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Background: Biphenotypic sinonasal sarcoma is a rare low-grade tumor arising from the sinonasal tract, featuring locally aggressive biological behavior, with a tendency to invade the orbit and skull base. There are no defined guidelines of treatment; thus, the management varies among different institutions. The aim of the present study is to provide a modular system of surgical approaches according to the lesion pattern of growth from a literature review. Materials and Methods: A comprehensive and detailed literature review on the PubMed and Embase online electronic databases on biphenotypic sinonasal sarcoma with orbital invasion was conducted. A personal case exhibiting peculiar features was also added. Demographic (patient's sex and age), clinical (presenting symptoms and time to treatment), neuroradiological (anatomical origin and pattern of growth), and treatment (type of treatment, surgical approach, extent of resection, peri- and postoperative complications, and adjuvant therapies) data, as well as clinical outcome, recurrence rates, and overall survival, were analyzed. Results: Thirty-one patients harboring biphenotypic sinonasal sarcoma with orbital invasion were identified. Tumors mainly affected female patients (66.7%) and a middle-aged population (median 55.2 years old). Simultaneous skull base involvement occurred in most cases (80.6%). Surgery was performed in all but one case (97%), as unique treatment (59%) or in association with radio-(23.5%) and/or chemotherapy (5.9%/2.9%), allowing for gross total tumor resection in most cases (66.7%). The endoscopic endonasal approach was the most adopted surgical corridor (51.7%). The local recurrence rate was 19.3%, and only two cases of tumor-related mortality occurred. Conclusions: Surgery is the only curative treatment, with the main goal to restore/improve/arrest progression of clinical manifestations. The endoscopic endonasal route represents the master approach for lesions confined to the midline. Microsurgical transcranial and endoscopic transorbital approaches have a complementary role for addressing the lesion's component with large intracranial extension or affecting the paramedian aspect of the anterior cranial fossa and superior-lateral orbital compartment, respectively. The approach selection should be made case by case according to the tumor pattern of growth.

Ollier disease (OD), acute myeloid leukemia (AML), and brain glioma (BG) are three apparently completely different neoplasms in terms of histopathology, clinic, natural history, and management, but they can affect the same patient. This study aimed to identify the common molecular pathways involved in the pathogenesis of all three diseases and discuss their current and potential role as therapeutic targets. A detailed and comprehensive systematic literature review according to PRISMA guidelines on OD patients harboring BG and/or AML was made. In addition, the unique case of a patient affected by all three considered diseases has been added to our case series. Demographic, pathological, treatment, and outcome data were analyzed and discussed, mainly focusing on the molecular findings. Twenty-eight studies reported thirty-three patients affected by OD and BG, and only one study reported one patient with OD and AML, while only our patient harbored all three pathologies. The IDH R132H mutation was the only genetic alteration shared by all three pathologies and was simultaneously detected in enchondromas and brain glioma in 100% (3/3) of OD patients with BG and also in the neoplastic blood cells of the single patient hosting all three diseases. The IDH1-R132H gene mutation is the etiopathogenetic common denominator among three apparently different tumors coexisting in the same patient. The adoption of mutant-specific IDH1 inhibitor molecules could represent a potential panacea for these conditions in the era of targeted therapies. Further studies with larger clinical series are needed to confirm our results and hypothesis.

Competing Interests: The author declares that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Background: While several risk factors for recurrences have been defined, the topographic pattern of meningioma recurrences after surgical resection has been scarcely investigated. The possibility of theoretically predicting the site of recurrence not only allows us to better understand the pathogenetic bases of the disease and consequently to drive the development of new targeted therapies, but also guides the decision-making process for treatment strategies and tailored follow-ups to decrease/prevent recurrence., Methods: The authors performed a comprehensive and detailed systematic literature review of the EMBASE and MEDLINE electronic online databases regarding the topographic pattern of recurrence after surgical treatment for intracranial meningiomas. Demographics and histopathological, neuroradiological and treatment data, pertinent to the topography of recurrences, as well as time to recurrences, were extracted and analyzed., Results: Four studies, including 164 cases of recurrences according to the inclusion criteria, were identified. All studies consider the possibility of recurrence at the previous dural site; three out of four, which are the most recent, consider 1 cm outside the previous dural margin to be the main limit to distinguish recurrences closer to the previous site from those more distant. Recurrences mainly occur within or close to the surgical bed; higher values of proliferation index are associated with recurrences close to the original site rather than within it., Conclusions: Further studies, including genomic characterization of different patterns of recurrence, will better clarify the main features affecting the topography of recurrences. A comparison between topographic classifications of intracranial meningioma recurrences after surgery and after radiation treatment could provide further interesting information.

