Special Issue "Skull Base Tumors"

A special issue of Cancers (ISSN 2072-6694).

Deadline for manuscript submissions: 31 July 2023 | Viewed by 6112

Special Issue Editor

1. Associate Professor of Rhinology and Skull Base Surgery, Mayo Clinic College of Medicine, Rochester, MN 55905, USA
2. Chair of Quality in the Department of Otolaryngology—Head and Neck Surgery and Department of Neurologic Surgery, Mayo Clinic College of Medicine, Rochester, MN 55905, USA
Interests: endoscopic skull base surgery; sinonasal cancers; skull base tumors

Special Issue Information

Dear Colleagues,

Anterior skull base tumors encompass a range of benign and malignant pathologies that occur along the junction of the cranial base and sinonasal cavity. Optimal management of these tumors is multidisciplinary and must balance oncologic outcomes with patient function and quality of life. In recent years, surgical management has been transformed by the advent of endoscopic endonasal resection. However, open approaches remain a necessary tool in extensive tumors in specific anatomic regions. Additional treatment modalities also play a critical role, including radiation therapy and systemic chemotherapeutics.

This Special Issue will highlight the current state-of-the-art management of skull base tumors, outcomes of treatment, anatomic features of skull base surgery, and advances in the management of sinonasal malignancies.

We look forward to receiving your contributions.

Dr. Garret W. Choby
Guest Editor

Manuscript Submission Information

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Keywords

  • skull base tumors
  • cranial base
  • sinonasal malignancies
  • chordoma
  • pituitary tumor
  • esthesioneuroblastoma
  • quality of life
  • endoscopic skull base surgery
  • skull base reconstruction

Published Papers (8 papers)

