radiation paradigms and targeted systemic therapies. Early access to coordinated multi-disciplinary subspecialty care immediately at suspicion of diagnosis has further improved outcomes. There are several ongoing trials in the realms of radiation therapy and systemic therapy that will hopefully provide further insight about the optimal management of bony sarcomas of the skull base.

Management of solid tumors involving the skull base are primarily managed with surgery and radiation, though proximity to important vascular and neuroanatomic structures often limit the extent of resection and permissible radiation dose. Meningiomas are the most common primary brain tumor in adults, and although the majority of skull base meningiomas are low-grade, their location in proximity to critical anatomical structures precludes aggressive surgical resection, and larger tumors are often resistant to radiation treatment. In patients with clinically aggressive, unresectable meningiomas, several molecular biomarkers of angiogenesis, as well as genetic mutations (SMO, AKT1, PIK3CA, KLF4, POLR2, SMARCE1, and TRAF7), have been shown to play a crucial role in the pathophysiology of these tumors. Pituitary adenomas are commonly slow growing tumors that are amenable to surgical resection, but tumors with higher Ki67 proliferative indices are associated with an increased risk of relapse and resistance to standard therapies. Chemotherapeutic agents and checkpoint inhibitors have been trialed, albeit with limited success, to treat these aggressive pituitary adenomas. Craniopharyngiomas are categorized as adamantinomatous and papillary subtypes, each with unique molecular mechanisms that drive pathogenesis of these tumors, and have introduced the possibility that targeted therapies may be developed for improved neurologic and endocrinological outcomes. Skull base tumors that exhibit recurrence despite surgical resection and radiation treatment pose a unique challenge, and systemic agents offer a non-invasive option of treating tumors that are refractory to conventional approaches. Recent insights into the molecular aberrations that elucidate the pathophysiology of these difficult-to-treat tumors have provided potential therapeutic targets for drug delivery. In this review, the authors discuss promising therapies and current knowledge gaps needed for the development of effective targeted agents for meningioma, pituitary adenoma, and craniopharyngioma.

Purpose: The purpose of this review is to assess the recent evidence regarding the management of squamous cell carcinoma of the skull-base and to discuss the implications of these findings on clinical practice., Method: Free text Medline and MeSH term search of publications relating to Squamous Cell Carcinoma & Skull-base and Skull base, Neoplasm respectively. Multidisciplinary clinical guidelines were also reviewed., Results: The primary search yielded a total of 271 papers which following initial review was reduced to 28. Secondary search yielded 56 papers. There were no randomised controlled trials relating to squamous cell carcinoma of the skull-base and as such this review is based on cohort studies, case series and expert opinion., Conclusion: Squamous cell carcinoma (SCC) is the most common cancer occurring in the Head and Neck. Squamous cell carcinoma is also the most common cancer arising within the nose and sinuses of which skull-base squamous cell carcinoma is a rare subgroup. Evidence relating to the management and survival of skull-base SCC is based on expert opinion and. retrospective analyses Clinical examination and biopsy, imaging and a broad multidisciplinary team are key to the management of skull-base SCC. The information gathered should be used to guide informed discussion by suitably trained experts with patients regarding surgical approach, post-operative recovery and adjuvant or neoadjuvant treatments. The standard of care is currently to perform skull base resection with or without additional craniotomy, pedicled or free flap reconstruction in multiple layers and post-operative radiation (usually photons or protons). Open approaches have traditionally been the mainstay, however in certain cases endoscopic approaches can yield equivalent results and offer many advantages. Despite advances in care survival remains poor with a nearly one in five risk of nodal recurrence within two years.

Introduction: Sinonasal adenocarcinomas (SNAC) are rare and heterogeneous. Management of SNAC follows a rather standardized and internationally accepted paradigm. Several refinements have been introduced during the last decade., Methods: A narrative review of most updated literature on SNACs has been conducted., Results: SNACs are classified as intestinal-type and non-intestinal-type, which are further categorized according to grade. Preoperative work-up should include magnetic resonance imaging (or contrast-enhanced computed tomography as a secondary or complementary choice) and biopsy under general anesthesia, or under local anesthesia in case of a history of exposure to wood and/or leather dust. Positron emission tomography, neck ultrasound, and fine-needle aspiration cytology are indicated in selected cases. Surgery represents the most common upfront modality of treatment and is usually accomplished via a transnasal endoscopic approach. Adjuvant radiation therapy is indicated for high-grade, locally advanced tumors and/or in case of margins involvement. Neoadjuvant chemotherapy with cisplatin, 5-fluorouracil and leucovorin may offer high response rates and long-term control in a subgroup of patients affected by intestinal-type adenocarcinoma, and in particular in those whose tumors harbor a functional p53 protein. Most of the bio- and immune-therapeutic potentials on SNACs still remain theoretical, and no clinical data are currently available., Conclusions: Management of SNAC consists of histological diagnosis, radiological staging, radical surgery, and adjuvant radiation therapy. Neoadjuvant chemotherapy can be indicated in selected cases. The role of biotherapy and immune therapy still needs to be elucidated.