Surgery stands as the primary treatment for spheno-orbital meningiomas, following a symptoms-oriented approach. We discussed the decision-making process behind surgical strategies through a review of medical records from 80 patients who underwent surgical resection at the University of Naples Federico II. Different surgical approaches were employed based on the tumor's location relative to the optic nerve's long axis, categorized into lateral (type I), medial (type II), and diffuse (type III). We examined clinical, neuroradiological, surgical, pathological, and outcome factors. Proptosis emerged as the most frequent symptom (97%), followed by visual impairment (59%) and ocular motility issues (35%). Type I represented 20%, type II 43%, and type III 17%. Growth primarily affected the optic canal (74%), superior orbital fissure (65%), anterior clinoid (60%), and orbital apex (59%). The resection outcomes varied, with Simpson grades I and II achieved in all type I cases, 67.5% of type II, and 18% of type III. Recurrence rates were highest in type II (41.8%) and type III (59%). Improvement was notable in proptosis (68%) and visual function (51%, predominantly type I). Surgery for spheno-orbital meningiomas should be tailored to each patient, considering individual characteristics and tumor features to improve quality of life by addressing primary symptoms like proptosis and visual deficits.

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.

Objective: The superior eyelid endoscopic transorbital approach (SETOA) provides a direct and short minimally invasive route to the anterior and middle skull base. Nevertheless, it uses a narrow corridor that limits its angles of attack. The aim of this study was to evaluate the feasibility and potential benefits of an "extended" conservative variant of the "standard" endoscopic transorbital approach-termed "open-door"-to enhance the exposure of lesions affecting the paramedian aspect of the anterior and middle cranial fossae., Methods: First, the authors described the technical nuances of the open-door extended transorbital approach (ODETA). Next, they documented its morphometric advantages over standard SETOA. Finally, they provided a clinical-anatomical application to demonstrate enhanced exposure and better angles of attack to treat lesions occupying the paramedian anterior and middle cranial fossae. Five adult cadaveric specimens (10 sides) initially underwent standard SETOA and then extended open-door SETOA (ODETA to the paramedian anterior and middle fossae). The adjunct of hinge-orbitotomy, through three surgical steps and straddling the frontozygomatic suture, converted conventional SETOA to its extended open-door variant. CT scans were performed before dissection and uploaded to the neuronavigation system for quantitative analysis. The angles of attack on the axial plane that addressed four key landmarks, namely the tip of the anterior clinoid process (ACP), foramen rotundum (FR), foramen ovale (FO), and trigeminal impression (TI), were calculated for both operative techniques and compared., Results: Hinge-orbitotomy of the extended open-door SETOA resulted in several surgical, functional, and esthetic advantages: it provided wider axial angles of attack for each of the target points, with a gain angle of 26.68° ± 1.31° for addressing the ACP (p < 0.001), 29.50° ± 2.46° for addressing the FR (p < 0.001), 19.86° ± 1.98° for addressing the FO (p < 0.001), and 17.44° ± 2.21° for addressing the lateral aspect of the TI (p < 0.001), while hiding the skin scar, avoiding temporalis muscle dissection, preserving flap vascularization, and decreasing the rate of bone infection and degree of orbital content retraction., Conclusions: The extended open-door technique may be specifically suited for selected patients affected by paramedian anterior and middle fossae lesions, with prevalent anteromedial extension toward the anterior clinoid, the foremost compartment of the cavernous sinus and FR and not completely controlled with the pure endoscopic transorbital approach.

During the last few years, the superior eyelid endoscopic transorbital approach has been proposed as a new minimally invasive pathway to access skull base lesions, mostly in ophthalmologic, otolaryngologic, and maxillofacial surgeries. However, most neurosurgeons performing minimally invasive endoscopic neurosurgery do not usually employ the orbit as a surgical corridor. The authors undertook this technical and anatomical study to contribute a neurosurgical perspective, exploring the different possibilities of this novel route. Ten dissections were performed on ten formalin-fixed specimens to further refine the transorbital technique. As part of the study, the authors also report an illustrative transorbital surgery case to further detail key surgical landmarks. Herein, we would like to discuss equipment, key anatomical landmarks, and surgical skills and stress the steps and details to ensure a safe and successful procedure. We believe it could be critical to promote and encourage the neurosurgical community to overcome difficulties and ensure a successful surgery by following these key recommendations.

