Supratotal Surgical Resection for Low-Grade Glioma: A Systematic Review.

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.

An interdisciplinary consensus on the management of brain metastases in patients with renal cell carcinoma.

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.

A case of multiple synchronously diagnosed brain metastases from alveolar soft part sarcoma without concurrent lung involvement.

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.

Bilateral Recurrent Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease) in Cowden Syndrome: A Case Report and Literature Review.

BACKGROUND: 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. CASE DESCRIPTION: 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. CONCLUSIONS: 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.

Multiple lesions of skull and cervical spine: a rare presentation of unicameral bone cysts.

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. ß-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.

BRAF-Mutated Pleomorphic Xanthoastrocytoma of the Spinal Cord with Eventual Anaplastic Transformation.

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.