Low-grade gliomas (LGGs) are optimally treated with up-front maximal safe surgical resection, typically defined as maximizing the extent of tumor resection while minimizing neurologic risks of ...surgery. Supratotal resection of LGG may improve outcomes beyond gross total resection by removing tumor cells invading beyond the tumor border as defined on MRI. However, the evidence regarding supratotal resection of LGG, in terms of impact on clinical outcomes, such as overall survival and neurologic morbidities, remains unclear. Authors independently searched the PubMed, Medline, Ovid, CENTRAL (Cochrane Central Register of Controlled Trials), and Google Scholar databases for studies evaluating overall survival, time to progression, seizure outcomes, and postoperative neurologic and medical complications of supratotal resection/FLAIRectomy of WHO-defined LGGs. Papers in languages other than English, lacking full-text availability, evaluating supratotal resection of WHO-defined high-grade gliomas only, and nonhuman studies were excluded. After literature search, reference screening, and initial exclusions, 65 studies were screened for relevancy, of which 23 were evaluated via full-text review, and 10 were ultimately included in the final evidence review. Studies were evaluated for quality using the MINORS criteria. After data extraction, a total of 1301 LGG patients were included in the analysis, with 377 (29.0%) undergoing supratotal resection. The main measured outcomes were extent of resection, pre- and postoperative neurological deficits, seizure control, adjuvant treatment, neuropsychological outcomes, ability to return to work, progression-free survival, and overall survival. Overall, low- to moderate-quality evidence was supportive of aggressive, functional boundary-based resection of LGGs due to improvements in progression-free survival and seizure control. The published literature provides a moderate amount of low-quality evidence supporting supratotal surgical resection along functional boundaries for low-grade glioma. Among patients included in this analysis, the occurrence of postoperative neurological deficits was low, and nearly all patients recovered within 3 to 6 months after surgery. Notably, the surgical centers represented in this analysis have significant experience in glioma surgery in general, and supratotal resection specifically. In this setting, supratotal surgical resection along functional boundaries appears to be appropriate for both symptomatic and asymptomatic low-grade glioma patients. Larger clinical studies are needed to better define the role of supratotal resection in LGG.
A 55-year-old man with a history of Benign Paroxysmal Positional Vertigo unalleviated by Epley manoeuvre presented to an otolaryngologist for dizziness, right ear fullness and headache. MRI of the ...brain showed numerous marrow-replacing lesions throughout the calvarium, skull base and upper cervical spine which were hypointense on T1-weighted images, hyperintense on T2-weighted images and avidly enhanced following contrast, concerning for a malignant process such as metastatic disease or multiple myeloma (figure 1). Systemic X-ray survey (spine, skull, chest, pelvis, all long bones) and nuclear medicine whole body bone scan were negative except for the lesions seen on MRI. β−2microglobin, immunoglobin and monoclonal protein electrophoresis were negative for myeloma or immunological process. Given the concern for metastatic disease, biopsy of a skull lesion was recommended. Pathological analysis of a calvarial lesion was consistent with unicameral bone cyst (figure 1). No ongoing therapy was offered; however, brain and spine surveillance imaging will continue.
Brain metastases are a challenging manifestation of renal cell carcinoma. We have a limited understanding of brain metastasis tumor and immune biology, drivers of resistance to systemic treatment, ...and their overall poor prognosis. Current data support a multimodal treatment strategy with radiation treatment and/or surgery. Nonetheless, the optimal approach for the management of brain metastases from renal cell carcinoma remains unclear. To improve patient care, the authors sought to standardize practical management strategies. They performed an unstructured literature review and elaborated on the current management strategies through an international group of experts from different disciplines assembled via the network of the International Kidney Cancer Coalition. Experts from different disciplines were administered a survey to answer questions related to current challenges and unmet patient needs. On the basis of the integrated approach of literature review and survey study results, the authors built algorithms for the management of single and multiple brain metastases in patients with renal cell carcinoma. The literature review, consensus statements, and algorithms presented in this report can serve as a framework guiding treatment decisions for patients. CA Cancer J Clin. 2022;72:454‐489.
INTRODUCTION:
Approximately two-thirds of all cancer patients will develop bone metastasis, and the spine is the most common site.
1
Spine metastases may lead to significant pain, instability, and/or ...neurological morbidity. A high rate of local control can be achieved with local therapies such as radiation and surgery, but persistent pain is described among some patients. Prior clinical research demonstrated that arterial embolization without surgery may improve pain associated with spine metastases.2 This study aimed to determine the effects of embolization on pain in patients undergoing surgical decompression and stabilization.
METHODS:
A retrospective single-center review between 2012 and 2020 identified 117 patients receiving adjuvant SRS or SBRT following surgical intervention for spine metastases. The study group included various primary tumor types and patients treated with laminectomy and/or varying degrees of vertebrectomy. Data analyzed included demographic information, embolization and surgical details, KPS, defensive veterans pain rating scale (DVPRS), dose of radiotherapy, and daily doses of analgesic medications.