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Research

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Article
Proton or Carbon Ion Therapy for Skull Base Chordoma: Rationale and First Analysis of a Mono-Institutional Experience
Cancers 2023, 15(7), 2093; https://doi.org/10.3390/cancers15072093 - 31 Mar 2023
Viewed by 521
Abstract
Background: Skull base chordomas are radio-resistant tumors that require high-dose, high-precision radiotherapy, as can be delivered by particle therapy (protons and carbon ions). We performed a first clinical outcome analysis of particle therapy based on the initial 4-years of operation. Methods: Between August [...] Read more.
Background: Skull base chordomas are radio-resistant tumors that require high-dose, high-precision radiotherapy, as can be delivered by particle therapy (protons and carbon ions). We performed a first clinical outcome analysis of particle therapy based on the initial 4-years of operation. Methods: Between August 2017 and October 2021, 44 patients were treated with proton (89%) or carbon ion therapy (11%). Prior gross total resection had been performed in 21% of lesions, subtotal resection in 57%, biopsy in 12% and decompression in 10%. The average prescription dose was 75.2 Gy RBE in 37 fractions for protons and 66 Gy RBE in 22 fractions for carbon ions. Results: At a median follow-up of 34.3 months (range: 1–55), 2-, and 3-year actuarial local control rates were 95.5% and 90.9%, respectively. The 2-, and 3-year overall and progression-free survival rates were 97.7%, 93.2%, 95.5% and 90.9%, respectively. The tumor volume at the time of particle therapy was highly predictive of local failure (p < 0.01), and currently, there is 100% local control in patients with tumors < 49 cc. No grade ≥3 toxicities were observed. There was no significant difference in outcome or side effect profile seen for proton versus carbon ion therapy. Five patients (11.4%) experienced transient grade ≤2 radiation-induced brain changes. Conclusions: The first analysis suggests the safety and efficacy of proton and carbon ion therapy at our center. The excellent control of small to mid-size chordomas underlines the effectiveness of particle therapy and importance of upfront maximum debulking of large lesions. Full article
(This article belongs to the Special Issue Skull Base Tumors)
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Article
Recurrent Esthesioneuroblastoma: Long-Term Outcomes of Salvage Therapy
Cancers 2023, 15(5), 1506; https://doi.org/10.3390/cancers15051506 - 28 Feb 2023
Viewed by 668
Abstract
Introduction: Esthesioneuroblastoma (ENB) is a rare malignant neoplasm arising from the olfactory epithelium of the cribriform plate. Although survival is excellent with a reported 5-year overall survival (OS) of 82%, recurrence is frequent and occurs in 40–50% of cases. This study investigates the [...] Read more.
Introduction: Esthesioneuroblastoma (ENB) is a rare malignant neoplasm arising from the olfactory epithelium of the cribriform plate. Although survival is excellent with a reported 5-year overall survival (OS) of 82%, recurrence is frequent and occurs in 40–50% of cases. This study investigates the characteristics of ENB recurrence and the subsequent prognosis of patients with recurrence. Methods: The clinical records of all patients diagnosed as having ENB with subsequent recurrence at a tertiary hospital from 1 January 1960 to 1 January 2020 were retrospectively reviewed. Overall survival (OS) and progression-free survival (PFS) were reported. Results: A total of 64 out of 143 ENB patients had recurrences. In total, 45 out of 64 recurrences met the inclusion criteria and were included in this study. From these, 10 (22%) had a sinonasal recurrence, 14 (31%) had an intracranial recurrence, 15 (33%) had a regional recurrence, and 6 (13%) had a distal recurrence. The average interval from initial treatment to recurrence was 4.74 years. There were no differences in rates of recurrence with respect to age, sex, or types of surgery (endoscopic, transcranial, lateral rhinotomy, and combined). The time to recurrence was shorter for Hyams grades 3 and 4 compared to Hyams grades 1 and 2 (3.75 years vs. 5.70 years, p < 0.05). Patients with recurrence limited to the sinonasal region had a lower overall primary Kadish stage compared to recurrences beyond the sinonasal region (2.60 vs. 3.03, p < 0.05). A total of 9 (20%) out of 45 patients developed secondary recurrence. Following recurrence, the subsequent 5-year OS and PFS were 63 and 56%, respectively. The mean time to secondary recurrence after treatment of the primary recurrence was 32 months, which was significantly shorter than the time to primary recurrence (32 months vs. 57 months, p = 0.048). The mean age of the secondary recurrence group is significantly older than the primary recurrence group (59.78 years vs. 50.31 years, p = 0.02). No statistically significant differences were observed between the secondary recurrence group and the recurrence group in terms of their overall Kadish stages or Hyams grades. Conclusions: Following an ENB recurrence, salvage therapy appears to be an effective therapeutic option with a subsequent 5-year OS of 63%. However, subsequent recurrences are not infrequent and may require additional therapy. Full article
(This article belongs to the Special Issue Skull Base Tumors)
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Article
Pseudoprogression of Vestibular Schwannoma after Stereotactic Radiosurgery with Cyberknife®: Proposal for New Response Criteria
Cancers 2023, 15(5), 1496; https://doi.org/10.3390/cancers15051496 - 27 Feb 2023
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Abstract
(1) Background: Transient increase in volume of vestibular schwannomas (VS) after stereotactic radiosurgery (SRS) is common and complicates differentiation between treatment-related changes (pseudoprogression, PP) and tumor recurrence (progressive disease, PD). (2) Methods: Patients with unilateral VS (n = 63) underwent single fraction robotic-guided [...] Read more.
(1) Background: Transient increase in volume of vestibular schwannomas (VS) after stereotactic radiosurgery (SRS) is common and complicates differentiation between treatment-related changes (pseudoprogression, PP) and tumor recurrence (progressive disease, PD). (2) Methods: Patients with unilateral VS (n = 63) underwent single fraction robotic-guided SRS. Volume changes were classified according to existing RANO criteria. A new response type, PP, with a >20% transient increase in volume was defined and divided into early (within the first 12 months) and late (>12 months) occurrence. (3) Results: The median age was 56 (range: 20–82) years, the median initial tumor volume was 1.5 (range: 0.1–8.6) cm3. The median radiological and clinical follow-up time was 66 (range: 24–103) months. Partial response was observed in 36% (n = 23), stable disease in 35% (n = 22) and PP in 29% (n = 18) of patients. The latter occurred early (16%, n = 10) or late (13%, n = 8). Using these criteria, no case of PD was observed. (4) Conclusion: Any volume increase after SRS for vs. assumed to be PD turned out to be early or late PP. Therefore, we propose modifying RANO criteria for SRS of VS, which may affect the management of vs. during follow-up in favor of further observation. Full article
(This article belongs to the Special Issue Skull Base Tumors)
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Article
Refusal of Surgery in Pituitary Adenoma Patients: A Population-Based Analysis
Cancers 2022, 14(21), 5348; https://doi.org/10.3390/cancers14215348 - 30 Oct 2022
Viewed by 698
Abstract
We characterized the clinical and sociodemographic factors predictive of surgery refusal in pituitary adenoma (PA) patients. We queried the National Cancer Database (NCDB) to identify adult PA patients treated from 2004–2015 receiving or refusing surgery. Multivariate logistic regression and Cox proportional-hazards analysis identified [...] Read more.
We characterized the clinical and sociodemographic factors predictive of surgery refusal in pituitary adenoma (PA) patients. We queried the National Cancer Database (NCDB) to identify adult PA patients treated from 2004–2015 receiving or refusing surgery. Multivariate logistic regression and Cox proportional-hazards analysis identified clinical and/or sociodemographic factors predictive of surgery refusal or mortality, respectively. Of the 34,226 patients identified, 280 (0.8%) refused surgery. On multivariate logistic regression, age > 65 (OR: 2.64; p < 0.001), African American race (OR: 1.70; p < 0.001), Charlson-Deyo Comorbidity (C/D) Index > 2 (OR: 1.52; p = 0.047), and government insurance (OR: 2.03; p < 0.001) or being uninsured (OR: 2.16; p = 0.03) were all significantly associated with surgery refusal. On multivariate cox-proportional hazard analysis, age > 65 (HR: 2.66; p < 0.001), tumor size > 2 cm (HR: 1.30; p < 0.001), C/D index > 1 (HR: 1.53; p < 0.001), having government insurance (HR: 1.66; p < 0.001) or being uninsured (HR: 1.67; p < 0.001), and surgery refusal (HR: 2.28; p < 0.001) were all significant predictors of increased mortality. Macroadenoma patients receiving surgery had a significant increase in overall survival (OS) compared to those who refused surgery (p < 0.001). There are significant sociodemographic factors that influence surgery refusal in PA patients. An individualized approach is warranted that considers functional status, clinical presentations, and patient choice. Full article
(This article belongs to the Special Issue Skull Base Tumors)
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Article
Mayo Clinic Clival Chordoma Case Series: Impact of Endoscopic Training on Clinical Care
Cancers 2022, 14(20), 5104; https://doi.org/10.3390/cancers14205104 - 18 Oct 2022
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Abstract
The management of clival chordoma in our group shifted around 2013 to mostly endoscopic, and proton beam was introduced for our multidisciplinary team. Consecutive patients who had surgical resection from 1987 to 2021 were reviewed. A total of 58 patients (39 patients after [...] Read more.
The management of clival chordoma in our group shifted around 2013 to mostly endoscopic, and proton beam was introduced for our multidisciplinary team. Consecutive patients who had surgical resection from 1987 to 2021 were reviewed. A total of 58 patients (39 patients after 2013) were analyzed. The mean tumor size was 3.7 cm, and the most common location was the upper clivus (43%). Compared to before 2013, after 2013, the endoscopic endonasal approach was more common (90%, p < 0.001), and more gross or near total resections (64%, p = 0.002) were attained. Ten cases (17%) were revision surgeries referred from elsewhere, and three cases (5%) underwent additional surgery elsewhere before adjuvant radiation. The postoperative cerebrospinal fluid leak occurred in 7%. Post-operative new cranial nerve deficits occurred in 32% before 2013, compared to 2.6% after 2013 (p = 0.004). For cases before 2013, 10 patients (53%) recurred during the median follow-up of 144 months (mean, 142 months), whereas for cases after 2013, seven patients (18%) recurred with a median follow-up of 35 months (mean, 42 months). 5-year progression-free survival was 58%, and 5-year overall survival was 87%. A specialized multidisciplinary team improved the resection rate compared to a historical cohort with an excellent morbidity profile. Full article
(This article belongs to the Special Issue Skull Base Tumors)
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Review

