NMC Case Report Journal最新文献

Gliosarcoma is a rare malignant neoplasm. It accounts for approximately 2% of all glioblastomas. To date, there is no established treatment method for gliosarcoma, and a variety of therapies, such as surgical resection, radiotherapy, and chemotherapy, are typically employed. Here, we describe a patient with gliosarcoma who, despite multiple tumor metastases throughout the body, including the lungs and lymph nodes, achieved a relatively long survival due to salvage therapy with local irradiation and remarkably effective chemotherapy with low-dose ifosfamide, carboplatin, and etoposide therapy. When the patient died, we performed autopsy and confirmed the nature of the primary and metastatic tumor cells that had spread throughout the patient's body. Clinical and systemic histological studies also suggested the possibility of re-metastasis to the brain from systemic metastatic foci. Gliosarcoma appears to have characteristics similar to sarcoma as well as a higher risk of systemic metastasis. Therefore, a careful follow-up is necessary in such patients.

Transforaminal full-endoscopic spine surgery (TF-FESS) is a novel minimally invasive spine surgery that requires only an 8-mm skin incision and causes minimal damage to the paravertebral muscles. To perform TF-FESS safely and efficiently, preoperative planning is quite important as the intervention requires anatomical understanding and high technical skills. Recently, three-dimensional (3D) printing has become a useful tool in various surgeries, and several studies have addressed its efficacy; however, there are no reports on the application of 3D printing to FESS. In this study, we present two cases of severe lumbar deformities for which preoperative 3D printing was useful. The 3D printing enabled the surgeons to visualize and plan the drilling of the superior articular process for a successful foraminoplasty at a low cost. The manufacturing equipment cost about USD 900 and is able to produce an actual-size model at a cost of less than USD 10 per patient. In conclusion, preoperative planning using 3D printing should be adopted to safely perform FESS.

First bite syndrome (FBS) is an extremely rare complication of carotid endarterectomy (CEA). FBS presents with unique characteristics, development of brief and intense pain in the ipsilateral parotid region at the first bite of each meal, and improvement with subsequent mastication. Here, we report two cases of FBS following CEA. Both cases had anatomical difficulty of a high carotid bifurcation and a high cervical lesion. The lingual and facial arteries (or their common arterial trunk) branched off the external carotid artery (ECA) close to a high carotid bifurcation. The operations required exposure of the ECA distal to these two branches and the distal internal carotid artery (ICA) with a wider range of dissection for clamping the vessels. Several days or weeks after CEA, the patients developed FBS, and their meal-related pain completely resolved in the ensuing weeks. Especially in patients with a high carotid bifurcation, several branches of the ECA tend to originate from the proximal portion of the ECA, similar to octopus arms. During CEA, in these patients, dissection around the ECA and its branches in a wider range is required for exposure of each vessel and placement of the cross-clamp. These procedures can lead to injury to the external carotid nerve and plexus, possibly causing FBS. Additionally, because of the close location of the superior cervical ganglion, external carotid nerves, and distal ICA, manipulation for exposure of the distal end of a high plaque can increase the risk of injury to the cervical sympathetic nerves.

Owing to recent advances in medical optical technology, a high-definition (4K) three-dimensional (3D) exoscope has been developed as an alternative tool to using conventional microscopes for microscopic surgery, and its efficacy for neurosurgery has been reported. We report a case who underwent simultaneous surgery aiming for en bloc resection of an anterior skull base malignancy with concurrent exoscopic transcranial and endoscopic endonasal approaches using a 4K 3D exoscope. The patient was a 76-year-old woman who underwent en bloc resection for an anterior skull base olfactory neuroblastoma 13 years ago. After confirming the recurrence of progressive olfactory neuroblastoma, tumor resection was again decided to be performed. As with the first procedure, surgery was performed in an en bloc manner, using both transcranial and endonasal approaches. Exoscope provided enough space above the surgical field to allow us to perform transcranial and endonasal surgeries simultaneously. Moreover, the surgeons could maintain a comfortable posture throughout the procedure, and total tumor removal was successfully achieved without any abnormal event. To our knowledge, this is the first report of the introduction of an exoscope aiming for en bloc resection of an anterior skull base malignancy while performing simultaneous surgery with both transcranial and endonasal approaches. We believe that the more cases are accumulated, the more efficacy of a 4K 3D exoscope will be elucidated.

