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Open access

Genomic characterization of an esthesioneuroblastoma with spinal metastases: illustrative case

Bianca M Marin, Nathan K Leclair, Erica Shen, Avery Buehler, Upendra P Hegde, Qian Wu, Kevin Becker, Lei Li, Seth Brown, Leo J Wolansky, Hilary Onyiuke, David Choi, and Ketan R Bulsara

BACKGROUND

Esthesioneuroblastoma (ENB) is a rare neoplasm of the sinonasal tract. Currently, the optimal treatment includes maximal resection combined with radiotherapy and/or chemotherapy. Although ENBs often recur and have an aggressive clinical course, spinal metastases are extremely rare and the underlying molecular mechanisms are poorly understood.

OBSERVATIONS

Here, the authors describe a 50-year-old male with an aggressive ENB, initially treated with resection and chemotherapy/radiation, who developed multiple thoracic and lumbar spinal metastases. The authors performed targeted exome sequencing on both the resected primary tumor and biopsied spinal metastases, which revealed 12 total variants of unknown clinical significance in genes associated with the PI3K/AKT/mTOR pathway, chromatin remodeling, DNA repair, and cell proliferation. Six of these variants were restricted to the metastatic lesion and included missense mutations with predicted functional effects in GRM3, DNMT3B, PLCG2, and SPEN.

LESSONS

This report discusses the potential impact of these variants on tumor progression and metastasis, as well as the implications for identifying potential new biomarkers and therapies.

Open access

Hemifacial spasm caused by multiple vascular attachments due to remote compression effects of a dermoid cyst in the cerebellar hemisphere: illustrative case

Kento Hirayama, Takafumi Tanei, Takenori Kato, Toshinori Hasegawa, Eiji Ito, Yusuke Nishimura, and Ryuta Saito

BACKGROUND

Dermoid cysts located laterally in the posterior fossa are rare. The authors report the case of a dermoid cyst in the cerebellar hemisphere presenting with hemifacial spasm (HFS) caused by multiple vascular attachments due to remote compression effects.

OBSERVATIONS

A 48-year-old man presented with left HFS. Computed tomography showed a mass lesion in the left cerebellar hemisphere with calcification and erosion of skull bone. Magnetic resonance imaging showed no contrast enhancement of the lesion and a dural defect. The lesion compressed the brainstem and cerebellopontine cistern, but no vascular attachments to the facial nerve were seen. Tumor removal and microvascular decompression were performed. The lesion was composed of soft tissue containing oil-like liquid and hairs, and the border of the cerebellar arachnoid was clear. There were multiple vascular attachments to the root exit zone, facial nerve, and brainstem. After displacing these arteries, the intraoperative abnormal muscle response disappeared. Histopathological findings showed stratified squamous epithelium, keratin flakes, calcifications, and hairs. The HFS disappeared completely and has remained absent for 27 months.

LESSONS

The dermoid cyst originating from occipital bone compressed the cerebellar hemisphere, displacing multiple vessels and leading to HFS. Tumor removal and the removal of all vascular factors can completely resolve HFS.

Open access

Introducing next-generation transcranial surgery with the head-mounted 3D View Vision display in extracorporeal microsurgery: illustrative cases

Young Ju Kim, Hidehito Kimura, Hiroto Kajimoto, Tatsuya Mori, Masahiro Maeyama, Kazuhiro Tanaka, and Takashi Sasayama

BACKGROUND

Exoscopy in neurosurgery offers various advantages, including increased freedom of the viewing axis while the surgeon maintains a comfortable upright position. However, the optimal monitor positioning to avoid interference with surgical manipulation remains unresolved. Herein, the authors describe two cases in which a three-dimensional head-mounted display (3D-HMD) was introduced into a transcranial neurosurgical procedure using an exoscope.

OBSERVATIONS

Case 1 was a 50-year-old man who presented with recurrent epistaxis and was diagnosed with an olfactory neuroblastoma that extended from the nasal cavity to the anterior cranial base and infiltrated the right anterior cranial fossa. Case 2 was a 65-year-old man who presented with epistaxis and was diagnosed with a left-sided olfactory neuroblastoma. In both cases, en bloc tumor resection was successfully performed via a simultaneous exoscopic transcranial approach using a 3D-HMD and an endoscopic endonasal approach, eliminating the need to watch a large monitor beside the patient.

LESSONS

This is the first report of using a 3D-HMD in transcranial surgery. The 3D-HMD effectively addressed issues with the field of vision and concentration while preserving the effectiveness of traditional microscopic and exoscopic procedures when observed on a 3D monitor. Combining the 3D-HMD with an exoscope holds the potential to become a next-generation surgical approach.

