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State of the Art—Treatment of Skull Base Diseases (Second Edition)
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State of the Art—Treatment of Skull Base Diseases (Second Edition)

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Clinical Neurology".

Deadline for manuscript submissions: 30 November 2024 | Viewed by 7646

Special Issue Editor

Dr. Marco Ferrari

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Guest Editor
Section of Otorhinolaryngology—Head and Neck Surgery, Department of Neurosciences, University of Padua, “Azienda Ospedale Università di Padova”, Padua, Italy
Interests: head and neck oncology; head and neck surgery; skull base diseases; skull base surgery
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Skull-based diseases, with their intricate challenges in treatment, demand a nuanced approach that balances therapeutic efficacy with patient well-being. This Special Issue delves into the dynamic landscape of therapies for these complex conditions with a gentle touch, acknowledging the delicate nature of interventions in this critical anatomical region. By synthesizing insights from diverse disciplines including neurosurgery, otolaryngology, and radiation oncology, the issue navigates through a rich tapestry of innovative surgical techniques, state-of-the-art imaging modalities, and targeted adjuvant therapies tailored to specific pathologies. From the exploration of minimally invasive endoscopic approaches to the intricacies of skull base reconstruction, the issue spans a comprehensive spectrum of interventions aimed at optimizing patient outcomes while mitigating morbidity.

Moreover, it is with great pride that we announce the successful publication of the first edition, which has seen the dissemination of nearly 15 outstanding manuscripts. These contributions, each representing the pinnacle of scholarly achievement in the field, have solidified the first edition's position as a cornerstone in the literature on skull base diseases. Their depth of insight, methodological rigour, and clinical relevance has not only advanced our understanding of these complex conditions but have also provided tangible benefits to clinicians and patients alike. As we embark on the second edition, we carry forward the momentum and excellence established by the first edition, with the collective aim of further enriching the discourse surrounding the treatment of skull-based diseases.

Moreover, it underscores the pivotal role of multidisciplinary collaboration in the holistic management of skull base diseases, underscoring the significance of teamwork in achieving therapeutic success. Through a synthesis of cutting-edge research and clinical expertise, this Special Issue emerges as a beacon, illuminating the path forward for clinicians and researchers alike. Serving as a comprehensive guide, it offers invaluable insights into the current state-of-the-art treatments while charting promising directions for future advancements. The first edition of this Special Issue has left an indelible mark, setting a precedent for success, and the second edition endeavours to carry forth this legacy of excellence, building upon its foundation to further refine treatment strategies and elevate the standard of care for patients with skull base pathologies.

Link to the first edition: https://www.mdpi.com/journal/jcm/special_issues/Treatment_of_Skull_Base_Diseases

Dr. Marco Ferrari
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • skull base
  • cranial base
  • tumor
  • cancer
  • lesion
  • surgery
  • adjuvant
  • neoadjuvant

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Related Special Issue

Published Papers (6 papers)

