Editorial

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Open AccessEditorial

Treatment of Skull Base Diseases: A Multidisciplinary Challenge

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J. Clin. Med. 2023, 12(4), 1492; https://doi.org/10.3390/jcm12041492 - 13 Feb 2023

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Abstract

The skull base has always been regarded as a frontier by surgeons and radiation oncologists since it represents the interface between the intracranial and the extracranial compartment and hosts several critical anatomical structures with an extremely complex and close relationship [...] Full article

(This article belongs to the Special Issue State of the Art—Treatment of Skull Base Diseases)

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Research

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Open AccessArticle

Particle Reirradiation of Malignant Epithelial and Neuroectodermal Sinonasal Tumors: A Case Series from CNAO

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J. Clin. Med. 2023, 12(7), 2624; https://doi.org/10.3390/jcm12072624 - 31 Mar 2023

Viewed by 437

Abstract

Sinonasal cancers (SNCs) are rare and heterogeneous in histology and biological behavior. The prognosis is generally unfavorable, especially in inoperable cases. In recent years, for some histologies, such as undifferentiated sinonasal carcinoma (SNUC), multimodal treatment with a combination of induction chemotherapy, surgery, and [...] Read more.

Sinonasal cancers (SNCs) are rare and heterogeneous in histology and biological behavior. The prognosis is generally unfavorable, especially in inoperable cases. In recent years, for some histologies, such as undifferentiated sinonasal carcinoma (SNUC), multimodal treatment with a combination of induction chemotherapy, surgery, and chemo/radiotherapy (RT) has improved the prognosis. Nevertheless, still about half of the patients treated incur a recurrence, in most of the cases at the local site. Surgery with and without RT is usually the treatment choice in cases of recurrence after previous RT in combination with systemic therapy or RT in a histology-driven fashion. In the case of inoperable disease or contraindications to surgery, RT is still a valid treatment option. In this context, hadron therapy with protons (PT) or carbon ions (CIRT) is often preferred due to the physical and biological characteristics of charged particles, allowing the administration of high doses to the tumor target while sparing the surrounding healthy tissues and potentially limiting the side effects due to the high cumulative dose. In the absence of a standard of care for the recurrent setting, we aimed to investigate the role of re-RT with PT or CIRT. We retrospectively analysed 15 patients with recurrent, previously irradiated, SNCs treated at our institution between 2013 and 2020. Local control (LC) and overall survival (OS) were estimated by the Kaplan–Meier method. Acute and late toxicities were scored according to the National Cancer Institute’s Common Terminology Criteria for Adverse Events CTCAE version 5.0. A total of 13 patients received CIRT and 2 patients received PT. The median re-RT dose was 54 GyRBE (range 45–64 GyRBE) delivered in 3 or 4 GyRBE/fr (fraction) for the CIRT, and 2 Gy RBE/fr for the PT schedule. LC was 44% at the 1-year follow-up and 35.2% at the 3-year follow-up. OS at 1 and 3 years were 92.9% and 38.2%, respectively. Fourteen patients developed G1–G2 acute toxicity (dermatitis and mucositis), and no patients developed G3–G5. Regarding late toxicity, 10 patients encountered at maximum G1–2 events, and 4 did not experience any toxicity. Only for one patient G3 late toxicity was reported (dysphagia requiring a percutaneous endoscopic gastrostomy). Full article

(This article belongs to the Special Issue State of the Art—Treatment of Skull Base Diseases)

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Open AccessArticle

Typical and Atypical Symptoms of Petrous Apex Cholesterol Granuloma: Association with Radiological Findings

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Alessandro Vinciguerra

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Apostolos Karligkiotis

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Paolo Castelnuovo

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Philippe Herman

J. Clin. Med. 2022, 11(15), 4297; https://doi.org/10.3390/jcm11154297 - 24 Jul 2022

Viewed by 1224

Abstract

Objective: Petrous apex cholesterol granuloma (PACG) is a lesion that can give rise to different symptoms, and correlations with etiopathology are ambiguous. The aim of this study is to analyze the association between PACG symptoms and radiological findings at presentation, in order to [...] Read more.