Introduction: Sinonasal tumors that harbor neuroendocrine histologic features include olfactory neuroblastoma (previously known as esthesioneuroblastoma), sinonasal neuroendocrine carcinoma, and sinonasal undifferentiated carcinoma. These tumors represent a diverse spectrum of clinical behavior and as such require histology-specific management. Herein, we review the management of these sinonasal tumors with neuroendocrine features and discuss fundamentals of multi-modality care for each histology. An emphasis is placed on olfactory neuroblastomas, given their relative frequency and skullbase origin., Methods: A comprehensive literature review on contemporary management of olfactory neuroblastoma, sinonasal neuroendocrine carcinoma, and sinonasal undifferentiated carcinoma was performed., Results: Management of sinonasal tumors with neuroendocrine features can include surgical resection, radiation therapy, and/or chemotherapy. Due to their site of origin, these tumors can frequently involve the skullbase, which can require site-specific care. The optimal treatment modalities and the sequence in which they are performed are largely dependent on histology. In most cases, olfactory neuroblastoma is best managed with surgical resection followed by radiation therapy. Sinonasal neuroendocrine carcinomas represent a variety of histologic phenotypes (carcinoid, atypical carcinoid, small cell, and large cell), which determine the optimal treatment modality. Finally, sinonasal undifferentiated carcinoma is likely best managed by induction chemotherapy with subsequent therapy dictated by the initial response., Conclusions: A team approach to multi-modality care is essential in the treatment of olfactory neuroblastoma, sinonasal neuroendocrine carcinoma, and sinonasal undifferentiated carcinoma. Early biopsy, histologic diagnosis, and comprehensive imaging are critical to determining the appropriate management paradigm.

Objective: The purpose of this article was to determine the impact of employing a telephone clinic for follow-up of patients with stable lateral skull-base tumours., Method: An analysis of 1515 patients in the national lateral skull-base service was performed, and 148 patients enrolled in the telephone clinic to date were identified. The length of time that patients waited for results of their follow-up scans and the travel distance saved by patients not having to attend the hospital for their results was determined., Results: The mean time from scan to receiving results was 30.5 ± 32 days, 14 days sooner than in the face-to-face group (p = 0.0016). The average round-trip distance travelled by patients to the hospital for results of their scans was 256 ± 131 km., Conclusion: The telephone clinic led to a significant reduction in time until patients received their scan results and helped reduce travel distance and clinic numbers in traditional face-to-face clinics.

Introduction: This article provides an overview of the natural history and management of adenoid cystic carcinoma infiltrating the skull base (SB ACC)., Methods: A comprehensive review of the available literature and discussion regarding the current management of SB ACC., Results: This review describes the unique pathology, appropriate diagnostic work-up and contemporary management options in SB ACC. There is a specific focus on the role of surgical resection with post-operative radiotherapy, given the literature suggesting a superior locoregional control rate. The importance of surgical decision making and varying surgical approaches are detailed. In addition, the emerging role of biological agents is discussed., Conclusion: The gold standard for SB ACC is surgical resection and post-operative radiation, given the superior locoregional control. In patients not amendable to surgery, palliative radiation should be considered. The role of biological agents is still evolving. The complexity of SB ACC management mandates experienced multi-speciality management.