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Review
Surgical Anatomy and Approaches of the Anterior Cranial Fossa from a Transcranial and Endonasal Perspective
Cancers 2023, 15(9), 2587; https://doi.org/10.3390/cancers15092587 - 01 May 2023
Viewed by 537
Abstract
The anterior cranial fossa (ACF) is a complex anatomical region that can be affected by a broad spectrum of pathology. For the surgical treatment of these lesions, many approaches have been described, each of them with different scope and potential surgical complications, often [...] Read more.
The anterior cranial fossa (ACF) is a complex anatomical region that can be affected by a broad spectrum of pathology. For the surgical treatment of these lesions, many approaches have been described, each of them with different scope and potential surgical complications, often associated with significant morbidity. Traditionally, tumors involving the ACF have been operated by transcranial approaches; however, in the last two decades, endoscopic endonasal approaches (EEAs) have been gaining popularity. In this work, the authors review and describe the anatomical aspects of the ACF and the technical nuances of transcranial and endoscopic approaches for tumors located in this region. Four approaches were performed in embalmed cadaveric specimens and the key steps were documented. Four illustrative cases of ACF tumors were selected to demonstrate the clinical application of anatomical and technical knowledge, which are essential in the preoperative decision-making process. Full article
(This article belongs to the Special Issue Skull Base Tumors)
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Review
Quality of Life Considerations in Endoscopic Endonasal Management of Anterior Cranial Base Tumors
Cancers 2023, 15(1), 195; https://doi.org/10.3390/cancers15010195 - 28 Dec 2022
Cited by 1 | Viewed by 612
Abstract
Considering quality of life (QOL) is critical when discussing treatment options for patients undergoing endoscopic endonasal skull base surgery (EESBS) for cancers at the base of the skull. Several questionnaires have been developed and validated in the last 20 years to explore QOL [...] Read more.
Considering quality of life (QOL) is critical when discussing treatment options for patients undergoing endoscopic endonasal skull base surgery (EESBS) for cancers at the base of the skull. Several questionnaires have been developed and validated in the last 20 years to explore QOL in this patient population, including the Anterior Skull Base Questionnaire, Skull Base Inventory, EESBS Questionnaire, and the Sino-Nasal Outcome Test for Neurosurgery. The Sino-Nasal Outcomes Test-22 and Anterior Skull Base Nasal Inventory-12 are other tools that have been used to measure sinonasal QOL in anterior cranial base surgery. In addition to pathology-related perturbations in QOL endoscopic surgical options (transsellar approaches, anterior cranial base surgery, and various reconstructive techniques) all have unique morbidities and QOL implications that should be considered. Finally, we look ahead to new and emerging techniques and tools aimed to help preserve and improve QOL for patients with anterior cranial base malignancies. Full article
(This article belongs to the Special Issue Skull Base Tumors)
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Other

