Giant unruptured middle cerebral artery aneurysm revealed by intracranial hypertension: is a systematic decompressive hemicraniotomy mandatory?

Our study aimed to evaluate the postoperative outcome of patients with unruptured giant middle cerebral artery (MCA) aneurysm revealed by intracranial hypertension associated to midline brain shift. From 2012 to 2022, among the 954 patients treated by a microsurgical procedure for an intracranial aneurysm, our study included 9 consecutive patients with giant MCA aneurysm associated to intracranial hypertension with a midline brain shift. Deep hypothermic circulatory flow reduction (DHCFR) with vascular reconstruction was performed in 4 patients and cerebral revascularization with aneurysm trapping was the therapeutic strategy in 5 patients. Early (< 7 days) and long term clinical and radiological monitoring was done. Good functional outcome was considered as mRS score ≤ 2 at 3 months. The mean age at treatment was 44 yo (ranged from 17 to 70 yo). The mean maximal diameter of the aneurysm was 49 mm (ranged from 33 to 70 mm). The mean midline brain shift was 8.6 mm (ranged from 5 to 13 mm). Distal MCA territory hypoperfusion was noted in 6 patients. Diffuse postoperative cerebral edema occurred in the 9 patients with a mean delay of 59 h and conducted to a postoperative neurological deterioration in 7 of them. Postoperative death was noted in 3 patients. Among the 6 survivors, early postoperative decompressive hemicraniotomy was required in 4 patients. Good functional outcome was noted in 4 patients. Complete aneurysm occlusion was noted in each patient at last follow-up. We suggest to discuss a systematic decompressive hemicraniotomy at the end of the surgical procedure and/or a partial temporal lobe resection at its beginning to reduce the consequences of the edema reaction and to improve the postoperative outcome of this specific subgroup of patients. A better intraoperative assessment of the blood flow might also reduce the occurrence of the reperfusion syndrome.

Microsurgical management of recurrent intracranial aneurysm after endovascular treatment: a series of 60 consecutive patients.

OBJECTIVE: The aim of this study was to evaluate the morbidity associated with microsurgical treatment in patients with a recurrent aneurysm to improve their surgical management. METHODS: From 2012 to 2022, among the 3128 patients with ruptured or unruptured intracranial aneurysms managed at the authors' institution, 954 patients were treated by a microsurgical procedure. Of these 3128 patients, 60 consecutive patients (6.3%) who had a recurrent microsurgically treated aneurysm after previous endovascular treatment were included in this study. Additional microsurgical treatment was considered in case of progressive remnant growth or significant aneurysm recurrence. Intraoperative and postoperative complications were noted. Early (< 7 days) and long-term clinical and radiological monitoring were performed. Good functional outcome was considered as a modified Rankin Scale score < 3. RESULTS: The mean age at initial treatment was 45 years (range 26-65 years). The mean delay between the first treatment and microsurgical treatment of the recurrence was 64 months (range 2 days-296 months). The mean size of the fundus recurrence was 5 mm, and the mean size of the neck recurrence was 4.6 mm. Five patients (8.3%) presented with subarachnoid hemorrhage associated with rupture of the recurrent aneurysm. Three patients died (6%) of aneurysm rupture and/or intensive care complications. The total morbidity rate associated with the microsurgical procedure was 14.5% (8/55) in patients with unruptured recurrent aneurysms. Among these patients, postoperative definitive complications (ischemic lesions) directly related to the microsurgical procedure were present in 3 patients (5.5%). Intraoperative rupture was recorded in these 3 patients. In the 54 surviving patients with unruptured recurrent aneurysms, good functional outcome was noted in 49 (91%). Poor functional outcome was significantly associated with intraoperative rupture. CONCLUSIONS: Microsurgery remains an effective therapeutic option for recurrent intracranial aneurysms. However, in the authors' experience, postoperative morbidity is higher than in patients with nonrecurrent aneurysms. Therefore, a pretherapeutic multidisciplinary evaluation is mandatory to reduce the potential morbidity associated with the retreatment as much as possible. When endovascular occlusion of the aneurysm requires both stenting and coiling, alternative microsurgical treatment should be carefully evaluated, as microsurgical clipping will become much more challenging in cases of aneurysm recurrence.

CNS tumors with PLAGL1-fusion: beyond ZFTA and YAP1 in the genetic spectrum of supratentorial ependymomas.

A novel methylation class, "neuroepithelial tumor, with PLAGL1 fusion" (NET-PLAGL1), has recently been described, based on epigenetic features, as a supratentorial pediatric brain tumor with recurrent histopathological features suggesting an ependymal differentiation. Because of the recent identification of this neoplastic entity, few histopathological, radiological and clinical data are available. Herein, we present a detailed series of nine cases of PLAGL1-fused supratentorial tumors, reclassified from a series of supratentorial ependymomas, non-ZFTA/non-YAP1 fusion-positive and subependymomas of the young. This study included extensive clinical, radiological, histopathological, ultrastructural, immunohistochemical, genetic and epigenetic (DNA methylation profiling) data for characterization. An important aim of this work was to evaluate the sensitivity and specificity of a novel fluorescent in situ hybridization (FISH) targeting the PLAGL1 gene. Using histopathology, immunohistochemistry and electron microscopy, we confirmed the ependymal differentiation of this new neoplastic entity. Indeed, the cases histopathologically presented as "mixed subependymomas-ependymomas" with well-circumscribed tumors exhibiting a diffuse immunoreactivity for GFAP, without expression of Olig2 or SOX10. Ultrastructurally, they also harbored features reminiscent of ependymal differentiation, such as cilia. Different gene partners were fused with PLAGL1: FOXO1, EWSR1 and for the first time MAML2. The PLAGL1 FISH presented a 100% sensitivity and specificity according to RNA sequencing and DNA methylation profiling results. This cohort of supratentorial PLAGL1-fused tumors highlights: 1/ the ependymal cell origin of this new neoplastic entity; 2/ benefit of looking for a PLAGL1 fusion in supratentorial cases of non-ZFTA/non-YAP1 ependymomas; and 3/ the usefulness of PLAGL1 FISH.