Background : The cavernous sinus (CS) is a highly vulnerable anatomical space, mainly due to the neurovascular structures that it contains; therefore, a detailed knowledge of its anatomy is mandatory for surgical unlocking. In this study, we compared the anatomy of this region from different endoscopic and microsurgical operative corridors, further focusing on the corresponding anatomic landmarks encountered along these routes. Furthermore, we tried to define the safe entry zones to this venous space from these three different operative corridors, and to provide indications regarding the optimal approach according to the lesion's location. Methods : Five embalmed and injected adult cadaveric specimens (10 sides) separately underwent dissection and exposure of the CS via superior eyelid endoscopic transorbital (SETOA), extended endoscopic endonasal transsphenoidal-transethmoidal (EEEA), and microsurgical transcranial fronto-temporo-orbito-zygomatic (FTOZ) approaches. The anatomical landmarks and the content of this venous space were described and compared from these surgical perspectives. Results : The oculomotor triangle can be clearly exposed only by the FTOZ approach. Unlike EEEA, for the exposure of the clinoid triangle content, the anterior clinoid process removal is required for FTOZ and SETOA. The supra- and infratrochlear as well as the anteromedial and anterolateral triangles can be exposed by all three corridors. The most recently introduced SETOA allowed for the exposure of the entire lateral wall of the CS without entering its neurovascular structures and part of the posterior wall; furthermore, thanks to its anteroposterior trajectory, it allowed for the disclosure of the posterior ascending segment of the cavernous ICA with the related sympathetic plexus through the Mullan's triangle, in a minimally invasive fashion. Through the anterolateral triangle, the transorbital corridor allowed us to expose the lateral 180 degrees of the Vidian nerve and artery in the homonymous canal, the anterolateral aspect of the lacerum segment of the ICA at the transition zone from the petrous horizontal to the ascending posterior cavernous segment, surrounded by the carotid sympathetic plexus, and the medial Meckel's cave. Conclusions : Different regions of the cavernous sinus are better exposed by different surgical corridors. The relationship of the tumor with cranial nerves in the lateral wall guides the selection of the approach to cavernous sinus lesions. The transorbital endoscopic approach can be considered to be a safe and minimally invasive complementary surgical corridor to the well-established transcranial and endoscopic endonasal routes for the exposure of selected lesions of the cavernous sinus. Nevertheless, peer knowledge of the anatomy and a surgical learning curve are required.

Introduction: Posterior reversible encephalopathy syndrome (PRES) is a neurological disorder characterized by neurological symptoms and distinctive neuroimaging findings. There are a few cases reported in the literature in which PRES can occur after surgery, and there is no clear direct relationship between a procedure and its debut. Methods: We performed a review of the literature by analyzing all reported cases of PRES syndrome which debuted after a surgical procedure with the aim of identifying the clinical features, the timing of the symptoms' onset and the therapy of patients suffering from this unusual surgical complication. Results: The total number of patients collected was 47, with a mean age of 40.9 years. Postoperative PRES can occur in either pediatric or adult patients (ages 4-82 years). The most frequent form of comorbidity reported was cardiovascular disease (fourteen patients, 29.78%). Sixteen patients (36%) had no relevant risk factors or comorbidities at the time of the surgical procedure. The types of surgery most correlated were cranial neuro and maxillofacial surgery (twenty-one patients, 44.68%) followed by transplant surgery (eight patients, 17%). The time of onset of PRES after surgery occurred within the first 3 weeks (mean time of onset 4.7 days), and when rapidly treated with antihypertensive and antiepileptic drugs appeared to have a reversible and benign course. Conclusion: PRES syndrome can be considered a rare complication of procedures and can occur following a wide range of surgeries, especially cranial and transplant surgery. Being able to recognize it in time and treat it ensures a full reversibility of symptoms in most cases.

Giant pituitary adenomas are a subgroup of pituitary adenomas defined by a diameter greater than 4 cm, and they account for 5-14% of adenomas in surgical series. Because of their growth patterns and locations, often involving critical neurovascular structures, they represent a true surgical challenge, and gross total resection is difficult to achieve. There is no consensus on the optimal surgical strategy for giant pituitary adenomas, and, often, integrated multi-staged treatment strategies have been considered. Transcranial or transsphenoidal approaches, alone or combined, according to tumor and patient features are the two main routes. Each of these strategies has pros and cons. The conventional transcranial approach has for a long time been considered the first choice for the removal of giant pituitary adenomas. Currently, with endoscopic techniques, it is also possible to remove lesions that involve the intradural compartment and the adjacent neurovascular structures with the use of extended approaches. Our policy for the management of these lesions is to adopt the endoscopic endonasal approach as the first choice unless the tumor presents significant intracranial extension that results in it being outside the visibility and maneuverability of the endoscopic endonasal route. In these latter cases, we agree that the transcranial approach is more appropriate. However, accurate preoperative evaluation and refined treatment plans for each patient are mandatory to define a proper strategy in order to achieve the most effective long-term result.

