1. Makarenko S, Alzahrani I, Karsy M, Deopujari C, Couldwell WT. Outcomes and surgical nuances in management of giant pituitary adenomas: a review of 108 cases in the endoscopic era. J Neurosurg. 2022;137(September):1-12. doi:10.3171/2021.10.jns21659
Pituitary adenomas are histologically benign lesions that are the third most common intracranial neoplasm. They account for 10%–25% of intracranial neoplasms, with a prevalence of 17%. A subgroup of these lesions that are ≥ 4 cm in maximum diameter are classified as giant pituitary adenomas. Estimated at 5%–10% of all pituitary lesions, these giant pituitary adenomas are particularly challenging to manage because surgery can be problematic in large lesions that grow beyond the sella turcica due to limited space and the proximity of key anatomical structures such as the optic nerves, chiasm, carotid arteries, and cranial nerves, as well as the normal pituitary gland. The lesions may present with symptoms of mass effect such as headaches, visual loss, and diplopia, but also symptoms resulting from inappropriate hormonal secretion.
The authors present a 108-patient, single-surgeon series of management of giant pituitary adenomas. They characterize the lesions and discuss surgical outcomes. They also outline how the surgical management paradigm has evolved with experience and illustrate surgical nuances utilized to treat these lesions with case examples.
Patients in this study underwent resection of a pituitary adenoma with stereotactic image guidance and use of microvascular Doppler probes when necessary to identify the carotid arteries and to guide the extent of bony resection. The approach was selected on a case-by-case basis: either transnasal microscopic transsphenoidal resection with or without assistance of an endoscope for visualization or a transcranial approach. In select cases, a combination of transsphenoidal and transcranial approaches was used to achieve GTR, which was defined as no visible tumor on follow-up imaging. Autologous fat or fascia lata graft was used in cases in which there was a high risk of postoperative CSF leak. Surgicel (Ethicon) was used to buttress the fat graft packing in a “sling technique” within the sphenoid sinus.
The mean preoperative tumor diameter in this cohort was 4.6, with a mean volume of 25.9 cm3. Ninety-seven patients underwent transsphenoidal approaches only, 3 underwent transcranial resection, and 8 patients underwent a combined approach. Gross-total resection was achieved in 42 patients. Tumor stability without a need for additional therapy was achieved in 77 patients, with 26 patients undergoing subsequent adjuvant radiotherapy. Among 100 patients with sufficient follow-up, 14 underwent adjuvant therapy—repeat operation and/or adjuvant radiation therapy—because of recurrence or tumor progression. Six patients with recurrence were observed without additional treatment. Overall, the morbidity associated with removal of these lesions was 11.1%; the most common morbidities were cerebrospinal fluid leak (5 patients, 4.6%) and hydrocephalus (4 patients, 3.7%).
They conclude that giant pituitary tumors still represent a surgical challenge, with significant morbidity. Gross-total resection occurs in a minority of patients. Surgical goals for removal of giant pituitary tumors should include attempts at removal of most tumor tissue to minimize the risk of residual tumor apoplexy by tailoring the approach along the major axis of the tumor.
7 figures with MR
2. Peng D, Yan M, Liu T, et al. Prognostic Factors and Treatments Efficacy in Spontaneous Spinal Epidural Hematoma. Neurology. 2022;99(8):e843-e850. doi:10.1212/WNL.0000000000200844
Spontaneous spinal epidural hematoma (SSEH) is a relatively uncommon but potentially devastating disorder. Patients generally present with sudden onset acute back or neck pain, followed by progressive motor, sensory symptoms, or sphincter dysfunction, which ultimately leads to complete/incomplete motor deficit caused by spinal cord/nerve root compression or cauda equina. Once the diagnosis is suspected, early surgical intervention should be considered. Although there is no consensus on the ideal timing for surgery, most authors consider that surgery performed within 12 hours after the onset of symptoms is associated with better outcomes. Owing to the relative rarity of SSEH, many of these reports were gleaned from single-center data with limited sample sizes. The clinical implication of these studies is therefore might be limited. A recent individual patient data meta-analysis from the published literature enrolled 617 patients and identified that severity of preoperative neurologic deficit and use of anticoagulants determine the postoperative outcomes, but not the time interval between symptom onset and surgery.
