Response of treatment-naive brain metastases to stereotactic radiosurgery.

With improvements in survival for patients with metastatic cancer, long-term local control of brain metastases has become an increasingly important clinical priority. While consensus guidelines recommend surgery followed by stereotactic radiosurgery (SRS) for lesions >3 cm, smaller lesions (≤3 cm) treated with SRS alone elicit variable responses. To determine factors influencing this variable response to SRS, we analyzed outcomes of brain metastases ≤3 cm diameter in patients with no prior systemic therapy treated with frame-based single-fraction SRS. Following SRS, 259 out of 1733 (15%) treated lesions demonstrated MRI findings concerning for local treatment failure (LTF), of which 202 /1733 (12%) demonstrated LTF and 54/1733 (3%) had an adverse radiation effect. Multivariate analysis demonstrated tumor size (>1.5 cm) and melanoma histology were associated with higher LTF rates. Our results demonstrate that brain metastases ≤3 cm are not uniformly responsive to SRS and suggest that prospective studies to evaluate the effect of SRS alone or in combination with surgery on brain metastases ≤3 cm matched by tumor size and histology are warranted. These studies will help establish multi-disciplinary treatment guidelines that improve local control while minimizing radiation necrosis during treatment of brain metastasis ≤3 cm.

Intraventricular Glioma in Pediatric Patients: A Systematic Review of Demographics, Clinical Characteristics, and Outcomes.

OBJECTIVE: We conducted a systematic review on pediatric intraventricular gliomas to survey the patient population, tumor characteristics, management, and outcomes. METHODS: PubMed, Scopus, Web-of-Science, and Cochrane were searched using PRISMA guidelines to include studies reporting pediatric patients with intraventricular gliomas. RESULTS: A total of 30 studies with 317 patients were included. Most patients were male (54%), diagnosed at a mean age of 8 years (0.2-19), and frequently exhibited headache (24%), nausea and vomiting (21%), and seizures (15%). Tumors were predominantly located in the fourth (48%) or lateral ventricle (44%). Most tumors were WHO grade 1 (68%). Glioblastomas were rarely reported (2%). Management included surgical resection (97%), radiotherapy (27%), chemotherapy (8%), and cerebrospinal fluid diversion for hydrocephalus (38%). Gross total resection was achieved in 59% of cases. Cranial nerve deficit was the most common postsurgical complication (28%) but most were reported in articles published prior to the year 2000 (89%). Newer cases published during or after the year 2000 exhibited significantly higher rates of gross total resection (78% vs. 39%, P < 0.01), lower rates of recurrence (26% vs. 47%, P < 0.01), longer average overall survival time (42 vs. 21 months, P = 0.02), and a higher proportion of patients alive (83% vs. 70%, P = 0.03) than in older cases. CONCLUSIONS: Pediatric intraventricular gliomas correlate with parenchymal pediatric gliomas in terms of age at diagnosis and general outcomes. The mainstay of management is complete surgical excision and more recent studies report longer overall survival rates and less cranial nerve complications.

Leiomyogenic Tumor of the Spine: A Systematic Review.

The study cohort consisted of 83 patients with a mean age of 49.55 (SD 13.72) with a female preponderance (60 patients). Here, 32.14% of patients had primary LTS; the remaining were metastases. Clinical presentation included nonspecific back pain (57.83%), weakness (21.69%) and radicular pain (18.07%). History of uterine neoplasia was found in 33.73% of patients. LTS preferentially affected the thoracic spine (51.81%), followed by the lumbar (21.67%) spine. MRI alone was the most common imaging modality (33.33%); in other cases, it was used with CT (22.92%) or X-ray (16.67%); 19.23% of patients had Resection/Fixation, 15.38% had Total en bloc spondylectomy, and 10.26% had Corpectomy. A minority of patients had laminectomy and decompression. Among those with resection, 45.83% had a gross total resection, 29.17% had a subtotal resection, and 16.67% had a near total resection. Immunohistochemistry demonstrated positivity for actin (43.37%), desmin (31.33%), and Ki67 (25.30). At a follow-up of 19.3 months, 61.97% of patients were alive; 26.25% of 80 patients received no additional treatment, 23.75% received combination radiotherapy and chemotherapy, only chemotherapy was given to 20%, and radiotherapy was given to 17.5%. Few (2.5%) had further resection. For an average of 12.50 months, 42.31% had no symptoms, while others had residual (19.23%), other metastasis (15.38%), and pain (7.69%). On follow-up of 29 patients, most (68.97%) had resolved symptoms; 61.97% of the 71 patients followed were alive.

