Schwannomas are tumors that originate from myelinating Schwann cells and can occur in cranial, spinal, and peripheral nerves. Although our understanding of the molecular biology underlying schwannomas remains incomplete, numerous studies have identified various molecular findings and biomarkers associated with schwannomas of the central nervous system (CNS). The development of these tumors is primarily linked to mutations in the NF2 gene. Merlin, the protein encoded by NF2, is integral to several signaling pathways, including Ras/Raf/MEK/ERK, PI3K/Akt/mTORC1, Wnt/β-catenin, and the Hippo pathway. Recent research has also uncovered novel genetic alterations, such as the SH3PXD2A::HTRA1 fusion gene, VGLL-fusions in intraparenchymal CNS schwannomas, and the SOX10 mutation particularly in non-vestibular cranial nerve schwannomas. In addition to genetic alterations, research is also being conducted on gene expression and epigenetic regulation, with a focus on NF2 methylation and post-transcriptional silencing by micro RNA. Furthermore, the advent of advanced techniques like single-cell sequencing and multi-omics analysis has facilitated rapid discoveries related to the tumor microenvironment and tumor heterogeneity in schwannomas. A deeper exploration of these molecular findings could clarify the mechanisms of schwannoma tumorigenesis and progression, ultimately guiding the development of new therapeutic targets. This review offers a comprehensive overview of the current molecular understanding of CNS schwannomas, emphasizing the insights gained from previous research, while addressing existing controversies and outlining future research and treatment perspectives.
Ischemic complications can occur after revascularization surgery for moyamoya disease, but acute contralateral internal carotid artery (ICA) occlusion is an extremely rare complication. The patient was a 51-year-old woman with no medical history. Left frontal lobe infarction and bilateral ICA terminal stenosis were identified by repeated transient right paresis and aphasia. We diagnosed her with quasi-moyamoya disease associated with hyperthyroidism and performed revascularization surgery for the symptomatic left side. Although neurological symptoms did not worsen immediately after the surgery, disturbance of consciousness, right conjugate deviation, and left paresis appeared 4 hr after the surgery. New infarction appeared in the right frontal lobe, and the blood signal beyond the right middle cerebral artery (MCA) disappeared on MRI and MRA. Mechanical thrombectomy (MT) using a suction catheter improved antegrade blood flow in the MCA. The left paresis remained at discharge (modified Rankin Scale score = 4), but she was able to walk independently 3 months after the operation and was independent at home. Acute contralateral ICA occlusion after revascularization for moyamoya disease is an extremely rare complication, but the symptoms can be severe and treatment should be considered. To the best of our knowledge, there have been no reports of MT for postoperative acute contralateral ICA occlusion. Since the results of endovascular treatment such as percutaneous transluminal angioplasty and stent placement for patients with moyamoya disease are poor, MT using an aspiration catheter could be a good treatment option.
AIM:To present a substitute strategy for clipping: coil embolization of the ruptured aneurysm followed by intentional, staged clipping. CASE DESCRIPTION:We treated five cases of ruptured intracranial aneurysms with branches arising from the neck.The mean aneurysm diameter was 4.4 mm.In the acute rupture phase, coiling was performed without adjunctive endovascular techniques with intentional preservation of the neck in all cases.To treat recurrence during follow-up, the previously coiled aneurysm was clipped, which did not occasion any complications.Postoperative imaging showed completely obliterated aneurysms and preserved branches.No rebleeding occurred during the interval between coiling and clipping, and no rebleeding or recurrence occurred after clipping.No treatment-related complications occurred after coiling and clipping. CONCLUSION:This two-stage strategy may be effective for hemostasis and branch preservation for small-and medium-sized ruptured aneurysms with a branch arising from the neck.This intentional two-stage strategy can be a substitute strategy for clipping in the acute rupture phase with an acceptable outcome if the patient cannot undergo clipping as the first-line treatment.
We present a 52-year-old male case of right trigeminal neurinoma at lateral cavernous sinus. The tumor was 40 mm in maximum diameter, obviously compressed temporal lobe and encased internal carotid artery. Extradural middle fossa and transcavernous approach was applied and the tumor was resected via Parkinson's triangle. This approach enabled safe and effective tumor resection, sufficient visualization, and operative field ( Figs. 1 and 2 ). We performed dissection of the tumor from trigeminal nerve, tentorium, and middle fossa and resect the tumor around internal carotid artery sufficiently. Postoperative course was good without any new neurological deficit. This surgical method is considered safe and effective for the resection of the tumor at lateral cavernous sinus. The link to the video can be found at: https://youtu.be/2ekuILIgEuo.
Very large and giant aneurysms (≥20 mm) of the internal carotid artery (ICA) bifurcation (ICAbif) are definitely rare, and optimal treatment is not established. Endovascular treatments are reported as suboptimal due to difficulties of complete occlusion and tendencies to recanalization. Therefore, direct surgery remains an effective strategy if the clipping can be performed safely and reliably, although very difficult.Two cases of ICAbif aneurysms (>20 mm) were treated. Prior assistant superficial temporal artery (STA)-middle cerebral artery (MCA) bypass was performed to avoid ischemic complications during prolonged temporary occlusion of the arteries in both cases. In Case 1 (22-mm aneurysm), the dome was inadvertently torn in applying the clip because trapping had resulted in insufficient decompression. Therefore, in Case 2 (28-mm aneurysm), almost complete trapping of the aneurysm and subsequent dome puncture was performed, and the aneurysm was totally deflated by suction from the incision. This complete aneurysm decompression allowed safe dissection and successful clipping.Trapping, deliberate aneurysm dome puncture, and suction decompression from the incision in conjunction with assistant STA-MCA bypass can achieve complete aneurysm deflation, and these techniques enable safe dissection of the aneurysm and direct clipping of the aneurysm neck. Direct clipping with this technique for very large and giant ICAbif aneurysms may be the optimal treatment choice with the acceptable outcome if endovascular treatment remains suboptimal.