A total of 105 patients with SSEH were retrieved from medical records, with a mean age of 51.3 years. Eighty-three patients (79%) complained of acute onset of severe neck or back pain. Eighty-two patients (78%) suffered from moderate to severe neurologic deficits. Anticoagulation usage was found in 20% of cases. Lower thoracic spine, use of anticoagulants, sphincter function disfunction, severe neurologic deficits at admission, and rapid deterioration (<1 hour) were found to be associated with poor outcomes. Surgical decompression was performed in 74 (70%) cases.
They conclude that preoperative neurologic deficit and extended paraplegia time were strongly correlated with the prognosis in the subset of patients who underwent surgical decompression. Timely surgical decompression is recommended for patients with moderate/severe neurologic deficits or progressive neurologic deterioration.
3 tables, 1 figure, no imaging
3. Neth BJ, Carabenciov ID, Ruff MW, Johnson DR. Temporal Trends in Glioblastoma Survival. The Neurologist. 2022;27(3):119-124. doi:10.1097/NRL.0000000000000393
Glioblastoma is the most common primary CNS malignancy with a current median overall survival (OS) of ~15 months, ranking near the worst prognoses of all cancer. Historical management has focused on surgical resection and radiation therapy (RT) alone, with little additional progress until 2005 with the FDA approval of temozolomide (TMZ) as a first-line chemotherapy in combination with RT and maximal surgical resection, leading to a population-level survival benefit.
Multiple analyses have also documented a subsequent improvement in survival several years after TMZ was fully integrated into the standard of care. The reasons for this later improvement well after the FDA approval of TMZ are less clear, with hypotheses including use of bevacizumab (BEV), incremental improvements in RT and surgical techniques, and better quality of supportive care. In recent years, there have been further advances in GBM management such as the FDA approval of tumor-treating fields (TTF) (TT Fields represent a non-invasive anticancer modality consisting of low-intensity, intermediate-frequency, alternating electric fields delivered via cutaneous transducer arrays configured to provide optimal tumor-site coverage), continued advancements to RT, and surgical techniques with the tumor visualizing agent 5-ALA.
The authors evaluated population-based data (2000-2017) from the Survival, Epidemiology, and End Results (SEER) program of the National Cancer Institute (NCI) to identify recent trends in GBM survival.
A total of 38,352 patients diagnosed with GBM between 2000 and 2017 met inclusion criteria. Median survival and percent survival to 12 and 24 months all progressively increased between 2000 and 2011. There were no significant differences in survival comparing 2009-2011 with 2012-2014 or 2015-2017. During the 2015-2017 period, median survival was 11 months.
After a period of progressive improvement in GBM survival between 2000 and 2011, survival plateaued. Subsequent advances since 2011 have not yet been translated to improved survival on the population-level as of 2017. As the most common primary CNS malignancy, GBM continues to have dismal survival despite persistent efforts in management and therapeutic development. After modest gains in short-term survival after the introduction of TMZ and BEV, there has been negligible survival improvement since 2011.
2 figures, 3 tables, no imaging
4. Winegar BA. Imaging of Painful Ophthalmologic Disorders. Neurol Clin. 2022;40(3):641-660. doi:10.1016/j.ncl.2022.03.002
Nice pictorial review with 21 figures. Always disconcerting to see a diagnosis that you have never made in 35+ years of neuroradiology…meaning that I don’t know about this pathology. Hence, the following: Inflammation of the optic nerve sheath is termed optic perineuritis. Most cases are a form of idiopathic orbital inflammation but may be secondary to a variety of systemic diseases such as sarcoidosis, IgG4-related disease, Behcet’s disease, granulomatosis with polyangiitis, and systemic lupus erythematosus or infections (eg, tuberculosis, syphilis, herpes simplex virus, herpes zoster virus). Symptoms of optic perineuritis may mimic optic neuritis, including pain, typically exacerbated by eye movement, and variable degree of vision loss. As opposed to optic neuritis, optic perineuritis is not self-limited and requires corticosteroids for treatment. On MRI, contrast enhancement and inflammation are centered on the optic nerve sheath, resulting in “tramtrack” appearance on axial images.