Objective: Petrous apex cholesterol granuloma (PACG) is a lesion that can give rise to different symptoms, and correlations with etiopathology are ambiguous. The aim of this study is to analyze the association between PACG symptoms and radiological findings at presentation, in order to establish a reproduceable pre-operative radiological evaluation and guide the surgical indication. Methods: PACG patients were collected in two tertiary care hospitals. All cases underwent CT/MRI to evaluate the cyst localization and erosion of surrounding structures. Typical and atypical symptoms were then analyzed and compared to radiologic findings established in accordance with the literature. Results: Twenty-nine patients were recruited; the most common symptoms were headache (69%), diplopia (20.7%) and fainting (24.1%), an atypical clinical manifestation related to jugular tubercle involvement. Significant associations between symptoms and radiologic findings were noted in terms of headache and temporal lobe compression (p = 0.04), fainting and jugular tubercle erosion (p < 0.001), vestibular symptoms and internal auditory canal erosion (p = 0.02), facial paresthesia and Meckel’s cave compression (p = 0.03), diplopia and Dorello canal involvement (p = 0.001), and tinnitus and cochlear basal turn erosion (p < 0.001). All patients were treated via an endoscopic–endonasal approach, in which extension was tailored to each case. At a median follow-up of 46 months, 93.1% of patients experienced resolution of symptoms. Conclusions: This clinico-radiological series demonstrates associations between symptoms and anatomical subsites involved with PACG. Hence, it may guide the surgeon at the time of surgical decision, since it asserts that typical and atypical symptoms are actually related to PACG. Full article

(This article belongs to the Special Issue State of the Art—Treatment of Skull Base Diseases)

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Open AccessArticle

Endoscopic-Assisted Keyhole Middle Cranial Fossa Approach for Small Vestibular Schwannomas

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J. Clin. Med. 2022, 11(9), 2324; https://doi.org/10.3390/jcm11092324 - 21 Apr 2022

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Abstract

The classical middle cranial fossa approach (MCFA) for vestibular schwannoma (VS) removal often requires a large incision and craniotomy, excessive temporal lobe manipulation, and a longer recovery. We describe a keyhole MCFA (KMCFA) with endoscopic assistance that allows for adequate access with minimal [...] Read more.

The classical middle cranial fossa approach (MCFA) for vestibular schwannoma (VS) removal often requires a large incision and craniotomy, excessive temporal lobe manipulation, and a longer recovery. We describe a keyhole MCFA (KMCFA) with endoscopic assistance that allows for adequate access with minimal temporal lobe manipulation, resulting in a fast recovery and an invisible scar. Eight sides of four cadaveric heads were dissected through the endoscopic-assisted KMCFA to access the internal auditory canal (IAC). Furthermore, five patients with intracanalicular VS underwent tumor removal with the endoscopic-assisted KMCFA. During the endoscopic-assisted KMCFA with fine instruments, a 3-cm supra-auricular incision and a 2-cm diameter keyhole craniotomy achieved exposure of the entire length of the IAC in all cadaveric dissections without unintended violation of the cochlea, semicircular canal, and facial nerve. The gross tumor was totally removed in five patients with no major postoperative complications. The surgical time was reduced, the hearing outcomes were similar to those of the classical MCFA, and the scar was invisible 1 month after the surgery. The endoscopic-assisted KMCFA permits intracanalicular VS removal in a safe, efficient, and cosmetic way. For small intracanalicular VSs, this approach can replace the classical MCFA when indicated. Full article

(This article belongs to the Special Issue State of the Art—Treatment of Skull Base Diseases)

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Review

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Open AccessReview

Sinonasal Malignancies Involving the Frontal Sinus: A Mono-Institutional Experience of 84 Cases and Systematic Literature Review

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J. Clin. Med. 2023, 12(9), 3186; https://doi.org/10.3390/jcm12093186 - 28 Apr 2023

Viewed by 529

Abstract

Frontal sinus involvement by malignant tumors is a rare finding. Therefore, a systematic literature review along with a personal case series may contribute to defining more accurately the epidemiology, treatment options, and outcomes of these neoplasms. This is a retrospective review of patients [...] Read more.

