Multicompartmental Paranasal Sinus Osteoma Complicated by Frontal Bone Hyperostosis, Intracranial Mucocele, and Inflammatory Pseudotumor.

Parasinusal osteoma complicated by intracranial and orbit extension, cranial vault hyperostosis, intracranial mucocele, and inflammatory pseudotumor is exceptional. A 68-year-old man presented with a long history of progressive proptosis and recurrent episodes of keratoconjunctivitis in the left eye, with restriction in upward gaze. Contrast-enhanced magnetic resonance imaging revealed a frontal sinus lesion extending to the left anterior fossa and orbit, featuring an intracranial cystic component and heterogeneous contrast enhancement. Head computed tomography confirmed the double calcific-cystic nature of the lesion. A left supraorbital-pterional approach allowed complete resection of mucocele and drilling of intracranial and orbital osteoma, including the intrasinusal component. The frontal sinus was cranialized, and a flap of pericranium, reinforced by Gelfoam sponge, was reflected on the anterior cranial base/orbital roof. The postoperative course was uneventful; magnetic resonance imaging depicted resolution of proptosis. Histological examination favored parasinusal osteoma associated with intracranial mucocele, frontal bone hyperostosis, and inflammatory pseudotumor.

Topographic Patterns of Intracranial Meningioma Recurrences-Systematic Review with Clinical Implication.

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.

Spheno-Orbital Meningiomas: The Rationale behind the Decision-Making Process of Treatment Strategy.

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.

Cervical epidural abscess complicated by a pharyngoesophageal perforation after anterior cervical spine surgery for subaxial spondylodiscitis.

Background: The anterior approach to the cervical spine is safe and effective, but not without risks. The pharyngoesophageal perforation (PEP) is a rare but potentially life-threatening complication of this surgical route. A prompt diagnosis and adequate treatment are crucial for the prognosis; nevertheless, there is no unique consent about the best management. Case Description: A 47-year-old woman was referred to our neurosurgical unit for clinical and neuroradiological signs of multilevel cervical spine spondylodiscitis, which was conservatively treated with long-term antibiotic therapy and cervical immobilization after computed tomography-guided biopsy. Nine months later, when the infection was resolved, the patient underwent C3-C6 spinal fusion with anterior plate and screws through anterior approach to the cervical spine for degenerative vertebral changes causing severe myelopathy, and C5- C6 retrolisthesis with instability. Five days after surgical procedure, the patient developed a pharyngoesophageal-cutaneous fistula, detected through wound drainage, and confirmed by swallowing contrast study, without systemic signs of infection. The PEP was conservatively treated, with antibiotic therapy and parenteral nutrition, and it was monitored through seriate swallowing contrast and magnetic resonance studies up to the complete resolution. Conclusion: The PEP is a potentially fatal complication of the anterior cervical spine surgery. We suggest an accurate intraoperative control of the pharyngoesophageal's tract integrity at the end of the surgical procedure and a longtime follow-up, because the risk of occurrence is up to several years after surgery.

Management of the skull base invasion in spheno-orbital meningiomas.

BACKGROUND: The tumor invasion of the skull base structures is very frequent in spheno-orbital meningiomas. The aim of the present study is to evaluate the invasion rate of skull base structures and the best surgical approach and management. METHODS: The surgical series of 80 spheno-orbital meningiomas was reviewed. The tumors were classified according to the intraorbital location with respect to the optic nerve axes into three types: I-lateral: II-medial; III-diffuse. The invasion of the orbital apex, optic canal, superior orbital fissure, anterior clinoid, ethmoid-sphenoid sinuses, and infratemporal fossa was evaluated. The rate and extension of involvement of these structures was correlated with the intraorbital location and the surgical approach. The preoperative ophtalmological symptoms and signs and their outcome were also evaluated. RESULTS: Proptosis was found in 79 patients (97%), variable decrease of the visual function in 47 patients (59%), and deficits of the eye movements in 28(35%). The invasion of the optic canal (74%), superior orbital fissure (65%), anterior clinoid (60%), and orbital apex (59%) was more frequently found, whereas the tumor extension into the ethmoid-sphenoid sinuses (4%) and infratemporal fossa (4%) was rare. Types II and III meningiomas showed significantly higher involvement of the skull base structures than type I ones, which only had 15% invasion of the optic canal. Remission or significant improvement of the visual function occurred postoperatively in 24 among 47 cases (51%), with a higher rate for type I meningiomas vs. other types (p = 0.021, p = 0.019) and worsening in 7 (15%). CONCLUSIONS: Spheno-orbital meningiomas growing in the lateral orbital compartment show no involvement of the skull base structures excepting the optic canal as compared to those growing medially or diffusely. The surgical resection of tumor invading the skull base structures should be more extensive as possible, but the risk of optic and oculomotor deficits must be avoided.

