Diffuse Midline H3K27-Altered Gliomas in the Spinal Cord: A Systematic Review.

PURPOSE: To systematically review the clinical features, management, and outcomes of diffuse midline H3K27-altered gliomas of the spinal cord (DMG-SCs). METHODS: PubMed, Ovid EMBASE, Scopus, and Web of Science were searched from database inception to 23 September 2023 for histologically confirmed cases of DMG-SC. Patient demographics, tumor characteristics, management information, and survival outcomes were extracted and analyzed. RESULTS: A total of 279 patients from 39 studies were collected. Patients were mostly male (61%), with an average age of 32 years. Patients were treated with surgery, radiotherapy, and chemotherapy combined (31%) or surgery only (24%), and extent of resection was most often subtotal (38%). Temozolomide was the most common chemotherapeutic agent (81%). Radiation therapy was delivered with mean dose of 47 Gy in 23 fractions. At mean follow-up time of 21 months, 13% of patients were alive. Average median overall survival was 24 months (range of 13 to 40 months) with a median progression-free survival of 14 months. Historical WHO grades of 2 or 3 appeared to exhibit a longer average median overall survival time than that of grade 4 DMG-SCs (32 vs. 23 months, p = 0.009). CONCLUSIONS: Outcomes for DMG-SCs are poor overall but appear to be favorable compared to intracranial DMGs. Despite the recent WHO 2021 grade 4 classification for all DMGs, given the differences in overall survival reported based on historical grading systems, future studies on DMG-SCs are needed to further define if DMG-SCs may represent a heterogeneous group of tumors with different prognoses.

Awake resection of recurrent astroblastoma with intraoperative 5-ALA-induced fluorescence: illustrative case.

BACKGROUND: Astroblastoma is a rare neoplasm characterized as a circumscribed glial neoplasm most often arising in the frontoparietal cerebral hemispheres in older children. OBSERVATIONS: We report an intriguing case of an astroblastoma recurrence 21 years after gross-total resection and radiation. A 32-year-old right-handed female presented to the emergency department for a generalized tonic-clonic seizure. She had a history of bipolar disorder, intractable migraines, and prior seizures linked to an astroblastoma previously resected three times. Magnetic resonance imaging on the current visit showed growth of the recurrent lesion to a 3.8-cm maximal diameter. Left-sided awake craniotomy was performed to remove the tumor while using speech mapping and 5-aminolevulinic acid (5-ALA). Targeted next-generation sequencing of the tumor revealed in-frame MN1::BEND2 fusion transcripts. LESSONS: We found that 5-ALA can be used in astroblastoma patients to assist in gross-total resection, which is important for long-term survival. Our astroblastoma case demonstrated classic astroblastoma morphology, with typical perivascular astroblastic rosettes, and was brightly fluorescent after 5-ALA administration.

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.

A Systematic Review of Primary Temporal Bone Mucosal Melanoma.

OBJECTIVE: Temporal bone mucosal melanomas (MMs) are rare, and patients may experience delays in diagnosis and treatment. Our objective was to better characterize the presentation, diagnosis, treatment modalities, and outcomes of this process. DATA SOURCES: PubMed/Medline, CINAHL (EBSCOhost), and Web of Science databases were searched in all languages without restriction of publication dates. STUDY SELECTION: Inclusion criteria included that the article was either a case report or a case series with individual case data. All non-English articles were excluded if the corresponding abstract lacked data on demographics, initial presentation, and clinical management. DATA EXTRACTION: After full-text analysis, data pertaining to demographics, diagnosis, medical and surgical management modalities, and outcomes were extracted. DATA SYNTHESIS: Data were qualitatively synthesized, and means and averages were obtained for all continuous variables. Overall survival was measured by the Kaplan-Meier method, and significance was measured through log-rank testing. CONCLUSIONS: Clinicians should suspect temporal bone MM in the differential diagnosis of patients with bloody otorrhea in the context of a chronic serous otitis media or an associated cranial nerve palsy. If suspected, physicians should not delay the acquisition of a biopsy or imaging studies. Management is highly variable and must be decided on a case-by-case basis. Outcomes remain poor because of the high propensity for MM to metastasize.

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.

Metastatic skull base chordoma: A systematic review.