Background: Ollier disease (OD) is a rare nonhereditary type of dyschondroplasia characterized by multiple enchondromas, with typical onset in the first decade of life. Surgery is the only curative treatment for primary disease and its complications. Patients with OD are at risk of malignant transformation of enchondromas and of occurrence of other neoplasms. Methods: A wide literature review disclosed thirty cases of glioma associated with OD, most of them belonging to the pre-molecular era. Our own case was also included. Demographic, clinical, pathologic, molecular, management, and outcome data were analyzed and compared to those of sporadic gliomas. Results: Gliomas associated with OD more frequently occur at younger age, present higher rates of multicentric lesions (49%), brainstem localizations (29%), and significantly lower rates of glioblastomas (7%) histotype. The IDH1 R132H mutation was detected in 80% of gliomas of OD patients and simultaneously in enchondromas and gliomas in 100% of cases. Conclusions: The molecular data suggest a higher risk of occurrence of glioma in patients with enchondromas harboring the IDH1 R132H mutation than those with the IDH1 R132C mutation. Thus, we suggest considering the IDH1 R132H mutation in enchondromas of patients with OD as a predictive risk factor of occurrence of glioma.

Background: Due to the few reported cases of spinal intradural metastases from renal cell carcinoma (RCC), there is no unanimous consensus on the best treatment strategy, including the role of surgery., Methods: A wide and accurate literature review up to January 2022 has disclosed only 51 cases of spinal intradural metastases from RCC. Patients with extramedullary (19) and those with intramedullary (32) localization have been separately considered and compared. Demographics, clinical, pathological, management, and outcome features have been analyzed., Results: Extramedullary lesions more frequently showed the involvement of the lumbar spine, low back pain, and solitary metastasis at diagnosis. Conversely, the intramedullary lesions were most often detected in association with multiple localizations of disease, mainly in the brain. Surgery resulted in improvement of clinical symptoms in both groups., Conclusion: Several factors affect the prognosis of metastatic RCC. The surgical removal of spinal metastases resulted in pain relief and the arresting of neurological deficit progression, improving the quality of life and overall survival of the patient. Considering the relative radioresistant nature of the RCC, the surgical treatment of the metastasis is a valid option even if it is subtotal, with a consequent increased risk of recurrence, and/or a nerve root should be sacrificed.

Background: Several epidemiological and pathological findings suggest that the female sex hormones may influence the development of meningiomas. However, the role of pregnancy, oral contraceptives, and fertilization therapies is still controversial., Methods: From the surgical series of 354 patients with meningiomas operated between 2006 and 2019, the group of 72 premenopausal women was separately considered. The tumor location, WHO grade, Ki67-labeling index (LI), progesterone receptor (PR) expression, and histological types were studied in premenopausal women with and without hormone-related conditions were compared., Results: In this premenopausal group, 24 patients had hormone-related conditions, including use of oral contraceptives in 16, intrauterine fertilization in one, pregnancy in three, and tumors of the female reproductive system in four. The group of patients with hormone-related conditions, as compared to that with no hormone related conditions, showed slightly lower median age (38 versus 43 years) and no significant difference of meningioma location WHO grade, Ki 67-Li, PR expression and histological type. The clinical onset during pregnancy in three patients and tumor growth during contraceptive progesterone therapy in two others were evidenced., Conclusion: The biological behavior of meningiomas and their pathological findings, including PR expression, are not correlated with the different hormone related conditions in premenopausal female patients. Contraceptives and fertilization therapies, mainly with progesterone, should be avoided in patients with meningiomas., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2020 Maiuri, Mariniello, Somma, Guadagno, Corvino, Pagano, Orlando and Del Basso De Caro.)

Background: Anterior cervical osteophytes (ACOs) may rarely cause dysphagia, dysphonia, and dyspnea. Symptomatic ACOs are most commonly located between C3 and C7, whereas those at higher cervical (C1-C2) levels are rarer. We report a case series of 4 patients and discuss the best surgical approach according to the ostheophyte location and size, mainly for those located at C1-C2, and the related surgical problems., Materials and Methods: Four patients (two males and two females) aged from 57 to 72 years were operated on for ACOs, causing variable dysphagia (and dyspnea with respiratory arrest in one). Three patients with osteophytes between C3 and C5 were approached through antero-lateral cervical approach, and one with a large osteophyte between C1 and C3-C4 level underwent a two-stage transcervical and transoral approach. All had significant postoperative improvement of dysphagia., Results: The patient operated on though the transoral approach experienced postoperative flogosis of the prevertebral tissues and occipital muscles and thrombosis of the right jugular vein and transverse-sigmoid sinuses (Lemierre syndrome)., Conclusion: The transoral approach is the best surgical route to resect C1 and C2 ACOs, whereas the endoscopic endonasal approach is not indicated. The anterior transcervical approach is easier to resect osteophytes at C3, as well as those located below C3. A combined transoral and anterior cervical approach may be necessary for multilevel osteophytes., Competing Interests: There are no conflicts of interest., (Copyright: © 2020 Journal of Craniovertebral Junction and Spine.)