Frontal sinus involvement by malignant tumors is a rare finding. Therefore, a systematic literature review along with a personal case series may contribute to defining more accurately the epidemiology, treatment options, and outcomes of these neoplasms. This is a retrospective review of patients affected by frontal sinus malignancies surgically treated in a tertiary-care referral center over a period of 20 years. Moreover, a systematic literature review of studies describing frontal sinus cancers from 2000 to date was performed according to PRISMA guidelines in order to analyze current evidence about the treatment and outcomes of such a rare disease. Our retrospective review was basedon 84 cases, treated with an exclusive endoscopic approach in 43 cases (51.2%), endoscopic approach with frontal osteoplastic flap in 6 cases (7.1%), and transfacial or transcranial approaches in 35 cases (41.7%). The five-year overall, disease-specific, disease-free, and recurrence-free survivals were 54.6%, 62.6%, 33.1%, and 59.1%, respectively. Age, dural involvement, type of surgical resection, and surgical margin status were significantly associated with the survival endpoints. In conclusion, the involvement of the frontal sinus is associated with a poor prognosis. Multidisciplinary management, including specific histology-driven treatments, represents the gold standard for improving outcomes and minimizing morbidity. Full article

(This article belongs to the Special Issue State of the Art—Treatment of Skull Base Diseases)

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Open AccessReview

Skull-Base Surgery—A Narrative Review on Current Approaches and Future Developments in Surgical Navigation

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J. Clin. Med. 2023, 12(7), 2706; https://doi.org/10.3390/jcm12072706 - 04 Apr 2023

Viewed by 663

Abstract

Surgical navigation technology combines patient imaging studies with intraoperative real-time data to improve surgical precision and patient outcomes. The navigation workflow can also include preoperative planning, which can reliably simulate the intended resection and reconstruction. The advantage of this approach in skull-base surgery [...] Read more.

Surgical navigation technology combines patient imaging studies with intraoperative real-time data to improve surgical precision and patient outcomes. The navigation workflow can also include preoperative planning, which can reliably simulate the intended resection and reconstruction. The advantage of this approach in skull-base surgery is that it guides access into a complex three-dimensional area and orients tumors intraoperatively with regard to critical structures, such as the orbit, carotid artery and brain. This enhances a surgeon’s capabilities to preserve normal anatomy while resecting tumors with adequate margins. The aim of this narrative review is to outline the state of the art and the future directions of surgical navigation in the skull base, focusing on the advantages and pitfalls of this technique. We will also present our group experience in this field, within the frame of the current research trends. Full article

(This article belongs to the Special Issue State of the Art—Treatment of Skull Base Diseases)

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Open AccessReview

Survival Outcomes in Squamous Cell Carcinoma of the External Auditory Canal: A Systematic Review and Meta-Analysis

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Diego Cazzador

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Leonardo Franz

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Giulia Tealdo

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Andrea Luigi Camillo Carobbio

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J. Clin. Med. 2023, 12(7), 2490; https://doi.org/10.3390/jcm12072490 - 24 Mar 2023

Viewed by 503

Abstract

Squamous cell carcinomas (SCC) of the external auditory canal (EAC) are rare tumors representing a surgical challenge. Current knowledge is based largely on case series; thus, the level of evidence is weak. This study sought to systematically review the available SCC of the [...] Read more.

Squamous cell carcinomas (SCC) of the external auditory canal (EAC) are rare tumors representing a surgical challenge. Current knowledge is based largely on case series; thus, the level of evidence is weak. This study sought to systematically review the available SCC of the EAC literature and to identify risk factors for overall survival (OS) and disease-specific survival (DSS). A systematic review and meta-analysis of papers searched up to December 2022 through PubMed, Scopus, Web of Science, and Cochrane Library databases was conducted. Quality assessment of the eligible studies was done according to the Newcastle-Ottawa Scale. Pooled univariate and multivariable analyses and meta-analysis using a random-effects or fixed-effects Mantel-Haenszel model were performed. Fifteen articles (282 patients) met the inclusion criteria and were included in the quantitative analysis. The pooled multivariable analysis revealed cT3 and cT4 as independent prognostic factors for OS (p = 0.005, and p < 0.001, respectively) and DSS (p = 0.002, and p < 0.001, respectively). Local recurrence rate was 32.3%. The meta-analysis estimated significantly higher odds ratios for advanced T categories, than cT1-T2 tumors for OS and DSS (OR = 3.55; 95% CI, 1.93–6.52, and OR = 3.73; 95% CI, 2.00–6.97, respectively). In conclusion, locally advanced tumors were associated with poor prognosis. Poor outcomes mostly occurred due to local recurrence. Full article