Autopsy studies suggest that skull base metastases are likely underrecognized in patients with cancer. Patients frequently present with one or a combination of skull base clinical syndromes that manifest as pain or cranial neuropathy. Once a skull base metastasis is suspected, establishing a histologic diagnosis, dedicated imaging, and restaging (if appropriate) are the first steps in management. A multidisciplinary approach should then be used to identify the optimal histology-based treatment strategy, taking into account the burden of systemic disease. Finally, definitive treatment may include one or a combination of surgical management, radiation therapy, or chemotherapy.", Competing Interests: Disclosure The authors have nothing to disclose. The authors received no funding., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Chordomas are the rare malignant bone lesions derived from the embryonic notochord. They are slow-growing tumors with a locally aggressive behavior. The clival chordomas are extradural, exophytic, and lytic lesions centered on the clivus, and are managed differently from those arising elsewhere because of the emphasis on preserving the neurological function. The gold standard for therapy is the complete resection followed by radiation therapy for a better local control of the tumor. This case report concerns a 20-year old girl with an incidental diagnosis of the clival chordoma, which was first treated via an endoscopic anterior approach to remove the exophytic portion of the tumor. The adjuvant radiation therapy was not feasible because of the macroscopic intradural residual tumor being at the level of jugular foramen. The girl was referred to our institute for the removal of the intradural residual tumor via a petro-occipital trans-sigmoid (POTS) approach followed by adjuvant proton beam therapy. The choice of the surgical approach depends on the size of the tumor, its location, and its anatomical distribution, but should also be balanced against the morbidity, considering the patient's age, and the feasibility of postoperative rehabilitation.

The SARS-CoV-2 pandemic has rapidly transformed health care delivery around the globe. Because of the heavy impact of COVID-19 spread, cancer treatments have necessarily been de-prioritized, thus exposing patients to increased risk of morbidity and mortality due to delayed care. In this scenario, cancer specialists need to assess critical oncology patients case by case to carefully balance risk vs benefit in treating tumors and preventing SARS-CoV-2 infection. Here, we report early insights into how the management of patients with sinonasal and anterior skull base cancer might be affected by the COVID-19 pandemic. We provide recommendations for preoperative tests, indications for immediate care vs possible delayed treatment, and warnings relating to dural resection and intracranial dissection, given the potential neurotropism of SARS-CoV2 and practical suggestions for managing cancer care in a period of limited resources. We also postulate some thoughts on the promising role of telemedicine in multidisciplinary case discussions and posttreatment surveillance., (© 2020 Wiley Periodicals, Inc.)

Objective: Infratemporal fossa (ITF) tumors are unique in histological characteristics and difficult to treat. Predictors of patient outcomes in this context are not known. The objective of this study was to identify independent predictors of outcome and to characterize patterns of failure in patients with ITF carcinoma., Methods: All patients who had been surgically treated for anterolateral skull base malignancy between 1999 and 2017 at the authors' institution were retrospectively reviewed. Patient demographics, preoperative performance status, tumor stage, tumor characteristics, treatment modalities, and pathological data were collected. Primary outcomes were disease-specific survival (DSS) and local progression-free survival (LPFS) rates. Overall survival (OS) and patterns of progression were secondary outcomes., Results: Forty ITF malignancies with skull base involvement were classified as carcinoma. Negative margins were achieved in 23 patients (58%). Median DSS and LPFS were 32 and 12 months, respectively. Five-year DSS and OS rates were 55% and 36%, respectively. The 5-year LPFS rate was 69%. The 5-year overall PFS rate was 53%. Disease recurrence was noted in 28% of patients. Age, preoperative performance status, and margin status were statistically significant prognostic factors for DSS. Lower preoperative performance status and positive surgical margins increased the probability of local recurrence., Conclusions: The ability to achieve negative margins was significantly associated with improved tumor control rates and DSS. Cranial base surgical approaches must be considered in multimodal treatment regimens for anterolateral skull base carcinomas.