Spinal cervical extradural hemangioblastoma.

Spinal cervical extradural and intra-extradural hemangioblastomas are exceptional, with only nine reported cases. This study reviews the diagnostic and surgical problems of this rare entity. Two female patients, aged 80 years and 25 years, respectively, one with Von Hippel-Lindau disease (VHLD), experienced brachial pain and weakness. On magnetic resonance imaging, a dumbbell intra-extraspinal hemangioblastoma was evidenced. The surgical resection through posterior laminectomy resulted in clinical remission of brachial pain and weakness. The magnetic resonance aspect of a dumbbell lesion suggests a neurogenic tumor; the correct preoperative diagnosis is possible in individuals with VHLD. The surgical problems include high tumor vascularity, vertebral artery control, and nerve root preservation. However, the surgical excision results in clinical remission.

Brain Gliomas and Ollier Disease: Molecular Findings as Predictive Risk Factors?

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.

The Role of Surgery in Spinal Intradural Metastases from Renal Cell Carcinoma: A Literature Review.

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.

Recurrences of Spheno-Orbital Meningiomas: Risk Factors and Management.

BACKGROUND: The extent of resection constitutes one of the most important predictive risk factors of recurrence for spheno-orbital meningiomas; although gross total resection represents the gold standard, it is not always achievable, with a consequent high rate of recurrence. Management of these tumors is a surgical challenge and is represented by maximal safe resection with preservation of function. The aim of the present study is to discuss the risk factors for recurrence and the best management of the recurrent tumors. METHODS: Eighty patients operated on for spheno-orbital meningiomas were retrospectively reviewed. Two groups were identified: group 1 comprised 30 patients (37.5%) who experienced recurrence and group 2 comprised 50 patients with no recurrence from 5 to 28 years after the initial surgery. The analyzed factors in both groups include the involvement of the skull base structures, the extent of resection, and World Health Organization grade. In the recurrence group, the pattern of tumor regrowth, the entity of resection, and the management were also analyzed. RESULTS: The invasion of the whole orbit, the involvement of the orbital apex, superior orbital fissure, infratemporal fossa, and sphenoidal-ethmoidal sinuses, and World Health Organization grade II are risk factors of recurrence. All 30 patients with recurrence underwent reoperation, 9 of whom had 2 or 3 reoperations. Overall, 70 of the 80 patients (88%) had tumor control and no progression after one or more reoperations. CONCLUSIONS: We suggest re-surgery for spheno-orbital meningioma recurrences to prevent worsening of visual function and proptosis. Because of their slow natural course, even multiple reoperations may be performed, resulting in long overall survival with stable symptoms and good quality of life.

Microsurgical repair by autografting in traumatic injuries of peripheral nerves.

BACKGROUND: Peripheral nerve injuries (PNI) with traumatic origin in upper and lower extremities are pathologies with an important quality-of-life considerations. For neurosurgeons, it is mandatory to understand their physiopathological mechanism and a proper treatment. The authors describe a series of 50 patients with traumatic PNI who underwent microsurgical reconstruction by autologous graft between 1993 and 2014. METHODS: Patients aged less than 65, who had neurological deficit in a range 3-12 months and severe and persistent nerve injury at electrophysiological examination were included in the study. After grafting, the overall functional recovery of the neurological deficit, the main prognostic factors (age, injured nerve, damage mechanism, gap length and surgical timing) and the postoperative functional outcome were analyzed. Neurological and electrophysiological follow-up was at 6-/12-/24-/48 months after surgery. RESULTS: The results showed that the best neurological recovery can be found in young patients with cut injuries of the upper limbs and that the outcome mainly depends on involved nerve, lesion type and minimum gap length. Moreover, follow-up for these pathologies should last till 48 months. CONCLUSIONS: In the future, these results could be further improved by the rising new techniques of nerve repair and the advances in neurobiology.