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Research

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16 pages, 8058 KiB  
Article
3D Printing for Customized Bone Reconstruction in Spheno-Orbital Meningiomas: A Systematic Literature Review and Institutional Experience
by Simona Serioli, Alberto Pietrantoni, Alberto Benato, Marco Galeazzi, Amedeo Piazza, Liverana Lauretti, Pier Paolo Mattogno, Alessandro Olivi, Marco Maria Fontanella and Francesco Doglietto
J. Clin. Med. 2024, 13(13), 3968; https://doi.org/10.3390/jcm13133968 - 6 Jul 2024
Viewed by 1180
Abstract
Background: The treatment of spheno-orbital meningiomas (SOMs) requires extensive bone resections, creating significant defects in a complex geometrical space. Bone reconstruction represents a fundamental step that optimizes long-term aesthetic and functional outcomes. In recent years, 3D printing technology has also been exploited [...] Read more.
Background: The treatment of spheno-orbital meningiomas (SOMs) requires extensive bone resections, creating significant defects in a complex geometrical space. Bone reconstruction represents a fundamental step that optimizes long-term aesthetic and functional outcomes. In recent years, 3D printing technology has also been exploited for complex skull base reconstructions, but reports remain scarce. Methods: We retrospectively analyzed four consecutive patients who underwent SOM resection and one-step 3D PEEK customized reconstruction from 2019 to 2023. A systematic review of 3D printing customized implants for SOM was then performed. Results: All patients underwent a frontotemporal craniotomy, removal of SOM, and reconstruction of the superolateral orbital wall and pterional region. The aesthetic outcome was extremely satisfactory in all cases. No orbital implant malposition or infectious complications were documented. Eleven papers were included in the literature review, describing 27 patients. Most (23) patients underwent a single-stage reconstruction; in three cases, the implant was positioned to correct postoperative delayed enophthalmos. Porous titanium was the most used material (16 patients), while PEEK was used in three cases. Prosthesis malposition was described in two (7.4%) patients. Conclusions: Single-step reconstruction with a personalized 3D PEEK prosthesis represents a valid reconstruction technique for the treatment of SOMs with good aesthetic outcomes. Full article
(This article belongs to the Special Issue State of the Art—Treatment of Skull Base Diseases (Second Edition))
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12 pages, 7888 KiB  
Article
Combined Endoscopic Endonasal Transclival and Contralateral Transmaxillary Approach to the Petrous Apex and the Petroclival Synchondrosis: Working “Around the Corner” of the Internal Carotid Artery—Quantitative Anatomical Study and Clinical Applications
by Carmine Antonio Donofrio, Francesco Corrivetti, Lucia Riccio, Sergio Corvino, Iacopo Dallan, Antonio Fioravanti and Matteo de Notaris
J. Clin. Med. 2024, 13(9), 2713; https://doi.org/10.3390/jcm13092713 - 5 May 2024
Viewed by 1254
Abstract
The endoscopic contralateral transmaxillary (CTM) approach has been proposed as a potential route to widen the corridor posterolateral to the internal carotid artery (ICA). In this study, we first refined the surgical technique of a combined multiportal endoscopic endonasal transclival (EETC) and CTM [...] Read more.
The endoscopic contralateral transmaxillary (CTM) approach has been proposed as a potential route to widen the corridor posterolateral to the internal carotid artery (ICA). In this study, we first refined the surgical technique of a combined multiportal endoscopic endonasal transclival (EETC) and CTM approach to the petrous apex (PA) and petroclival synchondrosis (PCS) in the dissection laboratory, and then validated its applications in a preliminary surgical series. The combined EETC and CTM approach was performed on three cadaver specimens based on four surgical steps: (1) the nasal, (2) the clival, (3) the maxillary and (4) the petrosal phases. The CTM provided a “head-on trajectory” to the PA and PCS and a short distance to the surgical field considerably furthering surgical maneuverability. The best operative set-up was achieved by introducing angled optics via the endonasal route and operative instruments via the transmaxillary corridor exploiting the advantages of a non-coaxial multiportal surgery. Clinical applications of the combined EETC and CTM approach were reported in three cases, a clival chordoma and two giant pituitary adenomas. The present translational study explores the safety and feasibility of a combined multiportal EETC and CTM approach to access the petroclival region though different corridors. Full article
(This article belongs to the Special Issue State of the Art—Treatment of Skull Base Diseases (Second Edition))
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16 pages, 5856 KiB  
Article
Radiological Classification and Management Algorithm of Petrous Apex Cholesterol Granuloma
by Daniele Marchioni, Chiara Alberti, Nicola Bisi and Alessia Rubini
J. Clin. Med. 2024, 13(9), 2505; https://doi.org/10.3390/jcm13092505 - 24 Apr 2024
Viewed by 1030
Abstract
Background: Petrous apex cholesterol granulomas (PACGs) are benign inflammatory cystic lesions of the temporal bone. Usually, asymptomatic patients may develop symptoms as the lesions expand. The diagnosis is based on both CT and MRI scans and the management relies on “wait and [...] Read more.
Background: Petrous apex cholesterol granulomas (PACGs) are benign inflammatory cystic lesions of the temporal bone. Usually, asymptomatic patients may develop symptoms as the lesions expand. The diagnosis is based on both CT and MRI scans and the management relies on “wait and scan” or surgery. This paper aims at evaluating surgical outcomes and proposing a CT-based classification and a management algorithm. Methods: Patients with PACGs who were surgically treated between 2014 and 2024 were included. Symptoms, imaging, approach type and complications were considered. CT scans were classified as Type A (preserved apex cellularity), Type B (erosion of the apex cellularity), and Type C (involvement of the noble structures bone boundaries). The possible connection of the lesion with the infracochlear, perilabyrinthine and sphenoidal cellularity was assessed. Results: Nineteen patients with symptoms like diplopia, headache and sensorineural hearing loss were included. According to our classification, 1/19 patients was Type A, 4/19 were Type B and 14/19 were Type C. Five patients underwent a total resection, seven a subtotal and seven a surgical drainage. Only two complications were recorded, and 17/19 patients showed symptom regression and stability during follow-up. Conclusions: While the management of PACGs is still controversial, according to our classification and surgical outcomes, Type A, being mostly asymptomatic, should be managed with “wait and scan”, Type B should undergo surgery when symptoms are present, while Type C should always undergo surgery because of their invasiveness and potential complications. When possible, a drainage should be attempted; otherwise, a surgical resection is chosen, and its completeness depends on the preoperative general and hearing status. Full article
(This article belongs to the Special Issue State of the Art—Treatment of Skull Base Diseases (Second Edition))
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Review