Subependymomas of the fourth ventricle: To operate or not to operate?

BACKGROUND: There is a paucity of literature regarding the clinical characteristics and management of subependymomas of the fourth ventricle due to their rarity. Here, we describe the operative and non-operative management and outcomes of patients with such tumors. METHODS: This retrospective single-institution case series was gathered after Institutional Review Board (IRB) approval. Patients diagnosed with a subependymoma of the fourth ventricle between 1993 and 2021 were identified. Clinical, radiology and pathology reports along with magnetic resonance imaging (MRI) images were reviewed. RESULTS: Patients identified (n = 20), showed a male predominance (n = 14). They underwent surgery (n = 9) with resection and histopathological confirmation of subependymoma or were followed with imaging surveillance (n = 11). The median age at diagnosis was 51.5 years. Median tumor volume for the operative cohort was 8.64 cm3 and median length of follow-up was 65.8 months. Median tumor volume for the non-operative cohort was 0.96 cm3 and median length of follow-up was 78 months. No tumor recurrence post-resection was noted in the operative group, and no tumor growth from baseline was noted in the non-operative group. Most patients (89 %) in the operative group had symptoms at diagnosis, all of which improved post-resection. No patients were symptomatic in the non-operative group. CONCLUSIONS: Surgical resection is safe and is associated with alleviation of presenting symptoms in patients with large tumors. Observation and routine surveillance are warranted for smaller, asymptomatic tumors.

Orbital Exenteration for Craniofacial Lesions: A Systematic Review and Meta-Analysis of Patient Characteristics and Survival Outcomes.

BACKGROUND: The outcomes of orbital exenteration (OE) in patients with craniofacial lesions (CFLs) remain unclear. The present review summarizes the available literature on the clinical outcomes of OE, including surgical outcomes and overall survival (OS). METHODS: Relevant articles were retrieved from Medline, Scopus, and Cochrane according to PRISMA guidelines. A systematic review and meta-analysis were conducted on the clinical characteristics, management, and outcomes. RESULTS: A total of 33 articles containing 957 patients who underwent OE for CFLs were included (weighted mean age: 64.3 years [95% CI: 59.9-68.7]; 58.3% were male). The most common lesion was squamous cell carcinoma (31.8%), and the most common symptom was disturbed vision/reduced visual acuity (22.5%). Of the patients, 302 (31.6%) had total OE, 248 (26.0%) had extended OE, and 87 (9.0%) had subtotal OE. Free flaps (33.3%), endosseous implants (22.8%), and split-thickness skin grafts (17.2%) were the most used reconstructive methods. Sino-orbital or sino-nasal fistula (22.6%), flap or graft failure (16.9%), and hyperostosis (13%) were the most reported complications. Regarding tumor recurrences, 38.6% were local, 32.3% were distant, and 6.7% were regional. The perineural invasion rate was 17.4%, while the lymphovascular invasion rate was 5.0%. Over a weighted mean follow-up period of 23.6 months (95% CI: 13.8-33.4), a weighted overall mortality rate of 39% (95% CI: 28-50%) was observed. The 5-year OS rate was 50% (median: 61 months [95% CI: 46-83]). The OS multivariable analysis did not show any significant findings. CONCLUSIONS: Although OE is a disfiguring procedure with devastating outcomes, it is a viable option for carefully selected patients with advanced CFLs. A patient-tailored approach based on tumor pathology, extension, and overall patient condition is warranted.