Objective/Hypothesis: To investigate the clinical features, management strategies and outcomes for patients with metastatic primary skull base chordomas. Study Design: Systematic review. Methods: A systematic search through Pubmed/Medline, Web of Science, and EBSCOhost (CINAHL) was conducted without restriction on dates. After study screening and full-text assessment, two authors independently extracted all data using a pre-established abstraction form. Results: Forty cases were included from 38 studies. The average age (standard deviation [SD]) of the sample at presentation was 28.5 (23.3) and was equally distributed across genders. The average time (SD) between initial diagnosis to local recurrence was 40.1 (60.3) months. The average time (SD) from primary tumor detection to the diagnosis of metastatic disease was 55.2 (49.0) months. The most common subsite for metastatic spread were the lungs (32.5%). Of the 33 patients with data on outcomes, 48.5% were found to have expired by the time of publication. The median overall survival was estimated to be 84 months (95% confidence interval [CI] 62.3-105.7). Conclusions: The most common subsites for metastatic spread of skull base chordoma were the lungs and bone. Overall survival for patients in the current cohort was a median of 84 months, with no significant differences noted when stratifying by the extent of surgery or the site of metastases. Level of Evidence: 3a.

Cerebral toxoplasmosis in a patient with combined variable immunodeficiency.

Background: Cerebral toxoplasmosis is an opportunistic infection in patients but has rarely been described in the setting of compromised humoral immunodeficiency. Prompt diagnosis and treatment of the infection is critical in the care of these patients. Medical management is the mainstay of treatment of the infection. There have been very few reports of surgical management of cerebral toxoplasmosis. Case Description: We describe the case of a 40-year-old male who presented with headache, memory deficits, weight loss, and left-sided weakness in the setting of a known but undiagnosed brain lesion identified 1 month prior. Imaging demonstrated a right basal ganglia lesion which was initially presumed to be malignancy. On further workup including a positive serum test and biopsy including polymerase chain reaction analysis, diagnosis was confirmed as toxoplasmosis. On further investigation, he was found to have deficiencies in immunoglobulins consistent with common variable immunodeficiency (CVID). The patient underwent craniotomy with surgical debulking as repeat imaging showed increased size of mass with new satellite lesions and worsening hydrocephalus. Conclusion: Cerebral toxoplasmosis is an important differential to consider in cases of intracerebral lesions and should not necessarily be excluded in the absence of compromised cellular immunity. In cases where there is no immunocompromised state and malignancy cannot immediately be established, CVID should be considered as an etiology. Due to the subtlety of CVID diagnosis, careful attention should be paid to history taking and workup for CVID should be considered as soon as possible. Surgical removal of these lesions in conjunction with medications is an effective treatment option.

Intrathecal therapy for the management of leptomeningeal metastatic disease: a scoping review of the current literature and ongoing clinical trials.

PURPOSE: Leptomeningeal metastatic disease (LMD) from advanced malignancies has poor prognoses and limited treatments. Intrathecal therapy (ITT) protocols are available, showing variable outcomes. We reviewed the therapeutic and toxicity profiles of ITT in LMD. METHODS: PubMed, EMBASE, Web-of-Science, and Scopus were searched following the PRISMA-ScR guidelines to include studies reporting ITT for LMD. CLINICALTRIAL: gov and Cochrane were searched to identify ongoing clinical trials. RESULTS: We included 27 published studies encompassing 2161 patients and 4 ongoing trials. LMD originated from brain metastases (85.5%), lymphomas (5.4%), high-grade gliomas (4.6%), medulloblastomas (2.3%), and leukemias (2.1%). LMD was mostly diagnosed with the co-presence of neurological-related symptoms and positive imaging and/or cerebrospinal fluid cytology (60.8%). The most common ITT agents were methotrexate (35.9%), cytarabine (21.9%), and thiotepa (8.2%), standalone or combined. Patients received a median of 6.5 ITT cycles (range, 1.0-71.0) via intraventricular (58.8%) or lumbar intrathecal (41.2%) routes. The Ommaya reservoir was implanted in 38.5% cases. Concurrent systemic chemotherapy (45.2%) and/or radiotherapy (30.6%) were used. After 1-3 cycles, 44.7% patients had improved clinical status and 29.9% converted into negative cerebrospinal fluid cytology. The most common ITT-related severe adverse events were neutropenia (6.5%), meningitis (5.2%) and encephalopathy (4.5%). Median freedom from progression was 2.4 months (range, 0.1-59.5) and median overall survival 5.5 months (range, 0.1-148.0). CONCLUSION: Current ITT protocols are variable but effective and well-tolerated in LMD. Ongoing trials are investigating dose-limiting toxicity profiles and long-term overall survival. Future studies should analyze the therapeutic and safety profiles of ITT compared to newer systemic therapies.