RESULTS:
Of 117 patients, 47 (40.2%) underwent preoperative embolization. Forty-nine (70%) and 24 (51.1%) patients underwent laminectomy/partial vertebrectomy in the non-embolization and embolization cohorts, respectively. Demographics were similar between groups at baseline. DVPRS scores did not differ by primary tumor histopathology (p = 0.63). DVPRS daily average and maximum rest scores decreased from 5.6 to 3.7, p < 0.001, and 5.7 to 3.8, p < 0.001, respectively following embolization. Throughout postoperative days 1-14, pain scores and daily doses of analgesic medications were largely similar between cohorts.
CONCLUSIONS:
Preoperative arterial embolization decreases pain scores immediately after the procedure, consistent with prior literature. This palliative effect largely disappears in the postoperative period, likely due to surgical pain. Future prospective studies identifying long-term palliative effects of embolization for spine metastasis are needed.
Dysplastic gangliocytoma (Lhermitte-Duclos disease LDD) typically presents as a PTEN (phosphatase and tensin homolog)-positive, insidious unilateral mass of the cerebellar cortex. Patients can ...present with symptoms of increased intracranial pressure. Magnetic resonance imaging (MRI) will reveal a characteristic laminar/tigroid appearance. Surgical management has been superior to conservative measures for symptomatic lesions. The outcomes for bilateral craniotomy have not yet been described.
A 50-year-old patient with Cowden syndrome had initially presented with vertigo and imbalance. T2-weighted MRI showed a striated pattern of hypointensity and hyperintensity in bilateral cerebellar hemispheres consistent with Lhermitte-Duclos disease. He underwent right posterior fossa craniotomy with near total surgical resection and achieved symptom resolution. However, 3 years later, asymptomatic recurrence of the right-sided LDD and progression of left-sided LDD were noted. These were managed with a 1-year course of temozolomide. Both lesions remained stable until 4 years after completion of temozolomide. MRI demonstrated progression in the left hemisphere requiring a second surgical resection. PTEN analysis of the resected lesion was negative for mutation.
To the best of our knowledge, the present case is the first reported case of bilateral cerebellar hemisphere LDD ultimately managed by temporally dissociated bilateral surgical resections. Our patient was unique in that he had undergone surgery for resection of LDD, followed by a second surgery for contralateral progression 8 years later. The therapeutic options to prevent recurrence are limited, although temozolomide did seem to impede progression. Symptomatic patients will typically benefit most from surgical intervention. Given the strong association between adult LDD and Cowden syndrome, maintaining close follow-up care and possible surveillance imaging will be essential.
INTRODUCTION: Neurofibromatosis type 2 (NF2) patients develop spinal neoplasms. Determining indications for spine surgery remains challenging, as localization of spine related symptoms can be ...confounded by intracranial or peripheral neuropathology. Additionally, patient selection must be balanced with pre-existing comorbidities NF2 incurs with the goal to maintain or improve quality of life. METHODS: Seventy-nine patients were enrolled retrospectively based upon NF2 diagnosis and radiographic presence of spine tumors from three tertiary academic centers. Demographic data, clinical findings, treatment course, and spine tumor pathology were collected for all patients. RESULTS: Forty-eight percent of patients received spine surgery (38/79, 48.1%). Patients undergoing spine surgery had lower age of symptom onset (15.4 vs. 25.6, p = 0.015), increased cervical (4.2 vs 2.4, p = 0.005) and overall spine tumor burden (10.3 vs 6.2, p < 0.001) and presence of neck (9 vs 1, p = 0.006) and radicular pain (8 vs 1, p = 0.012). Over a quarter of our patients received BEV (25/79, 31.6%), with majority experiencing symptom onset before age 20 (17/19, 89.5%). Schwannoma was the most common BEV-treated pathology. Only one patient experienced worsening of tumor burden at one year while on BEV, and five patients required spine surgery after receiving BEV. CONCLUSIONS: NF2 patients who require spine surgery typically present at younger ages with large cervical spine tumor burden and associated symptomology. It is crucial that surgery-associated demographics and symptomology are identified. BEV was utilized in patients with aggressive, early-onset spine disease. Further, BEV halted spine disease progression in all patients except one, with minimal additional spine surgery required. Its ability to slow aggressive, NF2-associated spine tumors should be considered for clinical trial.
Background Pleomorphic xanthoastrocytoma (PXA) is an uncommon, primary neoplasm of the central nervous system with a relatively favorable prognosis. Most patients are managed with surgery alone and ...experience significant long-term survival. PXAs occur most commonly along the superficial surfaces of the temporal lobes. Although these tumors may occur in other regions of the brain, their origin within the spinal cord is rare, and it is unclear whether spinal cord PXAs should be managed differently from their intracranial counterparts. Case Description We describe a 31-year-old patient with a PXA of spinal cord origin who despite surgery, radiation, and multiple chemotherapy regimens experienced anaplastic transformation of his tumor and died of extensive leptomeningeal progression. Conclusions To our knowledge, our patient represents the seventh reported case of PXA of the spinal cord but is the first described to have a BRAF mutation. Specifically, both the initial and recurrent tumors of the patient showed the same BRAF V600E mutation, which refutes previous suggestions that BRAF mutations may be limited to intracranial PXAs and also shows that BRAF mutations may occur earlier in PXA tumorigenesis.