Subependymoma (SE) is a rare, usually asymptomatic, brain tumor predominantly affecting older adults and occurring in the fourth and lateral ventricles. We report a rare case of SE with intratumoral hemorrhage that could be removed by neuroendoscopy. The 81-year-old patient had been followed as an outpatient for 10 years due to an intraventricular tumor. It did not grow over the patient's lengthy follow-up. The patient was transferred to our hospital after he fainted near his home; at the time of admission, he had mild consciousness disturbance, and his Glasgow Coma Scale score was 10 points (E3V3M4). Computed tomography showed intratumoral hemorrhage and slight ventricular enlargement. Magnetic resonance (MR) imaging showed a 4 cm-sized tumor in the anterior horn of the right lateral ventricle. The lesion appeared as a mixed-intensity solid tumor and showed irregular enhancement with gadolinium. The patient underwent neuroendoscopic tumor resection on the 30^th day of the patient's hospital stay. A histopathological examination revealed small tumor cells with round nuclei scattered in the glial fibrillary background. Immunostaining was positive for glial fibrillary acidic protein; these findings are consistent with an SE diagnosis. The patient in this study had hypertension and used anticoagulants, risk factors for intratumoral hemorrhage. For intraventricular tumors with bleeding-particularly in older or more physically frail patients-minimally invasive neuroendoscopic surgery should be considered an option for tumor resection.

There are only a few case reports in which cholesterol crystals were found in the thrombus retrieved by mechanical thrombectomy for cryptogenic stroke, leading to a definitive diagnosis. We herein report a case of aortogenic embolic stroke diagnosed by the presence of rich cholesterol crystals in the retrieved thrombus and review the previously reported cases. A woman in her 80s was transferred as an emergency due to consciousness disturbance, right conjugate deviation, and severe left hemiparesis. Magnetic resonance imaging showed occlusion of the right middle cerebral artery (MCA) and acute infarction in the territory. The MCA was recanalized by thrombectomy using an aspiration catheter and stent retriever, and the symptoms improved. Although the physiological examination did not detect the embolic source during hospitalization, pathological examination of the thrombus revealed atheroma with numerous cholesterol crystal clefts and intermixing of fibrin. Contrast-enhanced computed tomography performed based on the pathological results showed atheromatous lesions in the aortic arch as the embolic source. As a subsequent treatment, medications of a strong statin and an antiplatelet agent were continued, and the patient had no recurrence. The finding that the retrieved thrombus is a simple atheroma containing cholesterol crystals with poor hemocytes suggests embolism due to plaque rupture. Pathological examination of the thrombus obtained by thrombectomy is one of the useful diagnostic approaches for stroke etiology and the determination of its treatment.

Methotrexate-associated lymphoproliferative disorder (MTX-LPD) occurs in patients with rheumatoid arthritis (RA) treated with methotrexate (MTX). MTX-LPD is typically associated with Epstein-Barr virus (EBV) infection and regresses with MTX discontinuation. On the other hand, EBV-negative MTX-LPDs are less common and are more likely to show partial or no regression after MTX discontinuation. There were no standard chemotherapeutic options for refractory MTX-LPD. We present a case of EBV-negative MTX-LPD in the central nervous system (CNS) that was successfully treated with rituximab, methotrexate, procarbazine, and vincristine (R-MPV), followed by reduced-dose whole-brain radiotherapy (rdWBRT), following the same treatment protocol as primary CNS lymphoma. A 59-year-old woman with RA treated with MTX presented with gradually developing staggered gait, memory deficit, and disorientation. Multiple lesions with heterogeneous contrast enhancement were discovered using brain magnetic resonance imaging. The patient was suspected of having MTX-LPD, but discontinuing MTX did not result in regression of the brain lesions. She underwent a biopsy from the left parietal lesion. The tissue was pathologically diagnosed as diffuse large B-cell lymphoma. Furthermore, pathological examination through EBV-encoded ribonucleic acid in situ hybridization demonstrated a lack of EBV infection. She was ultimately diagnosed with EBV-negative CNS MTX-LPD. We applied chemotherapy with R-MPV and rdWBRT. The patient achieved a complete response. In the case of CNS MTX-LPD without EBV infection, chemotherapy with R-MPV followed by rdWBRT may be considered.