Malignant intraventricular meningioma: literature review and case report.

Malignant intraventricular meningiomas (IVMs) are very rare with only a few reported cases. A midline search up to December 2020 selected 40 articles for a total of 65 patients. The inclusion criteria were series and case reports in English language, as well as papers written in other languages, but with abstracts written in English. Malignant IVMs at the first diagnosis (group A, 50 patients) and those with anaplastic transformation from previous WHO grades I and II tumors (group B, 15 patients) were separately analyzed. The unique personal case among 1285 meningiomas (0.078%) is also added. Malignant IVMs mainly occur in women (61%) with a median age of 45 years and are mainly located in the lateral ventricle (93%) and trigonal region (74%), with no cases in the fourth ventricle. Irregular borders (80%), heterogeneous enhancement (83%), and perilesional edema (76%) are the most frequent radiological findings. The histology was mainly pure anaplastic (85%), whereas papillary (7%), rhabdoid (5%), and mixed forms (3%) are very rare. The CSF spread was found in 60% of the cases. The prognosis is very dismal, with an overall median survival of 17.5 months after surgery for the anaplastic forms. Malignant IVMs at initial diagnosis (group A) show better overall survival (25 months) than those occurring from anaplastic transformation of lower grade tumors (group B) (10.1 months).

Progesterone Receptor Expression in Meningiomas: Pathological and Prognostic Implications.

BACKGROUND: The progesterone receptor (PR) is variably expressed in most meningiomas and was found to have prognostic significance. However, the correlation with patient age, tumor location, time to recurrence, and pattern of regrowth has scarcely been discussed. METHODS: A surgical series of 300 patients with meningiomas is reviewed. The PR expression was classified as: 0. absent; 1. low (<15%); 2. moderately low (16-50%); 3. moderately high (51-79%); 4. high (≥80%). The PR values were correlated with the patient age and sex, meningioma location, WHO grade, Ki-67 MIB1, recurrence rate, pattern of recurrence (local-peripheral versus multicentric diffuse), and time to recurrence. RESULTS: The PR expression has shown lower rate of high expression in the elderly group (p = 0.032) and no sex difference (including premenopausal versus postmenopausal women), higher expression in medial skull base and spinal versus other locations (p = 0.0036), inverse correlation with WHO grade and Ki67-MIB1 (p < 0.0001). Meningiomas which recurred showed at initial surgery higher rates of low or moderately low PR expression than the non-recurrent ones (p = 0.0004), whereas the pattern of regrowth was not significant. Higher rates of PR values ≥80% were found in cases with time to recurrence >5 years (p = 0.036). CONCLUSION: The higher PR expression in medial skull base meningiomas, the significant correlation with the time to recurrence, the lack of difference of PR expression between premenopausal and postmenopausal women and between local-peripheral versus multicentric-diffuse recurrences are the most relevant unreported findings of this study. The rate of PR expression must be included in the routine pathological diagnosis of meningiomas because of its prognostic significance.

Cavernous Malformations to Be Investigated for Familiarity: The Role of Ki67 MIB1.