5. Tang LL, Guo R, Zhang N, et al. Effect of Radiotherapy Alone vs Radiotherapy With Concurrent Chemoradiotherapy on Survival Without Disease Relapse in Patients With Low-risk Nasopharyngeal Carcinoma. JAMA. 2022;328(8):728. doi:10.1001/jama.2022.13997
Concurrent chemoradiotherapy has been the standard treatment for stage II nasopharyngeal carcinoma (NPC) based on data using 2-dimensional conventional radiotherapy. There is limited evidence for the role of chemotherapy with use of intensity-modulated radiation therapy (IMRT). The purpose of this clinical trial was to determine if the omission of concurrent chemotherapy (ie, treatment with IMRT alone) was noninferior to treatment with concurrent chemoradiotherapy in patients with low-risk NPC. For this trial, low risk was defined as stage II or T3N0M0 disease without adverse features.
In this randomized clinical trial that included 341 participants with low-risk nasopharyngeal carcinoma who received IMRT alone vs concurrent chemoradiotherapy, the 3-year failure-free survival rate was 90.5%vs 91.9%, respectively. The difference met the noninferiority margin criterion of 10%.
3 figures, 2 tables
6. Wingerchuk DM, Lucchinetti CF. Neuromyelitis Optica Spectrum Disorder. Ropper AH, ed. New England Journal of Medicine. 2022;387(7):631-639. doi:10.1056/NEJMra1904655
NMOSD accounts for 1 to 2% of all cases of CNS inflammatory demyelinating disease in the United States and Europe, with multiple sclerosis being much more common, but NMOSD accounts for one third or more of cases of CNS inflammation in Asian and other non-White populations.
Six core clinical syndromes have been identified in NMOSD. They are classified by their location: optic nerve, spinal cord, area postrema of the dorsal medulla, other brain-stem regions, diencephalon, or cerebrum. These syndromes are manifested as acute attacks and relapses of neurologic dysfunction, with symptoms that typically evolve over a period of days. The most common syndromes are optic neuritis and transverse myelitis, as well as area postrema syndrome, which causes intractable or repetitive vomiting and hiccups. Multifocal lesions may result in a simultaneous or rapidly sequential combination of syndromes (e.g., optic neuritis and myelitis).
The sentinel attack involves the optic nerve or spinal cord in more than 85% of affected adults. Patients with optic neuritis present with unilateral or bilateral visual loss or scotoma, dyschromatopsia, and ocular pain exacerbated by eye movement that is not distinguishable from optic neuritis in multiple sclerosis or from an idiopathic form. Acute transverse myelitis causes limb weakness, numbness, sensory loss or pain below the lesion level, and bladder and bowel dysfunction.
Confirmation that an attack is due to NMOSD is greatly aided by the detection on MRI of characteristic lesions in the brain, optic nerve, optic chiasm, or spinal cord. In the acute phase, T2-weighted MRI sequences may show new or enlarging inflammatory lesions, and T1-weighted sequences obtained with gadolinium may show enhancement of actively inflamed lesions. Abnormalities on orbital MRI have a predilection for the optic chiasm or the adjacent posterior optic nerve but may occupy the full length of the nerve. MRI of the spinal cord typically shows longitudinally extensive transverse myelitis, defined as a lesion that is at least three contiguous vertebral segments in length.
1 figure, 2 tables, with MR imaging
7. Diener HC, Easton JD, Hart RG, Kasner S, Kamel H, Ntaios G. Review and update of the concept of embolic stroke of undetermined source. Nat Rev Neurol. 2022;18(August):455-465. doi:10.1038/s41582-022-00663-4
The definition of cryptogenic stroke in the Trial of Org 10172 in Acute Stroke Treatment (TOAST) classification (the most widely used classification system for ischemic stroke) and other classification schemes of stroke etiology includes three distinct groups of patients. The first is patients in whom diagnostic evaluation is incomplete, precluding identification of the causative pathology. Typical examples include patients who die soon after hospital admission, leaving no time for diagnostic investigation, and patients with other terminal illnesses that render diagnostic evaluation and therapeutic decisions less important. The second is patients who are diagnosed with two or more co-existing potential causes of stroke. A typical example is a patient with atrial fibrillation and an atherosclerotic plaque that is located ipsilateral to the infarct and is causing high-degree stenosis. Approximately 5% of patients with ischemic stroke have multiple obvious stroke aetiologies. The third is patients in whom the recommended diagnostic work-up is completed but no cause of stroke is identified.