Objective: To determine factors affecting outcomes for patients with sinonasal and nasopharyngeal adenoid cystic carcinoma (SNACC) treated using the endoscopic endonasal approach (EEA) with preservation of key structures followed by adjuvant radiotherapy (RT)., Method: Retrospective case series of 30 patients treated at the University of Pittsburgh between 2000 and 2014. Hospital records were reviewed for clinical and pathologic data. Outcome measures included overall survival (OS), disease-free survival (DFS), local recurrence-free survival (LRFS) and distant metastasis-free survival (DMFS) rates., Results: The majority of patients had T4a and T4b disease (23.3%, and 63.3%). Microscopically positive margins were present in 21 patients (63.6%). Positive margins were present in nine patients (30.0%). The mean and median follow-up were 3.97 and 3.29 years. Five-year OS, DFS, LRFS, and DMFS were 62.66%, 58.45%, 87.54%, and 65.26%. High-/intermediate-grade tumors had worse DFS (P = .023), and LRFS (P = .026) (HR = 4.837, 95% CI, 1.181-19.812). No factors were associated with significantly worse DMFS. No patient suffered CSF leak, optic nerve, or internal carotid injury. The mean and median length of hospital stay was 4.1 days and 2.0 days (range: 0-32 days)., Conclusion: Organ-preserving EEA with adjuvant RT for low-grade SNACC offers 5-year survival similar to that reported by other studies, which include radical, open skull base surgery. Patients with high-grade disease do poorly and may benefit from novel treatment strategies. For low-grade disease, organ-preserving EEA with RT may be the best option, offering a balance of survival, quality of life, and decreased morbidity for patients with this difficult-to-cure disease., Level of Evidence: 4 Laryngoscope, 130:1414-1421, 2020., (© 2019 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: Jugular foramen paragangliomas are highly vascular tumors known to have significant venous hemorrhage during resection even after conventional transarterial embolization. The authors report a novel technique to the endovascular embolization of jugular foramen paragangliomas using a combined transarterial and transvenous access for better intraoperative control of blood loss and visualization., Methods: This is a retrospective data collection of 2 patients diagnosed with jugular foramen paragangliomas with novel embolization technique and surgical resection., Results: Two patients underwent embolization of jugular foramen paragangliomas through combined transarterial and transvenous routes using 2 double-lumen balloon microcatheters. In both cases, single arterial vessel embolization was performed through the occipital artery in Case 1 and the tympanic branch of the ascending pharyngeal artery in Case 2. Simultaneously, balloon microcatheter occlusion in the sigmoid sinus and single venous outflow vessel embolization was performed. Near-complete occlusion was established, with angiographic disappearance of tumor blush. Surgical resection was performed in both cases. Estimated blood loss BL was 600 mL in Case 1 and 200 mL in Case 2. No blood transfusions were required, intraoperatively or postoperatively. There were no cranial nerve deficits post embolization. One patient had a persistent House Brackman 2 facial nerve palsy after resection., Conclusions: The initial experience with simultaneous transvenous and transarterial paraganglioma embolization demonstrated the safety of the technique and superior embolic agent penetration. This was supported by our observations during embolization and intraoperatively during tumor resection. Additional patients need to be treated with this technique for better assessment of long-term efficacy and incidence of embolization-related cranial neuropathies., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Skull base paragangliomas are highly vascular tumors that are often embolized before surgical resection; however, the safety and efficacy of preoperative embolization using an ethylene vinyl alcohol copolymer (Onyx; Medtronic, Dublin, Republic of Ireland) in these tumors is unknown. This retrospective cohort study evaluated patient outcomes after preoperative embolization of skull base paragangliomas using Onyx., Methods: We retrospectively analyzed data from all patients with skull base paragangliomas who underwent preoperative Onyx embolization at our institution (January 01, 2005-December 31, 2017). Patient, tumor, embolization, and outcomes data were extracted by reviewing inpatient and outpatient clinical and imaging records., Results: Seven patients were studied (5/7 [71%] female), 6 with glomus jugulares and 1 with a glomus vagale. The median age was 52 years, and the most common presenting symptom was cranial neuropathy (6/7 [86%]). The tumor vascular supply was from the ascending pharyngeal artery in all 7 cases (100%) with additional feeders including the occipital artery in 5 (71%); internal carotid artery in 3 (43%); middle meningeal, vertebral, and internal maxillary artery each in 2 (29%); and posterior auricular artery in 1 (14%). The median postembolization tumor devascularization was 80% (range, 64-95%). The only postembolization complication was a facial palsy in 1 patient., Conclusion: Preoperative embolization with Onyx affords excellent devascularization for the majority of skull base paragangliomas, and it may facilitate resection of these hypervascular lesions. The advantages provided by Onyx with respect to penetration of intratumoral vessels must be weighed against the risk of cranial neuropathy.