BACKGROUND: Familial forms of cavernous malformations (CMs) often occur as multiple lesions. Nevertheless, the presence of a single CM does not exclude the familiarity. The aim of this study is to establish which patients who undergo surgery for a single cerebral cavernous malformation (CCM), with no family history at initial diagnosis, should be investigated for familiarity through genetic testing and counseling. METHODS: Eight families with 2 or more members affected by CCM have been studied. A control group of sporadic cases operated on, with no family history and followed up 10 years or more, was also included. Analyzed factors were patient age and sex, location, number and size of the lesions, associated developmental venous anomaly, presence of epileptic seizures, symptomatic hemorrhage, focal neurological deficits, and documented growth of the malformation and Ki67 MIB1 proliferation index. RESULTS: The familial group of CCMs showed higher incidence of pediatric patients (P = 0.01), more frequent occurrence of multiple lesions (P = 0.0004), higher rate of large CCMs, and symptomatic hemorrhage; besides, all 3 cases with documented growth belonged to the familial group (14%). The expression of Ki67 MIB1 was positive in 79% of the familial cases versus 0% in the sporadic ones (P < 0.00001). CONCLUSIONS: Patients with CCM and no known family history at the time of the initial diagnosis who present specific features should be studied by genetic screening. The Ki67 MIB1 is a useful biomarker in favor of familial occurrence and may be studied in all patients with CMs to define the indication to the genetic tests.

Postoperative Lemierre's syndrome: a previously unreported complication of transoral surgery. Illustrative case.

BACKGROUND: Lemierre's syndrome is a rare but potentially life-threatening clinical condition characterized by bacteremia and thrombophlebitis of the internal jugular vein, usually secondary to oropharyngeal infection and often caused by Fusobacterium necrophorum; rarely, it occurs after surgical procedures. The most common clinical presentation includes acute pharyngitis, high fever, and neck pain. The diagnosis is based on blood culture and cranial and cervical spine computed tomography (CT)/magnetic resonance imaging (MRI) with contrast. Antibiotic therapy for 3-6 weeks is the mainstay of treatment, while the use of anticoagulant drugs is controversial. OBSERVATIONS: The authors describe a case of Lemierre's syndrome that occurred after transoral surgery. The patient underwent a combined surgical approach from above (transoral) and below (anterolateral transcervical) to the upper cervical spine for the resection of a large anterior osteophyte causing dysphagia, globus sensation, and dysphonia. Three weeks after the surgical procedure, she developed fever and severe neck pain. LESSONS: The aim of this paper is to consider Lemierre's syndrome as a possible complication after the transoral approach, underlining the importance of its early diagnosis and with a suggestion to perform cranial and cervical spine CT or MRI venous angiography in patients who undergo surgery with a transoral approach and exhibit local or systemic signs of infection such as neck pain, persistent fever, and positive blood culture results.

Risks and Benefits of Surgical Excision of Orbital Cavernous Venous Malformations (So-Called Cavernous Hemangioma): Factors Influencing the Outcome.

PURPOSE: To assess risks and benefits associated with surgical excision of orbital cavernous venous malformation and analyze factors influencing the outcome. METHODS: Design: cohort study. Participants: 164 consecutive patients undergoing surgical removal of orbital cavernous venous malformation. Intervention: lateral or anterior orbitotomy. Outcome measures: visual acuity, visual field, pupillary function, ocular motility, and fundus examination. Influence of the mass volume and tumor location on the outcome was evaluated. The threshold value and risk group for the volume were evaluated using the receiver operating characteristic analysis; 8 orbital different quadrants, extraconal/intraconal position, and orbital apex extension were considered as variables to evaluate the location. RESULTS: Postoperatively overall visual acuity (p = 0.0084), visual field (p < 0.0001), diplopia (p < 0.0001), proptosis (p < 0.0001), ocular motility (p = 0.02), ptosis (p = 0.033), choroidal fold (p < 0.0001), disk edema (p = 0.0004) were significantly improved. The commonest location was the lateral quadrant (28%). Choroidal folds were associated with tumor greater than 1100 mm3 (p = 0.001). Visual loss occurred in 2 patients (1.2%) and tonic pupil in 5 (3%,) Visual deterioration was associated with the apical extension (p = 0.001). Pupil efferent defect was associated with lateral quadrant location (p = 0.011) and apical extension (p = 0.05). CONCLUSIONS: Surgical removal of orbital cavernous venous malformation is a useful treatment modality, particularly for large size tumors. Removal of cavernous venous malformations located at the lateral aspect of the orbit carries an increased risk of permanent tonic pupil. Postoperative blindness is rarely seen and is often associated with the apical extension. Tailoring the surgical approach, depending on the relative position to the optic nerve, may have an important bearing on outcome.

Meningiomas in Premenopausal Women: Role of the Hormone Related Conditions.

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.