Bilateral basal ganglia hemorrhage: a systematic review of etiologies, management strategies, and clinical outcomes.

Bilateral basal ganglia hemorrhages (BBGHs) represent rare accidents, with no clear standard of care currently defined. We reviewed the literature on BBGHs and analyzed the available conservative and surgical strategies. PubMed, Scopus, Web of Science, and Cochrane were searched following the PRISMA guidelines to include studies reporting patients with BBGHs. Clinical characteristics, management, and outcomes were analyzed. We included 64 studies comprising 75 patients, 25 (33%) traumatic and 50 (67%) non-traumatic. Traumatic cases affected younger patients (mean age 35 vs. 46 years, p=0.014) and males (84% vs. 71%, p=0.27) and were characterized by higher proportion of normal blood pressures at admission (66% vs. 13%, p=0.0016) compared to non-traumatic cases. Most patients were comatose at admission (56%), with a mean Glasgow Coma Scale (GCS) score of 7 and a higher proportion of comatose patients in the traumatic than in the non-traumatic group (64% vs. 52%, p=0.28). Among the traumatic group, motor vehicle accidents and falls accounted for 79% of cases. In the non-traumatic group, hemorrhage was most associated with hypertensive or ischemic (54%) and chemical (28%) etiologies. Management was predominantly conservative (83%). Outcomes were poor in 56% of patients with mean follow-up of 8 months. Good recovery was significantly higher in the traumatic than in the non-traumatic group (48% vs. 17%, p=0.019). BBGHs are rare occurrences with dismal prognoses. Standard management follows that of current intracerebral hemorrhage guidelines with supportive care and early blood pressure management. Minimally invasive surgery is promising, though substantial evidence is required to outweigh the potentially increased risks of bilateral hematoma evacuation.

Concurrent IDH1 and IDH2 mutations in glioblastoma: A case report.

Isocitrate dehydrogenase (IDH) mutations are cornerstone diagnostic features in glioma classification. IDH mutations are typically characterized by mutually exclusive amino acid substitutions in the genes encoding for the IDH1 and the IDH2 enzyme isoforms. We report our institutional case of a diffuse astrocytoma with progression to secondary glioblastoma and concurrent IDH1/IDH2 mutations. A 49-year-old male underwent a subtotal resection of a lobular lesion within the right insula in 2013, revealing a WHO grade 3 anaplastic oligoastrocytoma, IDH1 mutated, 1p19q intact. Symptomatic tumor progression was suspected in 2018, leading to a surgical tumor biopsy that demonstrated WHO grade 4 IDH1 and IDH2 mutant diffuse astrocytoma. The patient subsequently underwent surgical resection followed by medical management and finally died in 2021. Although concurrent IDH1/IDH2 mutations have been rarely reported in the current literature, further study is required to better define their impact on patients' prognoses and their response to targeted therapies.

Social media insights for neurosurgical oncologists: a survey of the American Association of Neurological Surgeons and Congress of Neurological Surgeons Joint Section on Tumors.

PURPOSE: There is a paucity in the literature regarding the characteristics and attitudes of social media (SM) utilization in a professional manner by neurosurgical oncologists. METHODS: A 34-question electronic survey was created using Google Forms and disseminated via email to members of the AANS/CNS Joint Section on Tumors. Demographic data were compared amongst those who utilize social media versus those who do not. Factors associated with positive effects of professional SM use and with having more followers on SM were analyzed. RESULTS: The survey received 94 responses, of which 64.9% reported that they currently use SM in a professional manner. Age < 50 years was found to be associated with SM use (p = 0.038). Facebook (54.1%), Twitter (60.7%), Instagram (41%), and LinkedIn (60.7%) were the most used SM platforms. Having a higher number of followers was associated with practicing in academics (p = 0.005), using Twitter (p = 0.013), posting about their own research publications (p = 0.018), posting interesting cases (p = 0.022), and posting about upcoming events (p = 0.001). Having a higher number of followers on SM was also associated with positive effects, specifically new patient referrals (p = 0.04). CONCLUSION: Neurosurgical oncologists can benefit by using social media professionally for increased patient engagement and networking within the medical community. Practicing in academics, making use of Twitter, and posting about interesting cases, upcoming academic events, and one's own research publications can help gain followers. In addition, having a large following on social media could lead to positive effects such as new patient referrals.