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.

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.

The Role of [68Ga]Ga-DOTA-SSTR PET Radiotracers in Brain Tumors: A Systematic Review of the Literature and Ongoing Clinical Trials.

BACKGROUND: The development of [68Ga]Ga-DOTA-SSTR PET tracers has garnered interest in neuro-oncology, to increase accuracy in diagnostic, radiation planning, and neurotheranostics protocols. We systematically reviewed the literature on the current uses of [68Ga]Ga-DOTA-SSTR PET in brain tumors. METHODS: PubMed, Scopus, Web of Science, and Cochrane were searched in accordance with the PRISMA guidelines to include published studies and ongoing trials utilizing [68Ga]Ga-DOTA-SSTR PET in patients with brain tumors. RESULTS: We included 63 published studies comprising 1030 patients with 1277 lesions, and 4 ongoing trials. [68Ga]Ga-DOTA-SSTR PET was mostly used for diagnostic purposes (62.5%), followed by treatment planning (32.7%), and neurotheranostics (4.8%). Most lesions were meningiomas (93.6%), followed by pituitary adenomas (2.8%), and the DOTATOC tracer (53.2%) was used more frequently than DOTATATE (39.1%) and DOTANOC (5.7%), except for diagnostic purposes (DOTATATE 51.1%). [68Ga]Ga-DOTA-SSTR PET studies were mostly required to confirm the diagnosis of meningiomas (owing to their high SSTR2 expression and tracer uptake) or evaluate their extent of bone invasion, and improve volume contouring for better radiotherapy planning. Some studies reported the uncommon occurrence of SSTR2-positive brain pathology challenging the diagnostic accuracy of [68Ga]Ga-DOTA-SSTR PET for meningiomas. Pre-treatment assessment of tracer uptake rates has been used to confirm patient eligibility (high somatostatin receptor-2 expression) for peptide receptor radionuclide therapy (PRRT) (i.e., neurotheranostics) for recurrent meningiomas and pituitary carcinomas. CONCLUSION: [68Ga]Ga-DOTA-SSTR PET studies may revolutionize the routine neuro-oncology practice, especially in meningiomas, by improving diagnostic accuracy, delineation of radiotherapy targets, and patient eligibility for radionuclide therapies.

Laser Interstitial Thermal Therapy for Cerebral Cavernous Malformations: A Systematic Review of Indications, Safety, and Outcomes.

BACKGROUND: Cerebral cavernous malformations (CCM) in deep eloquent areas present a surgical challenge. Laser interstitial thermal therapy (LITT) may present itself as a safe minimally invasive treatment option. OBJECTIVE: To systematically review the indications, safety, and outcomes of LITT for CCM. METHODS: Electronic databases were searched from inception to October 7, 2021 for articles with CCM and LITT keywords. Studies describing CCMs treated with LITT were included. RESULTS: A total of 32 patients with CCMs in lobar (79%), basal ganglia (12%), and brainstem (9%) locations were treated with LITT. Indications for LITT included drug-resistant seizures (75%), unacceptable surgical risk (22%), recurrent hemorrhage (16%), and early intervention to discontinue antiepileptic drugs (3%). No death or CCM-associated intracranial hemorrhage occurred intraoperatively or postoperatively, and most patients experienced no adverse effects or transient effects that resolved at follow-up (84%). Of those treated for CCM-associated epilepsy, 83% experienced Engel class I seizure freedom and most were class IA (61%). Most patients experienced symptomatic improvement (93%), and a decrease in antiepileptic drugs was reported in more than half of patients (56%), with 28% able to discontinue all antiepilepsy medications after LITT. CONCLUSIONS: LITT seems to be a safe treatment for CCMs located in deep eloquent areas and in lesions presenting with medically refractory seizures or recurrent hemorrhages. Randomized studies are needed to further elucidate its efficacy in treating CCM.

Gliomas Infiltrating the Corpus Callosum: A Systematic Review of the Literature.