(This article belongs to the Special Issue State of the Art—Treatment of Skull Base Diseases)

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Open AccessReview

Molecular Basis and Rationale for the Use of Targeted Agents and Immunotherapy in Sinonasal Cancers

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Nyall Robert Jr London

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Mario Hermsen

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Matt Lechner

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Paolo Bossi

J. Clin. Med. 2022, 11(22), 6787; https://doi.org/10.3390/jcm11226787 - 16 Nov 2022

Cited by 1 | Viewed by 989

Abstract

Despite the progress of surgery, radiotherapy, and neoadjuvant chemotherapy, the prognosis for advanced sinonasal cancers (SNCs) remains poor. In the era of precision medicine, more research has been conducted on the molecular pathways and recurrent mutations of SNCs, with the aim of understanding [...] Read more.

Despite the progress of surgery, radiotherapy, and neoadjuvant chemotherapy, the prognosis for advanced sinonasal cancers (SNCs) remains poor. In the era of precision medicine, more research has been conducted on the molecular pathways and recurrent mutations of SNCs, with the aim of understanding carcinogenesis, helping with diagnosis, identifying prognostic factors, and finding potentially targetable mutations. In the treatment of SNC, immunotherapy is rarely used, and no targeted therapies have been approved, partly because these tumors are usually excluded from major clinical trials. Data on the efficacy of targeted agents and immune checkpoint inhibitors are scarce. Despite those issues, a tumor-agnostic treatment approach based on targeted drugs against a detected genetic mutation is growing in several settings and cancer subtypes, and could also be proposed for SNCs. Our work aims to provide an overview of the main molecular pathways altered in the different epithelial subtypes of sinonasal and skull base tumors, focusing on the possible actionable mutations for which potential target therapies are already approved in other cancer types. Full article

(This article belongs to the Special Issue State of the Art—Treatment of Skull Base Diseases)

Other

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Open AccessCommentary

Lower-Neck Sparing Using Proton Therapy in Patients with Uninvolved Neck Nasopharyngeal Carcinoma: Is It Safe?

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Francesca De Felice

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Alessandro Vai

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Anna Maria Camarda

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Nicola Alessandro Iacovelli

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Ester Orlandi

J. Clin. Med. 2022, 11(12), 3297; https://doi.org/10.3390/jcm11123297 - 09 Jun 2022

Cited by 2 | Viewed by 898

Abstract

Undifferentiated carcinoma of the nasopharynx (NPC) is a rare disease, which usually occurs in the Asian population. Due to its anatomic location, it is characterised by rich lymph node drainage and has a high incidence of cervical node metastasis. However, cervical nodal metastasis [...] Read more.

Undifferentiated carcinoma of the nasopharynx (NPC) is a rare disease, which usually occurs in the Asian population. Due to its anatomic location, it is characterised by rich lymph node drainage and has a high incidence of cervical node metastasis. However, cervical nodal metastasis commonly involves retropharyngeal nodes and level II nodes, followed by level III nodes. In recent years, innovations in terms of systemic treatments and radiotherapy techniques have improved oncological outcome and treatment-related toxicities. Therefore, there is a growing interest in de-intensification strategies of reducing volumes and treatment-related side effects, especially in patients with NPC with N0–N1-stage disease. Proton therapy could represent a valid alternative to Intensity Modulated Radiotherapy (IMRT) in the management of NPC in this setting. With this Commentary, we aim to explore the feasibility of Intensity Modulated Proton Therapy (IMPT) in upper-neck irradiation of NPC N1-stage disease. We selected an NPC patient with N1 disease and compared the original IMRT plan with the IMPT plan in terms of dosimetric parameters. IMPT offers a minimal dosimetric advantage over IMRT in the bilateral lower-neck sparing. Clinical trials are needed to evaluate the significance of these proposed suggestions and their applicability in non-endemic areas. Full article

(This article belongs to the Special Issue State of the Art—Treatment of Skull Base Diseases)

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