Clinical Characteristics, Management, and Outcomes of Intramedullary Spinal Cord Ependymomas in Adults: A Systematic Review.

BACKGROUND: Intramedullary spinal cord ependymomas (IMSCEs) are rare tumors that mostly occur in adults. Management strategies and related outcomes are heterogeneously reported across the literature, demanding a comprehensive analysis to standardize guidelines. We performed a systematic review of the literature on IMSCEs. METHODS: A literature search was conducted using 6 databases from inception up to July 28, 2022. Studies with data on clinical characteristics, management strategies, and related outcomes in adult patients with histopathologically confirmed IMSCEs were pooled and analyzed. RESULTS: The analysis included 69 studies comprising 457 patients (52.7% males). Mean age was 42.4 ± 7.4 years. Sensory deficit (58.0%) was the most prevalent symptom, followed by radicular pain (50.5%). Tumors mostly involved the cervical (64.4%) or thoracic (18.8%) spinal cord and were mostly World Health Organization grade II (80.5%) and classic subtype (72.4%). Gross total resection was performed in most cases (83.4%), with adjuvant radiotherapy delivered in 10.5% of cases. Progression-free survival ≥2 years was reported in 61.1% of cases, and tumor recurrence or progression was reported in only 7.0% of the patients. At last follow-up, 97.4% of patients were alive. CONCLUSIONS: IMSCEs are uncommon tumors that frequently manifest with debilitating symptoms that require surgical treatment. When feasible, gross total resection may be pursued to improve the patient's functional status and prevent tumor progression, with adjuvant radiotherapy required only in some more aggressive grade III lesions. Future studies should investigate different growth patterns and prognoses based on different IMSCE subtypes.

Calcified spinal meningiomas: a systematic review of clinical characteristics, treatment strategies, and outcomes.

BACKGROUND: Calcified meningiomas involving the spine are rare but can pose significant surgical challenges. We systematically reviewed the literature on calcified spinal meningiomas. METHODS: PubMed, EMBASE, Web-of-Science, and Scopus databases were searched to include studies reporting clinical data of patients with calcified spinal meningioma. Included articles were analyzed for symptoms, imaging, spine level of the tumor, tumor location relative to the spinal cord, calcification status, treatment regimen, recurrence, progression-free survival, and outcomes. RESULTS: A total of 35 articles encompassing 94 patients were included. Most patients were female (90.4%), presenting with lower extremity weakness (44%) and/or lower extremity paresthesia (38.1%). Most calcified spinal meningiomas occurred in the thoracic spine (82%) and on the dorsal (33.3%) or ventral (27.2%) side relative to the spinal cord. Most tumors were intradural (87.2%). Histologically, most calcified spinal meningiomas were WHO grade I (97.4%) and psammomatous (50.7%). Most tumors demonstrated macroscopic calcification (48.9%). Most patients underwent gross total resection (91.5%) through a posterior approach (100%). Two patients (2.1%) received adjunctive radiotherapy. The most common treatment related complication was CSF leakage. Post-operatively, most patients demonstrated symptomatic improvement (75.5%) and 2 (2.1%) had local tumor recurrence. CONCLUSIONS: Calcified spinal meningiomas are uncommon but benign entities. These neoplasms tend to adhere to surrounding tissues and nerves and, thus, can be surgically challenging to remove. In most patients, safe gross total resection remains the standard of care, but accurate surgical planning is necessary to reduce the risks of postoperative complications.