Chordomas are rare primary malignant tumors of the bones that occur along the skull base, spine, and sacrum. Long-term survival and neurological outcome continue to be challenging with continued low percentages of long-term survival. Recent studies have used genome, exome, transcriptome, and proteome sequencing to assess the mutational profile of chordomas. Most notably, Brachyury, or T-protein, has been shown to be an early mutational event in chordoma evolution. Clinically actionable mutations, including in the PI3K pathway, were identified. Preliminary evidence suggests that there may be mutational differences associated with primary tumor location. In this study, we review the therapeutic landscape of chordomas and discuss emerging targets in the genomic era., (Copyright © 2019 by the Congress of Neurological Surgeons.)

Sinonasal tumours are rare, and among these there exist a small number of histologic subtypes that are infrequently encountered and rarely mentioned in the literature. These have been presented as either case reports or small case series, and their very low incidence makes prospective studies practically impossible. This review analyses the available literature, including our own experience and endeavours to outline management strategies, which involve a high index of suspicion and counselling of patients. In most instances, these tumours require aggressive multimodal treatment to improve survival outcomes. The overall prognosis remains dismal., (© 2020 S. Karger AG, Basel.)

Aim: To discuss the optimal and recent treatment options based on clinical review of 16 chordoma patients., Material and Methods: Data of the patients diagnosed and treated between 1999 and 2017 in Gazi University School of Medicine has been collected through patients’ files and the electronic database of hospital records. Statistical analysis was applied to evaluate the correlation between the progression free survival and treatment modalities., Results: Nine of the 16 patients were women (56.3%). Half of the patients had intracranially located tumors, whereas the other 50% of the sample had spinal (n=5) and sacral (n=3) chordomas. The median follow-up time was 51.7 months. Recurrence was observed in 50% of patients, while the median recurrence time equaled to 27.6 months. Multivariate analysis results showed that age, gender tumor size, intra or extracranial location of tumor, treatment modalities, subtotal or grosstotal resection of tumor, radiotherapy dose, and techniques were not associated with recurrence. On the other hand, 2 patients are still under chemoterapy (imatinib, bevacizumab) without evident of recurrent disease., Conclusion: Despite the fact that surgery remains to be the cornerstone of treatment, total resection is not reasonable for all patients with chordomas. For this reason, adjuvant treatment for ensuring local control is highly important. If the residual tumor is of a small volume, SBRT may provide more advantages. Targeted treatment or chemotheapeutic agents may also be benificial for maintanence therapy. As the clinical awareness about chordomas is based on our series, aggressive multi-modality treatment options should be applied in the adjuvant therapy.

Objective: The object of this study was to clarify the expression characteristics and prognostic value of survivin in skull base chordomas., Methods: In this retrospective study, the authors measured the expression of survivin at the mRNA level in 81 samples from 71 patients diagnosed with skull base chordomas at their hospital in the period from July 2005 to January 2015. Clinical data collection, follow-up, and survival analyses were performed, and correlations were analyzed., Results: Of the 71 patients, 50 had primary chordomas with a mean survivin expression level of 1.09; the other 21 patients had recurrent chordomas with a mean survivin expression level of 2.57, which was 2.36 times higher than the level in the primary chordoma patients (p < 0.001, Mann-Whitney U-test). In addition, an analysis of 18 paired samples derived from 9 patients showed that the expression level of survivin was 2.62 times higher in recurrent tumors than in primary tumors (p = 0.002, paired t-test). The Spearman rank correlation coefficient method showed that the expression level of survivin was positively correlated with the mean ratio of tumor signal intensity to the signal intensity of surrounding brainstem on T1-weighted sequences (RT1; rs = 0.274, p = 0.021) and was negatively correlated with the mean ratio of tumor signal intensity to the signal intensity of surrounding brainstem on T2-weighted sequences (RT2; rs = -0.389, p = 0.001). A multivariate Cox proportional-hazards model suggested that pathology (p = 0.041), survivin expression level (p = 0.018), preoperative Karnofsky Performance Status (KPS; p = 0.012), and treatment history (p = 0.009) were independent prognostic factors for tumor progression. Survivin expression level (p = 0.008), preoperative KPS (p = 0.019), tumor diameter (p = 0.027), and intraoperative blood loss (p = 0.015) were independent prognostic factors for death., Conclusions: Survivin expression level and preoperative KPS were independent significant prognostic factors for tumor progression and death in skull base chordoma patients. Recurrent skull base chordomas and chordomas with high RT1 and low RT2 were likely to have high survivin expression. Other independent risk factors related to tumor progression included conventional pathology and treatment history, whereas additional mortality-related risk factors included larger tumor diameter and greater intraoperative blood loss.