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18 pages, 1969 KiB  
Review
The Transorbital Approach: A Comprehensive Review of Targets, Surgical Techniques, and Multiportal Variants
by Matteo De Simone, Cesare Zoia, Anis Choucha, Doo-Sik Kong and Lucio De Maria
J. Clin. Med. 2024, 13(9), 2712; https://doi.org/10.3390/jcm13092712 - 5 May 2024
Cited by 12 | Viewed by 1758
Abstract
The transorbital approach (TOA) is gaining popularity in skull base surgery scenarios. This approach represents a valuable surgical corridor to access various compartments and safely address several intracranial pathologies, both intradurally and extradurally, including tumors of the olfactory groove in the anterior cranial [...] Read more.
The transorbital approach (TOA) is gaining popularity in skull base surgery scenarios. This approach represents a valuable surgical corridor to access various compartments and safely address several intracranial pathologies, both intradurally and extradurally, including tumors of the olfactory groove in the anterior cranial fossa (ACF), cavernous sinus in the middle cranial fossa (MCF), and the cerebellopontine angle in the posterior cranial fossa (PCF). The TOA exists in many variants, both from the point of view of invasiveness and from that of the entry point to the orbit, corresponding to the four orbital quadrants: the superior eyelid crease (SLC), the precaruncular (PC), the lateral retrocanthal (LRC), and the preseptal lower eyelid (PS). Moreover, multiportal variants, consisting of the combination of the transorbital approach with others, exist and are relevant to reach peculiar surgical territories. The significance of the TOA in neurosurgery, coupled with the dearth of thorough studies assessing its various applications and adaptations, underscores the necessity for this research. This extensive review delineates the multitude of target lesions reachable through the transorbital route, categorizing them based on surgical complexity. Furthermore, it provides an overview of the different transorbital variations, both standalone and in conjunction with other techniques. By offering a comprehensive understanding, this study aims to enhance awareness and knowledge regarding the current utility of the transorbital approach in neurosurgery. Additionally, it aims to steer future investigations toward deeper exploration, refinement, and exploration of additional perspectives concerning this surgical method. Full article
(This article belongs to the Special Issue State of the Art—Treatment of Skull Base Diseases (Second Edition))
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Other