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Systematic Review
Tumors Involving the Infratemporal Fossa: A Systematic Review of Clinical Characteristics and Treatment Outcomes
Cancers 2022, 14(21), 5420; https://doi.org/10.3390/cancers14215420 - 03 Nov 2022
Cited by 2 | Viewed by 1035
Abstract
Background: Infratemporal fossa (ITF) tumors represent various pathologies and are seldom described in the literature, reflecting their rarity. Here we review the literature on tumors invading ITF and describe patient characteristics, treatment strategies, and clinical outcomes. Methods: Relevant articles were retrieved from PubMed, [...] Read more.
Background: Infratemporal fossa (ITF) tumors represent various pathologies and are seldom described in the literature, reflecting their rarity. Here we review the literature on tumors invading ITF and describe patient characteristics, treatment strategies, and clinical outcomes. Methods: Relevant articles were retrieved from PubMed, Scopus, and Cochrane. A systematic review and meta-analysis were conducted on the clinical presentation, treatment protocols, and clinical outcomes. Result: A total of 27 articles containing 106 patients with ITF tumors (median tumor size: 24.3 cm3 [interquartile range, 15.2–42 cm3]) were included (median age: 46 years [interquartile range, 32–55 years]; 59.4% were males]). Of the confirmed tumor pathology data, schwannomas (n = 24; 26.1%) and meningiomas (n = 13; 14.1%) were the most common tumors. Facial hypoesthesia (n = 22; 18.5%), auricular/preauricular pain (n = 20; 16.8%), and headaches (n = 11; 9.2%) were the most common presenting symptoms. Of patients who had surgical resection (n = 97; 95.1%), 70 (73.7%) had transcranial surgery (TCS) and 25 (26.3%) had endoscopic endonasal surgery (EES). Among available details on the extent of resection (n = 84), gross-total resection (GTR) was achieved in 62 (73.8%), and 5 (6.0%) had biopsy only. Thirty-five (33.0%) patients had postoperative complications. Among cases with available data on reconstruction techniques (n = 8), four (50%) had adipofascial antero-lateral thigh flap, three (37.5%) had latissimus dorsi free flap, and one (12.5%) had antero-lateral thigh flap. Fourteen (13.2%) patients had adjuvant chemotherapy, and sixteen (15.1%) had adjuvant radiotherapy. During a median follow-up time of 28 months (IQR, 12.25–45.75 months), 15 (14.2%) patients had recurrences, and 18 (17.0%) patients died. The median overall survival (OS) time was 36 months (95% confidence interval: 29–41 months), and the 5-year progression-free survival (PFS) rate was 61%. Conclusion: Various tumor types with different biological characteristics invade the ITF. The present study describes patient demographics, clinical presentation, management, and outcomes. Depending on the tumor type and patient condition, patient-tailored management is recommended to optimize treatment outcomes. Full article
(This article belongs to the Special Issue Skull Base Tumors)
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