Squamous cell carcinoma (SCC) is one of the most common malignant neoplasms involving the anterior skull base, resulting from local invasion from an adjacent anatomical region. The primary sites of origin are most frequently the nasal cavity and paranasal sinuses, and less often the skin and orbit. Treatment strategies for these tumors have evolved, although management remains challenging due to the proximity to critical structures and their location at the intracranial-extracranial interface. The rarity of these tumors, and limited numbers at any one institution, has meant that most published series have grouped together different histologies in reporting outcomes. Data on SCC alone are thus limited. Treatment of SCC involving the anterior skull involves a multidisciplinary team approach. A number of potential treatment options exist, dependent on tumor, patient, and institutional factors. Current treatment strategies commonly involve multimodality therapy using a combination of surgery, radiotherapy, and chemotherapy. Primary surgery is considered the mainstay of initial treatment in those tumors which are deemed resectable, followed by adjuvant radiotherapy with or without chemotherapy. Preoperative assessment includes confirmation of the tissue diagnosis, and imaging to assess resectability, guide the surgical approach, and to plan the extent of surgery and method of reconstruction. Analysis of our institutional database for patients undergoing surgery with SCC involving the anterior skull base showed a 5-year disease-specific survival of 65% for sinonasal SCC and 71% for cutaneous SCC. The surgical margin status as well as dural and pterygopalatine fossa involvement were predictors of a poor prognosis., (© 2020 S. Karger AG, Basel.)

Anterior skull base meningiomas are a diverse group of tumors that involve different locations. The role of the expanded endoscopic transnasal approach in the management of anterior cranial fossa meningiomas has significantly changed over the past 2 decades. Patient selection is paramount to benefit from the advantages of endoscopic transnasal surgery such as direct access to ventral skull base lesions avoiding brain and brainstem retraction, early de-vascularization, removal of bone infiltrated by tumor, near-field magnification, better surgical field illumination, and minimal manipulation of neurovascular structures. Here we discuss some of the main limitations and advantages of the endoscopic transnasal approach related to patient selection, including nuances of their management, discussing preoperative imaging and planning, the surgical approach, and future perspectives in the treatment of these tumors., (© 2020 S. Karger AG, Basel.)

Malignancies of the anterior skull base are rare and recur in 40-80% of treated cases, varying with substantial variance according to histology, stage at primary diagnosis, and other factors. Most recurrences manifest within 2-5 years after primary treatment, but some histologies can relapse even decades after the first presentation. Management of recurrent anterior skull base tumors is challenging and a wide variety of treatment options are available. Similar to the primary setting, surgery is the mainstay of treatment. However, only few patients are likely to be suitable for salvage surgery after restaging. In this scenario, non-surgical options such as re-irradiation with photon or heavy particles may play a role, although the potential toxicity and benefits of treatment needs to be considered on a case-by-case basis. Moreover, stereotactic technologies are emerging as an adjunct valuable tool to minimize side effects. Chemotherapy is acquiring a relevant role in the primary treatment of sinonasal malignant lesions involving the anterior skull base in the neoadjuvant setting or in combination with radiotherapy, but evidence of its efficacy in the treatment of the recurrent/metastatic disease is very limited. The specific drugs employed vary considerably and need to be paralleled with the biology of the different histologies., (© 2020 S. Karger AG, Basel.)

Introduction: Cerebrospinal fluid (CSF) leaks usually happen in the early postoperative period. However, delayed CSF leak can happen several years after treatment. The purpose of this study was to review the characteristics and clinical course of delayed CSF leak after treating skull base tumors., Methods: We treated 9 patients with delayed CSF leak (occurring at least 3 months after treatment) between December 2015 and February 2018. Clinical data including initial treatment modality for skull base tumor, time between treatment and CSF rhinorrhea, and the result of endoscopic CSF repair were evaluated by retrospective chart review., Results: Nine patients with delayed CSF leak were evaluated. The mean age was 42.3 ± 5.2 (mean ± SD) years. Six patients with pituitary adenomas, 1 with a Rathke cleft cyst, 1 with a meningioma, and 1 with an osteosarcoma were enrolled. Seven patients (78%) received radiation, and the mean radiation dose was 55.7 ± 2.6 Gy. The median time between the end of treatment for the skull base tumor and CSF rhinorrhea was 137.0 (interquartile range 24-145) months. Seven patients (78%) had meningitis at the time of operation. CSF rhinorrhea was treated with endoscopic reconstruction using a multilayer technique. Meningitis was improved without neurologic sequelae after reconstruction and antibiotic therapy. One patient had recurrence of CSF leak., Conclusion: Patients with delayed CSF leak are more likely to have a history of radiation and to present with meningitis at diagnosis. Therefore, CSF rhinorrhea should always be suspected, even several years after treatment, if early symptoms of CSF leak develop in patients with a history of skull base tumor., (Copyright © 2019 Elsevier Inc. All rights reserved.)