Background: Gliomas infiltrating the corpus callosum (G-I-CC) majorly impact patient quality-of-life, but maximally safe tumor resection is challenging. We systematically reviewed the literature on G-I-CC. Methods: PubMed, EMBASE, Scopus, Web of Science, and Cochrane were searched following the PRISMA guidelines to include studies of patients with G-I-CC. Clinicopathological features, treatments, and outcomes were analyzed. Results: We included 52 studies comprising 683 patients. Most patients experienced headache (33%), cognitive decline (18.7%), and seizures (17.7%). Tumors mostly infiltrated the corpus callosum genu (44.2%) with bilateral extension (85.4%) into frontal (68.3%) or parietal (8.9%) lobes. Most G-I-CC were glioblastomas (84.5%) with IDH-wildtype (84.9%) and unmethylated MGMT promoter (53.5%). Resection (76.7%) was preferred over biopsy (23.3%), mostly gross-total (33.8%) and subtotal (32.5%). The tumor-infiltrated corpus callosum was resected in 57.8% of cases. Radiation was delivered in 65.8% of patients and temozolomide in 68.3%. Median follow-up was 12 months (range, 0.1−116). In total, 142 patients (31.8%) experienced post-surgical complications, including transient supplementary motor area syndrome (5.1%) and persistent motor deficits (4.3%) or abulia (2.5%). Post-treatment symptom improvement was reported in 42.9% of patients. No differences in rates of complications (p = 0.231) and symptom improvement (p = 0.375) were found in cases with resected versus preserved corpus callosum. Recurrences occurred in 40.9% of cases, with median progression-free survival of 9 months (0.1−72). Median overall survival was 10.7 months (range, 0.1−116), significantly longer in low-grade tumors (p = 0.013) and after resection (p < 0.001), especially gross-total (p = 0.041) in patients with high-grade tumors. Conclusions: G-I-CC show clinicopathological patterns comparable to other more frequent gliomas. Maximally safe resection significantly improves survival with low rates of persistent complications.

Endocrine disorders after primary gamma knife radiosurgery for pituitary adenomas: A systematic review and meta-analysis.

PURPOSE: Gamma Knife radiosurgery (GKRS) is feasible for pituitary adenomas, but post-surgery GKRS may cause severe hormone deficits. We reviewed the literature on primary GKRS for pituitary adenoma focusing on radiation-induced hormone deficiencies. METHODS: PubMed, Web-of-Science, Scopus, and Cochrane were searched upon the PRISMA guidelines to include studies describing primary GKRS for pituitary adenomas. Pooled-rates of GKRS-induced hormone deficiencies and clinical-radiological responses were analyzed with a random-effect model meta-analysis. RESULTS: We included 24 studies comprising 1381 patients. Prolactinomas were the most common (34.2%), and 289 patients had non-functioning adenomas (20.9%). Median tumor volume was 1.6cm3 (range, 0.01-31.3), with suprasellar extension and cavernous sinus invasion detected in 26% and 31.1% cases. GKRS was delivered with median marginal dose 22.6 Gy (range, 6-49), maximum dose 50 Gy (range, 25-90), and isodose line 50% (range, 9-100%). Median maximum point doses were 9 Gy (range, 0.5-25) to the pituitary stalk, 7 Gy (range, 1-38) to the optic apparatus, and 5 Gy (range, 0.4-12.3) to the optic chiasm. Pooled 5 year rates of endocrine normalization and local tumor control were 48% (95%CI 45-51%) and 97% (95%CI 95-98%). 158 patients (11.4%) experienced endocrinopathies at a median of 45 months (range, 4-187.3) after GKRS, with pooled 5-year rates of 8% (95%CI 6-9%). GKRS-induced hormone deficiencies comprised secondary hypothyroidism (42.4%) and hypogonadotropic hypogonadism (33.5%), with panhypopituitarism reported in 31 cases (19.6%). CONCLUSION: Primary GKRS for pituitary adenoma may correlate with lower rates of radiation-induced hypopituitarism (11.4%) than post-surgery GKRS (18-32%). Minimal doses to normal pituitary structures and long-term endocrine follow-up are of primary importance.

Primary Paraganglioma of the Spine: A Systematic Review of Clinical Features and Surgical Management in Cauda Equina versus Non-Cauda Equina Lesions.