Background:
Alveolar soft part sarcoma (ASPS) is a rare soft-tissue sarcoma with a propensity for early hematogenous dissemination to the lungs and frequent brain metastasis. The development of lung ...metastasis almost invariably precedes intracranial involvement. There are no previously reported cases in which a patient was synchronously diagnosed with ASPS and multiple brain metastasis without lung involvement.
Case Description:
A 29-year-old gentleman was found to have three intracranial lesions following the onset of generalized seizures. Staging studies identified a soft-tissue mass in the left thigh and an adjacent femoral lesion. Biopsy of the soft-tissue mass was consistent with ASPS. The patient then underwent neoadjuvant stereotactic radiotherapy to all three brain lesions, followed by
en bloc
resection of the dominant lesion. The patient was then started on a programmed death-ligand 1 (PD-L1) inhibitor. Subsequent surgical resection of the primary lesion and femur metastasis demonstrates a histopathologic complete response of the bony metastasis and partial response of the primary lesion. At present, the patient has received 14 cycles of atezolizumab without recurrence of the primary or bony lesions and the irradiated intracranial disease has remained stable without recurrence of the resected dominant lesion.
Conclusion:
While intracranial involvement is relatively common in ASPS, a case with multiple, synchronously diagnosed brain metastasis without concurrent lung metastasis has not been described. The presented case discusses the safety and efficacy of aggressive management of intracranial disease in the setting of atezolizumab. Prospective evaluation of the efficacy of checkpoint inhibitors and the prognostic value of PD-L1 expression in ASPS with brain metastasis are necessary.
Traumatic brain injury (TBI) is a risk factor for the later development of neurodegenerative diseases that may have various underlying pathologies. Chronic traumatic encephalopathy (CTE) in ...particular is associated with repetitive mild TBI (mTBI) and is characterized pathologically by aggregation of hyperphosphorylated tau into neurofibrillary tangles (NFTs). CTE may be suspected when behavior, cognition, and/or memory deteriorate following repetitive mTBI. Exposure to blast overpressure from improvised explosive devices (IEDs) has been implicated as a potential antecedent for CTE amongst Iraq and Afghanistan Warfighters. In this study, we identified biomarker signatures in rats exposed to repetitive low-level blast that develop chronic anxiety-related traits and in human veterans exposed to IED blasts in theater with behavioral, cognitive, and/or memory complaints. Rats exposed to repetitive low-level blasts accumulated abnormal hyperphosphorylated tau in neuronal perikarya and perivascular astroglial processes. Using positron emission tomography (PET) and the
FAV1451 (flortaucipir) tau ligand, we found that five of 10 veterans exhibited excessive retention of
FAV1451 at the white/gray matter junction in frontal, parietal, and temporal brain regions, a typical localization of CTE tauopathy. We also observed elevated levels of neurofilament light (NfL) chain protein in the plasma of veterans displaying excess
FAV1451 retention. These findings suggest an association linking blast injury, tauopathy, and neuronal injury. Further study is required to determine whether clinical, neuroimaging, and/or fluid biomarker signatures can improve the diagnosis of long-term neuropsychiatric sequelae of mTBI.
African-American and Latino patients are often difficult to recruit for asthma studies. This challenge is a barrier to improving asthma care and outcomes for these populations.
We sought to examine ...the recruitment experiences of 8 asthma comparative effectiveness studies that specifically targeted African-American and Latino patients, and identify the solutions they developed to improve recruitment.
Case report methodology was used to gather and evaluate information on study design, recruitment procedures and outcomes from study protocols and annual reports, and in-depth interviews with each research team. Data were analyzed for themes, commonalities, and differences.
There were 4 domains of recruitment challenges: individual participant, institutional, research team, and study intervention. Participants had competing demands for time and some did not believe they had asthma. Institutional challenges included organizational policies governing monetary incentives and staff hiring. Research team challenges included ongoing training needs of recruitment staff, and intervention designs often were unappealing to participants because of inconveniences. Teams identified a host of strategies to address these challenges, most importantly engagement of patients and other stakeholders in study design and troubleshooting, and flexibility in data collection and intervention application to meet the varied needs of patients.
Asthma researchers may have greater success with recruitment by addressing uncertainty among patients about asthma diagnosis, engaging stakeholders in all aspects of study design and implementation, and maximizing flexibility of study and intervention protocols. However, even with such efforts, engagement of African-American and Latino patients in asthma research may remain low. Greater investment in research on engaging these populations in asthma research may ultimately be needed to improve their asthma care and outcomes.
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