A 40-year-old man presented with acute-onset painless visual loss after preoperative embolization for a large skull base meningioma through the ophthalmic artery. We describe the clinical presentation of an isolated ipsilateral afferent and efferent pupillary defect in this patient who had radiographic documentation of embolization material in the intraorbital ophthalmic artery. Although ophthalmic and retinal artery occlusions have been described previously after endovascular embolization, our patient had a unique clinicoradiologic presentation.

While proton beam therapy (PBT) can offer increased sparing of healthy tissue, it is associated with large capital costs and as such, has limited availability. Furthermore, it has not been well established whether PBT has significant clinical advantages over conventional volumetric modulated arc therapy (VMAT) for all tumour types. PBT can potentially offer improved clinical outcomes for base of skull chordoma (BOSCh) patients compared with photon (X-ray) therapy, however the cost-effectiveness of these treatments is unclear. In this study, the cost-effectiveness of PBT in the treatment of BOSCh patients is assessed, based on an analysis of comparative radiotherapy treatment plans using a radiobiological Markov model. Seven BOSCh patients had treatment plans for the delivery of intensity modulated proton therapy and VMAT retrospectively analysed. The patient outcome (in terms of tumour local control and normal tissue complications) after receiving each treatment was estimated with a radiobiological Markov model. In addition, the model estimated the cost of both the primary treatment and treating any resultant adverse events. The incremental cost-effectiveness ratio (ICER) was obtained for each patient. PBT was found to be cost-effective for 5 patients and cost-saving for 2. The mean ICER was AUD$1,990 per quality adjusted life year gained. Variation of model parameters resulted in the proton treatments remaining cost-effective for these patients. Based on this cohort, PBT is a cost-effective treatment for patients with BOSCh. This supports the inclusion of PBT for BOSCh in the Medicare Services Advisory Committee 1455 application.

Owing to the special growth pattern of chordomas and the limited treatment options currently available, the treatment of chordoma still remains difficult. In this study, we hope to further clarify the relationship between surgical treatment and radiotherapy of chordoma and disease progression.All patients with a primary histopathological diagnosis of clival or spinal chordomas recorded in our institution between 1976 and 2017 were examined.A total of 60 patients (location: skull base/clival, n = 24; vertebral column, n = 5; sacrum, n = 31) had a mean follow-up time of 7.7 years (range 12 months-35 years). Compared with patients who received subtotal resection (n = 5, 5-year and 10-year survival = 61% and 39%, respectively), the annual survival rate of patients who received total resection (n = 55, 5-year and 10-year survival = 67%, respectively) was significantly higher. The overall 10-year survival rate (58%) of patients treated with surgery alone was significantly different from those treated with a combination of surgery and radiation (73%). The long-term prognosis of sacral chordoma was the worst (10-year survival rate = 48%).The best treatment strategy for improved long-term survival in chordoma was a combination of surgical resection and radiation therapy. Adjuvant radiotherapy for chordoma significantly improves disease-free survival, although the long-term survival benefit remains to be determined. A worse prognosis and poor long-term survival are seen in sacral chordomas.

For patients who require postoperative radiotherapy after endoscopic resection of skull base tumors, proton therapy with pencil beam scanning (PBS) may allow sparing of normal tissue compared to intensity-modulated photon radiation (IMRT). We compared PBS and IMRT radiation plans in the preoperative and postoperative settings for two patients with advanced skull base tumors following endoscopic resection. The benefits of PBS over IMRT appear greater in the postoperative setting following endoscopic resection with improved sparing of critical organs at risk. The multidisciplinary approach of endoscopic resection followed by PBS represents a treatment paradigm with potential for improvements in toxicity reduction. Laryngoscope, 129:1313-1317, 2019., (© 2018 The American Laryngological, Rhinological and Otological Society, Inc.)