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14 pages, 2133 KiB  
Systematic Review
Temporoparietal Fascia Flap (TPFF) in Extended Endoscopic Transnasal Skull Base Surgery: Clinical Experience and Systematic Literature Review
by Martina Offi, Pier Paolo Mattogno, Ginevra Federica D’Onofrio, Simona Serioli, Federico Valeri, Giuseppe Maria Della Pepa, Vincenzo Arena, Claudio Parrilla, Sabrina Chiloiro, Francesco D’Argento, Marco Gessi, Alessandro Pedicelli, Liverana Lauretti, Gaetano Paludetti, Jacopo Galli, Alessandro Olivi, Mario Rigante and Francesco Doglietto
J. Clin. Med. 2024, 13(23), 7217; https://doi.org/10.3390/jcm13237217 - 27 Nov 2024
Abstract
Background and Objectives: The temporoparietal fascia flap (TPFF) has recently emerged as an option for skull base reconstruction in endoscopic transnasal surgery when vascularized nasal flaps are not available. This study provides a systematic literature review of its use in skull base surgery [...] Read more.
Background and Objectives: The temporoparietal fascia flap (TPFF) has recently emerged as an option for skull base reconstruction in endoscopic transnasal surgery when vascularized nasal flaps are not available. This study provides a systematic literature review of its use in skull base surgery and describes a novel cohort of patients. Methods: PRISMA guidelines were used for the review. Patients undergoing skull base reconstruction with TPFF in our center from May 2022 to April 2024 were retrospectively included. Data were collected on pre- and post-operative clinical and radiological features, histology, surgical procedures, and complications. Results: Sixteen articles were selected, comprising 42 patients who underwent TPFF reconstruction for treatment of complex skull base pathologies. In total, 5 of 358 patients (0.9%) who underwent tumor resection via endoscopic transanal surgery in the study period in our institution required TPFF. All had been previously treated with surgery and radiation therapy for different pathologies (three chordomas, one giant pituitary neuroendocrine tumor (PitNET), and one sarcoma). Post-operative complications included CSF leak, which resolved after flap revision, and an internal carotid artery pseudoaneurysm requiring endovascular embolization. Conclusions: TPFF is an effective option for skull base reconstruction in complex cases and should be part of the armamentarium of the skull base surgeon. Full article
(This article belongs to the Special Issue State of the Art—Treatment of Skull Base Diseases (Second Edition))
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18 pages, 2255 KiB  
Systematic Review
Chordoma Genetic Aberrations and Targeted Therapies Panorama: A Systematic Literature Review
by Edoardo Agosti, Sara Antonietti, Marco Zeppieri, Tamara Ius, Alessandro Fiorindi, Alessandro Tel, Massimo Robiony, Pier Paolo Panciani and Marco Maria Fontanella
J. Clin. Med. 2024, 13(9), 2711; https://doi.org/10.3390/jcm13092711 - 5 May 2024
Viewed by 1794
Abstract
Background: Chordomas pose a challenge in treatment due to their local invasiveness, high recurrence, and potential lethality. Despite being slow-growing and rarely metastasizing, these tumors often resist conventional chemotherapies (CTs) and radiotherapies (RTs), making surgical resection a crucial intervention. However, achieving radical [...] Read more.
Background: Chordomas pose a challenge in treatment due to their local invasiveness, high recurrence, and potential lethality. Despite being slow-growing and rarely metastasizing, these tumors often resist conventional chemotherapies (CTs) and radiotherapies (RTs), making surgical resection a crucial intervention. However, achieving radical resection for chordomas is seldom possible, presenting therapeutic challenges. The accurate diagnosis of these tumors is vital for their distinct prognoses, yet differentiation is hindered by overlapping radiological and histopathological features. Fortunately, recent molecular and genetic studies, including extracranial location analysis, offer valuable insights for precise diagnosis. This literature review delves into the genetic aberrations and molecular biology of chordomas, aiming to provide an overview of more successful therapeutic strategies. Methods: A systematic search was conducted across major medical databases (PubMed, Embase, and Cochrane Library) up to 28 January 2023. The search strategy utilized relevant Medical Subject Heading (MeSH) terms and keywords related to “chordomas”, “molecular biology”, “gene aberrations”, and “target therapies”. The studies included in this review consist of preclinical cell studies, case reports, case series, randomized controlled trials, non-randomized controlled trials, and cohort studies reporting on genetic and biological aberrations in chordomas. Results: Of the initial 297 articles identified, 40 articles were included in the article. Two tables highlighted clinical studies and ongoing clinical trials, encompassing 18 and 22 studies, respectively. The clinical studies involved 185 patients diagnosed with chordomas. The tumor sites were predominantly sacral (n = 8, 44.4%), followed by clivus (n = 7, 38.9%) and lumbar spine (n = 3, 16.7%). Primary treatments preceding targeted therapies included surgery (n = 10, 55.6%), RT (n = 9, 50.0%), and systemic treatments (n = 7, 38.9%). Various agents targeting specific molecular pathways were analyzed in the studies, such as imatinib (a tyrosine kinase inhibitor), erlotinib, and bevacizumab, which target EGFR/VEGFR. Common adverse events included fatigue (47.1%), skin reactions (32.4%), hypertension (23.5%), diarrhea (17.6%), and thyroid abnormalities (5.9%). Clinical outcomes were systematically assessed based on progression-free survival (PFS), overall survival (OS), and tumor response evaluated using RECIST or CHOI criteria. Notably, stable disease (SD) occurred in 58.1% of cases, and partial responses (PRs) were observed in 28.2% of patients, while 13.7% experienced disease progression (PD) despite targeted therapy. Among the 22 clinical trials included in the analysis, Phase II trials were the most prevalent (40.9%), followed by I-II trials (31.8%) and Phase I trials (27.3%). PD-1 inhibitors were the most frequently utilized, appearing in 50% of the trials, followed by PD-L1 inhibitors (36.4%), CTLA-4 inhibitors (22.7%), and mTOR inhibitors (13.6%). Conclusions: This systematic review provides an extensive overview of the state of targeted therapy for chordomas, highlighting their potential to stabilize the illness and enhance clinical outcomes. Full article
(This article belongs to the Special Issue State of the Art—Treatment of Skull Base Diseases (Second Edition))
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