Cesium-131 brachytherapy for the treatment of brain metastases: Current status and future perspectives.

Adjuvant radiotherapy is often necessary following surgical resection of brain metastases to improve local tumor control and survival. Brachytherapy using cesium-131 offers a novel method for loco-regional radiotherapy. We reviewed the current literature reporting the use of cesium-131 brachytherapy for the treatment of brain metastases. Published studies and ongoing trials were reviewed to identify treatment protocols and clinical outcomes of cesium-131 brachytherapy for brain metastases. Cesium-131 brachytherapy was further compared to current outcomes for iodine-125 brachytherapy and stereotactic radiosurgery. Intraoperative brachytherapy allows patients to receive two treatment modalities in one setting while minimizing tumor cell repopulation. After initial interest, the use of iodine-125 brachytherapy has declined due to unfavorable rates of radiation necrosis without survival improvement. Recent data on intracavitary cesium-131 brachytherapy in brain metastases have demonstrated improved locoregional tumor control with low risks of radiation necrosis, with associated improvements in patients compliance and satisfaction. Cesium-131 isotope has a short half-life, delivers 90% of its dose within a month, shortens the time to initiation of systemic therapy compared to iodine-125 or external radiotherapy, and has an excellent radiation safety profile. Further analyses have demonstrated superior cost-effectiveness and quality-of-life improvement ratios of cesium-131 brachytherapy than adjuvant stereotactic radiosurgery. Cesium-131 brachytherapy is a safe and effective post-surgical treatment option for brain metastases with associated clinical and cost-effectiveness benefits in appropriately selected patients.

Degenerative Spine Surgery in Patients with Parkinson Disease: A Systematic Review.

BACKGROUND: Parkinson disease (PD) has been recognized as responsible for concurrent spinal disorders. Surgical correction may be necessary, but the complexity of such fragile patients may require specific considerations. We systematically reviewed the literature on degenerative spine surgery in patients with PD. METHODS: PubMed, Scopus, Web of Science, and Cochrane were searched according to the PRISMA guidelines to include studies reporting clinical data of patients with PD undergoing degenerative spine surgery. Clinical characteristics, treatment protocols, and outcomes were analyzed. RESULTS: We included 22 articles comprising 442 patients (61.5% female). Mean age was 66.9 ± 3.5 years (range, 41-83 years). Mean PD duration and modified Hoehn and Yahr stage were 4.46 ± 2.39 years and 2.3 ± 0.8, respectively. Operation types included fusion (55.3%) and decompression (41.6%). Mean operated spine levels were 6.0 ± 5.08. A total of 377 postoperative complications occurred in 34.6% patients, categorized into mechanical failure (58.0%), infection (15.1%), or neurologic (10.7%). Of patients, 31.8% required surgical revisions, with an average of 1.88 ± 1.03 revisions per patient. The average normalized presurgery, postsurgery, and final aggregate numeric patient outcome scores were 0.37 ± 0.13, 0.63 ± 0.18, and 0.61 ± 0.19, respectively, with a score of 0 and 1 representing the worst and best possible score. CONCLUSIONS: Degenerative spine surgery in patients with PD is challenging, with complications and revisions occurring in up to a third of treated patients. Surgery should be offered when other treatment options have proved ineffective and is typically reserved for patients with myelopathy or significant disability. Successful outcomes depend on strong interdisciplinary support to control the movement disorder before and after surgery.

The role of radiation therapy in the treatment of spine metastases from hepatocellular carcinoma: a systematic review and meta-analysis.