In the TOAST classification, large-artery atherosclerotic strokes are defined as being associated with either substantial (>50%) stenosis or occlusion of a major brain artery or branch cortical artery. This arbitrary definition is now considered a limitation of the TOAST classification, as it does not include thromboembolic strokes provoked by an unstable plaque causing <50% stenosis, commonly described as a non-stenotic plaque. Subsequent etiological classifications have attempted to address this limitation; for example, the Causative Classification System and the Atherosclerosis, Small vessel disease, Cardioembolism, Other, Dissection (ASCOD) classification both allow stenosis of <50% to contribute to the final etiological determination. The prevalence of supracardiac atherosclerosis — that is, non-stenotic atherosclerotic plaques in the carotid, vertebrobasilar or intracranial arteries, or in the aortic arch — is remarkably high among patients with embolic stroke of undetermined source (ESUS). Among these patients, aortic arch atherosclerosis is prevalent in 29% (complex in 8%), carotid atherosclerosis in 40%, vertebrobasilar atherosclerosis in 4% and intracranial atherosclerosis in 13%. The risk of recurrent stroke with each of these pathologies could differ so that optimal treatment might also differ.
2 tables and 1 information box, but no imaging
8. Gao P, Wang T, Wang D, et al. Effect of Stenting Plus Medical Therapy vs Medical Therapy Alone on Risk of Stroke and Death in Patients With Symptomatic Intracranial Stenosis. JAMA. 2022;328(6):534. doi:10.1001/jama.2022.12000
Prior randomized trials have generally shown harm or no benefit of stenting added to medical therapy for patients with symptomatic severe intracranial atherosclerotic stenosis, but it remains uncertain as to whether refined patient selection and more experienced surgeons might result in improved outcomes.
Multicenter, open-label, randomized, outcome assessor–blinded trial conducted at 8 centers in China. A total of 380 patients with transient ischemic attack or nondisabling, nonperforator (defined as nonbrainstem or non–basal ganglia end artery) territory ischemic stroke attributed to severe intracranial stenosis (70%-99%) and beyond a duration of 3 weeks from the latest ischemic symptom onset were recruited between March 5, 2014, and November 10, 2016, and followed up for 3 years.
The risk of stroke or death within 30 days or stroke in the qualifying artery territory beyond 30 days through 1 year occurred in 8.0% in the percutaneous transluminal angioplasty and stenting group vs 7.2% in the medical therapy alone group, a difference that was not statistically significant.
They conclude that among patients with transient ischemic attack or ischemic stroke due to symptomatic severe intracranial atherosclerotic stenosis, the addition of percutaneous transluminal angioplasty and stenting to medical therapy, compared with medical therapy alone, resulted in no significant difference in the risk of stroke or death within 30 days or stroke in the qualifying artery territory beyond 30 days through 1 year. The findings do not support the addition of percutaneous transluminal angioplasty and stenting to medical therapy for the treatment of patients with symptomatic severe intracranial atherosclerotic stenosis.
3 figures, 2 tables, no imaging
9. Anderson CS, Song L, Liu J. Therapeutic Strategies for Intracranial Atherosclerosis. JAMA. 2022;328(6):529-531
The China Angioplasty and Stenting for Symptomatic Intracranial Severe Stenosis (CASSISS) trial, was undertaken with special care to address perceived shortcomings of previous studies and allow an assessment of the effectiveness of the Wingspan stent combined with standard medical therapy vs standard medical therapy alone. The medical therapy included dual antiplatelet therapy, commenced 3 to 5 days prior to the procedure and continued for 90 days after, and guideline-recommended use of statins and antihypertensive therapy to manage stroke risk factors. Participating sites were carefully selected and narrow inclusion criteria were used to identify a potential “responder group” at low procedural risk: patients with TIA or nondisabling, non-perforator territory, ischemic stroke from severe isolated intracranial stenosis (70%-99%) that occurred between 3 weeks and 12 months prior to randomization. Rigorous performance, training, and quality control procedures were used, and efforts were made toward achieving a high level of patient follow-up, with blinded and objective outcome assessments over 3 years.
Despite the efforts to improve patient selection and operator experience, the CASSISS trial redemonstrated that any potential benefit of stenting for ICAS in terms of long-term stroke prevention appears to be at least counterbalanced by the short-term procedural risk, which includes both ischemic and hemorrhagic events. Although arguments can be made that the restrictive inclusion criteria inevitably led to a biased, stable, “lower-risk” patient group, the trial further highlights the importance of best medical management of cardiovascular risk factors, in particular, use of statins and blood pressure– lowering agents to achieve guideline recommended targets in this patient population.
No figures
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