BACKGROUND: Primary spine paragangliomas are rare tumors. Surgical resection plays a role, but aggressive lesions are challenging. We reviewed the literature on primary spine paragangliomas. METHODS: PubMed, Scopus, Web of Science, and Cochrane were searched following the PRISMA guidelines to include studies on primary spine paragangliomas. Clinical-radiologic features, treatments, and outcomes were analyzed and compared between cauda equina versus non-cauda equina tumors. RESULTS: We included 143 studies comprising 334 patients. Median age was 46 years (range, 6-85 years). The most frequent symptoms were lower back (64.1%) and radicular (53.9%) pain, and sympathetic in 18 patients (5.4%). Cauda equina paragangliomas (84.1%) had frequently lumbar (49.1%) or lumbosacral (29%) locations. Non-cauda equina tumors were mostly in the thoracic (11.4%), thoracolumbar (5.1%), and cervical (3.6%) spine. Median tumor diameter was 2.5 cm (range, 0.5-13.0 cm). Surgical resection (98.5%) was preferred over biopsy (1.5%). Decompressive laminectomy (53%) and spine fusion (6.9%) were also performed. Adjuvant radiotherapy was delivered in 39 patients (11.7%) with aggressive tumors. Posttreatment symptomatic improvement was described in 86.2% cases. Median follow-up was 19.5 months (range, 0.1-468.0 months), and 23 patients (3.9%) had tumor recurrences. No significant differences were found between cauda equina versus non-cauda equina tumors. CONCLUSIONS: Surgical resection is effective and safe in treating primary spine paragangliomas; however, adjuvant treatments may be needed for aggressive lesions.

Metastases in the Pineal Region: A Systematic Review of Clinical Features, Management Strategies, and Survival Outcomes.

BACKGROUND: Pineal region metastases are rare but often cause severe neurologic deficits. Surgical resection and chemoradiotherapy can provide therapeutic benefit. We investigated the literature to analyze clinical characteristics, management strategies, and survival of adult patients with pineal region metastases. METHODS: PubMed, Embase, Scopus, and Cochrane were searched following the PRISMA guidelines, including studies reporting clinical outcomes of patients with pineal region metastases. Clinical presentation, management, and survival were reviewed. RESULTS: We included 31 studies comprising 47 patients. Lung cancer (29.8%) and carcinomas of unknown origin (14.9%) were the most frequent primary tumors. In 48.9% of patients, symptomatic pineal metastases preceded primary tumor diagnosis. Headache (67.4%) and confusion (46.5%) were the most common symptoms. Parinaud syndrome (46.5%) and hydrocephalus (87.2%) were noted. Biopsy (65.9%) was preferred over resection (34.1%), and shunting strategies used were endoscopic third ventriculostomy (43.9%) and ventriculoperitoneal (26.8%). Eleven patients (32.3%) received adjuvant chemotherapy and 32 (68%) received radiotherapy. Posttreatment improvement in symptoms (56.6%) and hydrocephalus (80.5%) were noted. In patients who received adjuvant chemotherapy/radiotherapy, significant improvement in posttreatment performance status occurred with both biopsy (P < 0.001) and resection (P = 0.007). No survival differences were reported between surgery and biopsy (P = 0.912) or between complete and partial resection (P = 0.220). Overall survival was neither influenced by surgical approach (P = 0.157) nor by shunting strategy (P = 0.822). Mean follow-up was 8 months and median overall survival 3 months. Only 2 cases (4.8%) of pineal metastasis showed recurrence. CONCLUSIONS: Pineal region metastases carry significant morbidity. Biopsy or surgical resection, combined with adjuvant chemotherapy/radiotherapy and/or shunting, may significantly improve performance status.

Biopsy Artifact in Laser Interstitial Thermal Therapy: A Technical Note.

PURPOSE: The safety and effectiveness of laser interstitial thermal therapy (LITT) relies critically on the ability to continuously monitor the ablation based on real-time temperature mapping using magnetic resonance thermometry (MRT). This technique uses gradient recalled echo (GRE) sequences that are especially sensitive to susceptibility effects from air and blood. LITT for brain tumors is often preceded by a biopsy and is anecdotally associated with artifact during ablation. Thus, we reviewed our experience and describe the qualitative signal dropout that can interfere with ablation. METHODS: We retrospectively reviewed all LITT cases performed in our intraoperative MRI suite for tumors between 2017 and 2020. We identified a total of 17 LITT cases. Cases were reviewed for age, sex, pathology, presence of artifact, operative technique, and presence of blood/air on post-operative scans. RESULTS: We identified six cases that were preceded by biopsy, all six had artifact present during ablation, and all six were noted to have air/blood on their post-operative MRI or CT scans. In two of those cases, the artifactual signal dropout qualitatively interfered with thermal damage thresholds at the borders of the tumor. There was no artifact in the 11 non-biopsy cases and no obvious blood or air was noted on the post-ablation scans. CONCLUSION: Additional consideration should be given to pre-LITT biopsies. The presence of air/blood caused an artifactual signal dropout effect in cases with biopsy that was severe enough to interfere with ablation in a significant number of those cases. Additional studies are needed to identify modifying strategies.