OBJECTIVE: Spine hepatocellular carcinoma (HCC) metastases severely worsen quality of life and prognosis, with the role of radiotherapy being controversial. The authors systematically reviewed the literature on radiotherapy for spine metastatic HCCs. METHODS: The PubMed, Scopus, Web of Science, and Cochrane databases were searched according to the PRISMA guidelines to include studies of radiotherapy for spine metastatic HCCs. Outcomes, complications, and local control were analyzed with indirect random-effect meta-analyses. RESULTS: The authors included 12 studies comprising 713 patients. The median time interval from diagnosis of HCC to spine metastases was 12 months (range 0-105 months). Most lesions were thoracic (35.9%) or lumbar (24.7%). Radiotherapy was delivered with conventional external-beam (67.3%) or stereotactic (31.7%) techniques. The median dose was 30.3 Gy (range 12.5-52 Gy) in a median of 5 fractions (range 1-20 fractions). The median biologically effective dose was 44.8 Gy10 (range 14.4-112.5 Gy10). Actuarial rates of postradiotherapy pain relief and radiological response were 87% (95% CI 84%-90%) and 70% (95% CI 65%-75%), respectively. Radiation-related adverse events and vertebral fractures had actuarial rates of 8% (95% CI 5%-11%) and 16% (95% CI 10%-23%), respectively, with fracture rates significantly higher after stereotactic radiotherapy (p = 0.033). Fifty-eight patients (27.6%) had local recurrences after a median of 6.8 months (range 0.1-59 months), with pooled local control rates of 61.6% at 6 months and 40.8% at 12 months, and there were no significant differences based on radiotherapy type (p = 0.068). The median survival was 6 months (range 0.1-62 months), with pooled rates of 52.5% at 6 months and 23.4% at 12 months. CONCLUSIONS: Radiotherapy in spine metastatic HCCs shows favorable rates of pain relief, radiological responses, and local control. Rates of postradiotherapy vertebral fractures are higher after high-dose stereotactic radiotherapy.

Tumors Involving the Infratemporal Fossa: A Systematic Review of Clinical Characteristics and Treatment Outcomes.

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

Neoadjuvant Stereotactic Radiotherapy for Brain Metastases: Systematic Review and Meta-Analysis of the Literature and Ongoing Clinical Trials.

BACKGROUND: Brain metastases (BMs) carry a high morbidity and mortality burden. Neoadjuvant stereotactic radiotherapy (NaSRT) has shown promising results. We systematically reviewed the literature on NaSRT for BMs. METHODS: PubMed, EMBASE, Scopus, Web-of-Science, Cochrane, and ClinicalTrial.gov were searched following the PRISMA guidelines to include studies and ongoing trials reporting NaSRT for BMs. Indications, protocols, and outcomes were analyzed using indirect random-effect meta-analyses. RESULTS: We included 7 studies comprising 460 patients with 483 BMs, and 13 ongoing trials. Most BMs originated from non-small lung cell carcinoma (41.4%), breast cancer (18.7%) and melanoma (43.6%). Most patients had single-BM (69.8%) located supratentorial (77.8%). Patients were eligible if they had histologically-proven primary tumors and ≤4 synchronous BMs candidate for non-urgent surgery and radiation. Patients with primary tumors clinically responsive to radiotherapy, prior brain radiation, and leptomeningeal metastases were deemed non-eligible. Median planning target volume was 9.9 cm3 (range, 2.9-57.1), and NaSRT was delivered in 1-fraction (90.9%), 5-fraction (4.8%), or 3-fraction (4.3%), with a median biological effective dose of 39.6 Gy10 (range, 35.7-60). Most patients received piecemeal (76.3%) and gross-total (94%) resection after a median of 1-day (range, 1-10) post-NaSRT. Median follow-up was 19.2-months (range, 1-41.3). Actuarial post-treatment rates were 4% (95%CI: 2-6%) for symptomatic radiation necrosis, 15% (95%CI: 12-18%) and 47% (95%CI: 42-52%) for local and distant recurrences, 6% (95%CI: 3-8%) for leptomeningeal metastases, 81% (95%CI: 75-87%) and 59% (95%CI: 54-63%) for 1-year local tumor control and overall survival. CONCLUSION: NaSRT is effective and safe for BMs. Ongoing trials will provide high-level evidence on long-term post-treatment outcomes, further compared to adjuvant stereotactic radiotherapy.