Facial nerve function improvement is a challenging goal in facial nerve schwannoma (FNS) surgery. Intraoperative continuous monitoring of evoked facial nerve electromyograms (CFN-EMGs) is performed in acoustic neuroma surgery to preserve facial nerve function. CFN-EMGs were applied in decompression surgery for FNS with severe facial paresis. A 39-year-old woman presented with a sudden onset of vertigo, left hearing disturbance, and severe left facial palsy with House-Brackmann (HB) grade 5. FNS was strongly suspected based on the patient's clinical course and magnetic resonance imaging findings, and the patient underwent surgical decompression of the internal auditory canal (IAC) to improve facial nerve function 9 weeks after onset. CFN-EMG responses suddenly improved after removing the posterior wall of the IAC and incising its dura matter. Since the patient's facial nerve paresis improved to HB grade 2 after surgery, CFN-EMGs could detect the moment of facial nerve decompression. This would be the first report to show that CFN-EMGs applied in decompression surgery for FNS could detect the effects of decompression during surgery in real-time. Thus, CFN-EMGs may be an effective monitoring method in decompression surgery for FNS.

There has been a noted increase in the incidence of intracranial aspergillosis; this is often attributed to the wider use of antibiotics, corticosteroids, and immunosuppressants. Fungal cerebral aneurysms due to aspergillosis after neurosurgery remain extremely rare; in fact, only seven cases have been reported in the literature. In this study, we present a patient with an Aspergillus aneurysm that elicited subarachnoid hemorrhage after endoscopic endonasal surgery (EES) for craniopharyngioma. A 70-year-old woman with recurrent craniopharyngioma and steroid treatment underwent uneventful EES. On the 5th postoperative day, she suffered subarachnoid hemorrhage. As per her computed tomography angiography findings, an aneurysm was detected on the left internal carotid artery (ICA). Subsequent digital subtraction angiography showed occlusion of the ICA and an irregularly shaped wall. The diagnosis was pseudoaneurysm. We then performed craniotomy to place a left high-flow bypass and to trap the pseudoaneurysm. Despite continuous intensive care, she died on the 25th postoperative day of a huge, left cerebral infarct. The final diagnosis was made at autopsy; it revealed destruction of the ICA and Aspergillus invasion of the vessel wall, confirming the presence of a true fungal aneurysm. Perioperatively, patients with potential immunosuppression must be carefully managed. Advanced age is a risk factor. As surgery via the paranasal sinuses raises the risk for aspergillosis, fungal infection must be ruled out in patients whose postoperative course is deemed concerning.

Despite recent signs of progress in diagnostic radiology, it is quite rare that a glioblastoma (GBM) is detected asymptomatically. We describe two patients with asymptomatic nonenhancing GBMs that were not diagnosed with neoplasia at first. The patients had brain scans as medical checkups, and incidentally lesions were detected. In both cases, surgical specimens histopathologically showed no evidence of neoplasia, whereas molecular genetic findings were isocitrate dehydrogenase (IDH)-wildtype, O⁶-methylguanine-DNA methyltransferase promoter (pMGMT) unmethylated, and telomerase reverse transcriptase (TERT) promoter mutated, which matched to GBM. One patient was observed without adjuvant therapy and the tumor recurred 7 months later. Reoperation was performed, and histopathologically GBM was confirmed with the same molecular diagnosis as the first surgical specimen. Another patient was carefully observed, and chemoradiotherapy was begun 6 months after the operation following the extension of the lesion. Eventually, because of disease progression, both patients deceased. We postulate that in each case, the tumor was not lower-grade glioma but corresponded to the early growth phase of GBM cells. Thus far, cases of malignant transformation from lower-grade glioma or asymptomatic GBM with typical histologic features are reported. Nevertheless, to the best of our knowledge, no such case of nonenhancing, nonhistologically confirmed GBM was reported. We conjecture these cases shed light on the yet unknown natural history of GBM. GBM can take the form of radiological nonenhancing and histological nonneoplastic fashion before typical morphology. Molecular genetic analysis can diagnose atypical preceding GBM, and we recommend early surgical removal and adjuvant treatment.