Primary Spontaneous Inferior Clival Cerebrospinal Fluid Leak.

Primary, spontaneous cerebrospinal fluid (CSF) leaks secondary to defects in the clivus are exceedingly rare. Additionally, primary, spontaneous CSF leaks are typically present in obese women with idiopathic intracranial hypertension (IIH). In the present study, we report the first case of a primary, spontaneous CSF leak in the inferior-posterior wall of the clivus in an atypical patient with a BMI of 18.9 kg/m2 without IIH. Accurate diagnoses of CSF leaks are imperative in the context of preventing meningitis, and delays in diagnosis and treatment are associated with worse outcomes. Improved characterization of rare, spontaneous CSF leaks may prove beneficial in correctly diagnosing affected patients.

Meningioma: A Review of Epidemiology, Pathology, Diagnosis, Treatment, and Future Directions.

Meningiomas are the most common intracranial tumor, making up more than a third of all primary central nervous system (CNS) tumors. They are mostly benign tumors that can be observed or preferentially treated with gross total resection that provides good outcomes. Meningiomas with complicated histology or in compromising locations has proved to be a challenge in treating and predicting prognostic outcomes. Advances in genomics and molecular characteristics of meningiomas have uncovered potential use for more accurate grading and prediction of prognosis and recurrence. With the study and detection of genomic aberrancies, specific biologic targets are now being trialed for possible management of meningiomas that are not responsive to standard surgery and radiotherapy treatment. This review summarizes current epidemiology, etiology, molecular characteristics, diagnosis, treatments, and current treatment trials.

Orbital Metastases: A Systematic Review of Clinical Characteristics, Management Strategies, and Treatment Outcomes.

BACKGROUND: Orbital metastases often lead to severe functional impairment. The role of resection, orbital exenteration, and complementary treatments is still debated. We systematically reviewed the literature on orbital metastases. METHODS: PubMed, Scopus, Web-of-Science, and Cochrane were searched upon PRISMA guidelines to identify studies on orbital metastases. Clinical characteristics, management strategies, and survival were analyzed. RESULTS: We included 262 studies comprising 873 patients. Median age was 59 years. The most frequent primary tumors were breast (36.3%), melanoma (10.1%), and prostate (8.5%) cancers, with median time interval of 12 months (range, 0-420). The most common symptoms were proptosis (52.3%) and relative-afferent-pupillary-defect (38.7%). Most metastases showed a diffuse location within the orbit (19%), with preferential infiltration of orbital soft tissues (40.2%). In 47 cases (5.4%), tumors extended intracranially. Incisional biopsy (63.7%) was preferred over fine-needle aspiration (10.2%), with partial resection (16.6%) preferred over complete (9.5%). Orbital exenteration was pursued in 26 patients (3%). A total of 305 patients (39.4%) received chemotherapy, and 506 (58%) received orbital radiotherapy. Post-treatment symptom improvement was significantly superior after resection (p = 0.005) and orbital radiotherapy (p = 0.032). Mean follow-up was 14.3 months, and median overall survival was 6 months. Fifteen cases (1.7%) demonstrated recurrence with median local control of six months. Overall survival was statistically increased in patients with breast cancer (p < 0.001) and in patients undergoing resection (p = 0.024) but was not correlated with orbital location (p = 0.174), intracranial extension (p = 0.073), biopsy approach (p = 0.344), extent-of-resection (p = 0.429), or orbital exenteration (p = 0.153). CONCLUSIONS: Orbital metastases severely impair patient quality of life. Surgical resection safely provides symptom and survival benefit compared to biopsy, while orbital radiotherapy significantly improves symptoms compared to not receiving radiotherapy.