Adenoid Cystic Carcinoma (ACC) Infiltrating the Skull Base: A Systematic Review of Clinical Characteristics and Management Strategies.

BACKGROUND/AIM: To systematically review the patient characteristics and management approaches of adenoid cystic carcinoma (ACC) infiltrating the skull base. MATERIALS AND METHODS: According to PRISMA guidelines, PubMed, Scopus, and Cochrane were searched to retrieve studies reporting management protocols and survival outcomes of patients with skull base ACCs. Patient characteristics, management strategies, and outcomes were investigated. RESULTS: The review encompassed 17 studies involving 171 patients, with a female predominance (57.9%) and a mean age of 49±7.12 years. ACCs mostly infiltrated the paranasal sinus (22.2%), cavernous sinus (8.8%), and nasopharynx (7.1%). Perineural invasion was reported in 6.4% of cases. Facial pain, nasal obstruction, and facial paresthesia were the most common symptoms. Surgical resection (45.6%) was favored over biopsy (12.2%). Employing the free flap technique (4.7%), surgical reconstruction of the bony defect after resection was performed using abdominal and anterior thigh muscle grafts in 1.8% of patients each. As adjuvant management, 22.8% of cases had radiotherapy and 14.6% received chemotherapy. Recurrence of skull base ACCs occurred in 26.9% of cases during a mean follow up-time of 30.8±1.8 months. CONCLUSION: Skull base ACCs pose a surgical challenge mainly due to their proximity to critical neurovascular structures and aggressive behavior. Surgical resection and radiotherapy are shown to be safe and effective treatment modalities. The dismal prognosis and limited data on non-surgical strategies highlight the need for further evaluation of the current management paradigm and upraising innovative therapies to improve patient mortality and quality of life.

Craniopharyngiomas Invading the Ventricular System: A Systematic Review.

BACKGROUND/AIM: Craniopharyngiomas involving the ventricular system are rare but pose significant surgical challenges. We systematically reviewed the literature on craniopharyngiomas invading the ventricles (CP-V). MATERIALS AND METHODS: PubMed, EMBASE, Scopus, Web of Science, and Cochrane were searched to include studies reporting clinical data of patients with CP-Vs. Clinico-radiological features, management, and treatment outcomes were analyzed. RESULTS: We included 73 studies encompassing 407 patients. Patients were mostly male (61.5%), presenting with headache (57.9%) and/or endocrine disorders (52.1%). CP-Vs mostly involved the third ventricle (96.3%), followed by the lateral ventricles (2.9%), and the fourth ventricle (1%). Tumors had cystic components in 59% of cases and were mostly adamantinomatous (70.8%). Open resection was performed in 232 cases (57%), mostly with trans-lamina terminalis (36.6%) and trans-callosal (31.9%) approaches. Endoscopic resection was performed in 169 cases (41.5%), mostly with trans-sphenoidal (74.6%) and transventricular (24.9%) approaches. Gross-total tumor resection was obtained in most cases (62.9%). Adjuvant radiotherapy was delivered in 22.8% cases. A total of 178 patients experienced persistent complications, mostly including diabetes insipidus (47.1%) and panhypopituitarism (12.7%), not significantly different after open versus endoscopic resection (p=0.117). Symptom improvement was obtained in 88% of cases. CP-Vs recurrences were reported in 94 patients (23.1%), with median progression-free survival of 13.5 months (range=0.5-252.0 months). Fifty-nine patients died (14.5%), with median overall survival of 32.0 months (range=0.5-252.0 months), significantly longer after endoscopic resection than open resection (p=0.019). CONCLUSION: CP-Vs are uncommon and challenging entities. Surgical resection is feasible, but patient-tailored selection of open/endoscopic approaches is necessary to achieve optimal outcomes and minimize complication risks.

Intradural Extramedullary Spinal Metastases from Non-neurogenic Primary Tumors: A Systematic Review.

BACKGROUND/AIM: Intradural extramedullary spinal metastases (IESMs) may severely affect quality-of-life of oncological patients. Several treatments are available but their impact on prognosis is unclear. We systematically reviewed the literature on IESMs of non-neurogenic origin. MATERIALS AND METHODS: PubMed, Ovid EMBASE, Scopus, and Web-of-Science were screened to include articles reporting patients with IESMs from non-neurogenic primary tumors. Clinico-radiological presentation, treatments, and outcomes were analyzed. RESULTS: We included 51 articles encompassing 130 patients of a median age of 62 years (range=32-91 years). The most common primary neoplasms were pulmonary (26.2%), renal (20%), and breast (13.8%) carcinomas. Median time interval from primary tumor to IESMs was 18 months (range=0-240 months). The most common symptoms were sensory (58.3%) and motor (54.2%) deficits. Acute cauda equina syndrome was reported in 29 patients (37.7%). Lesions were diagnosed at magnetic resonance imaging (93.3%), myelography (25%), or computed tomography (16.7%). All patients underwent decompressive laminectomy with tumor resection, partial (54.6%) more frequently than complete (43.1%). Adjuvant radiation (67.5%) and/or systemic (13.3%) therapies were administered. After treatment, most patients had symptom improvement (70.8%) and optimal radiological response (64.2%). Four patients experienced IESMs recurrences (3.1%) with median local tumor control of 14.5 months (range=0.1-36 months). Deaths occurred in 50% of patients, with median overall survival of 6.7 months (range=0.1-108 months). CONCLUSION: Patients with IESMs have significant tumor burden with poor prognoses. Resection and locoregional radiation may offer favorable clinico-radiological responses but are limited in achieving optimal local control and survival.

Surgical Management of Craniovertebral Junction Schwannomas: A Systematic Review.

BACKGROUND: Craniovertebral junction (CVJ) schwannomas are rare, with surgery and stereotactic radiosurgery (SRS) being effective yet challenging options. We systematically reviewed the literature on CVJ schwannomas. METHODS: PubMed, Scopus, Web-of-Science, and Cochrane were searched following the PRISMA statement to include studies reporting CVJ schwannomas. Clinical features, management, and outcomes were analyzed. RESULTS: We collected 353 patients from 101 included articles. Presenting symptoms were mostly neck pain (30.3%) and headache (26.3%), with most cranial neuropathies involving the XII (31.2%) and X (24.4%) nerves. Most tumors originated from C2 (30.9%) and XII (29.4%) nerves, being extracranial (45.1%) and intradural-extradural (44.2%). Erosion of C1-C2 vertebrae (37.1%), the hypoglossal canal (28.3%), and/or jugular foramen (20.1%) were noted. All tumors were operated, preferably with the retrosigmoid approach (36.5%), with the far-lateral approach (29.7%) or with the posterior approach and cervical laminectomy (26.9%), far-lateral approaches (14.2%), or suboccipital craniotomy with concurrent cervical laminectomy (14.2%). Complete tumor resection was obtained most frequently (61.5%). Adjuvant post-surgery stereotactic radiosurgery was delivered in 5.9% patients. Median follow-up was 27 months (range, 12-252). Symptom improvement was noted in 88.1% of cases, and cranial neuropathies showed improvement in 10.2%. Post-surgical complications occurred in 83 patients (23.5%), mostly dysphagia (7.4%), new cranial neuropathies (6.2%), and cerebrospinal fluid leak (5.9%). A total of 16 patients (4.5%) had tumor recurrence and 7 died (2%), with median overall survival of 2.7 months (range, 0.1-252). CONCLUSIONS: Microsurgical resection is safe and effective for CVJ schwannomas. Data on SRS efficacy and indications are still lacking, and its role deserves further evaluation.