Open access

Radiofrequency thermocoagulation for the treatment of trigeminal neuralgia associated with a focal pontine lesion: illustrative case

Vadym Biloshytsky, Anna Skorokhoda, Inna Buvailo, and Maryna Biloshytska

BACKGROUND

Trigeminal neuralgia (TN) associated with a focal pontine lesion is a rare but challenging condition. The origin of the lesion, which does not fulfill the diagnostic criteria for multiple sclerosis, remains disputable. Pain in such conditions is often refractory to treatment, including microvascular decompression.

OBSERVATIONS

A 36-year-old female presented with a 3.5-year history of shooting pain in the right V2 distribution triggered by talking and chewing. She became less responsive to high-dose carbamazepine over time. Magnetic resonance imaging (MRI) revealed no neurovascular compression but an elongated lesion hyperintense on T2-weighted imaging and T2- fluid-attenuated inversion recovery and hypointense and nonenhancing on T1-magnetization prepared rapid gradient-echo imaging without restricted diffusion, hemorrhage, or supposed malformation along the right pontine trigeminal pathway (PTP). Two other similar lesions were found in the corpus callosum and left thalamus. All lesions were stable compared to MRI data obtained 2 years before. Cerebrospinal fluid contained no oligoclonal bands. Pain attacks ceased with right-sided gasserian radiofrequency thermocoagulation (RFTC), and at the 6-month follow-up, there was no recurrence.

LESSONS

In patients with TN, preoperative neuroimaging should assess for brainstem lesions along the PTP. RFTC can be considered a treatment option in medication-refractory TN associated with a focal pontine lesion.

Open access

Radiological features of internal carotid artery occlusion caused by pituitary apoplexy: illustrative case

Keigo Aramaki, Masanori Aihara, Yu Kanazawa, Takahiro Kawashima, Rei Yamaguchi, Masahiro Matsumoto, Masahiko Tosaka, and Yuhei Yoshimoto

BACKGROUND

Pituitary apoplexy rarely causes internal carotid artery (ICA) occlusion and acute ischemic stroke. Some cases have been reported, but the neuroimaging findings, including cerebral angiography, have not been discussed.

OBSERVATIONS

A 55-year-old male suffered the sudden onset of right cervical pain and left mild hemiparesis. Computed tomography indicated a pituitary mass, and magnetic resonance angiography showed a right ICA occlusion. The initial diagnosis was ICA occlusion caused by ICA dissection. His symptoms worsened and the region of cerebral infarction expanded, so the patient was transferred to our hospital. Magnetic resonance imaging and cerebral angiography showed the sudden stoppage of right ICA blood flow caused by local compression of the tumor near the distal dural ring. The diagnosis was acute ischemic stroke resulting from ICA pseudo-occlusion caused by pituitary apoplexy, and emergent endoscopic transsphenoidal resection was performed. Postoperatively, the right ICA was completely patent, and hemiparesis was improved with rehabilitation.

LESSONS

ICA occlusion caused by pituitary apoplexy is very rare, but emergent treatment is necessary. However, the pathology is difficult to diagnose quickly. Neuroimaging findings showing that the ICA is easily stenosed or occluded if rapidly compressed by the tumor near the distal dural ring may be useful to rapidly diagnose and treat.

Open access

Segmental extraneural lipomatosis of the superficial peroneal nerve: illustrative case

Kitty Y Wu, Aditya Raghunathan, and Robert J Spinner

BACKGROUND

Adipose lesions of nerve are rare tumors that can cause nerve symptoms from either intrinsic or extrinsic compression.

OBSERVATIONS

The authors present a case of a patient with a 10-year history of progressive, persistent leg pain and dorsal foot paresthesias/dysesthesias. Imaging revealed several nondistinct nodules of indeterminate significance along the course of the superficial peroneal nerve (SPN). Surgery demonstrated six distinct extraneural lipomas studded on a 10-cm segment of the main SPN and one of its muscular branches in the midleg. The lesions were adherent to the SPN, without an easy dissection plane; therefore, a neurectomy was performed. Histology revealed the nerve was associated with multiple extraneural lipomas with focal evidence of prior trauma. At 4 months postoperatively, the patient’s pain had resolved completely, and she was able to resume normal physical activities.

LESSONS

The current classification of adipose lesions of nerve includes intraneural and extraneural lipomas and lipomatosis of nerve (fibrolipomatous hamartoma). The unique features of the present case include the discrete and segmental nature of the extraneural lipomas adherent to the nerve. Its etiology is unknown, and the histology would be suggestive of either a traumatic or a degenerative process.

Open access

Surgically treated intracranial arteriovenous fistulas with hemorrhage, resulting in complete obliteration: illustrative cases

Ako Matsuhashi, Kei Yanai, Satoshi Koizumi, and Gakushi Yoshikawa

BACKGROUND

Intracranial arteriovenous fistula (AVF) is a rare disease, defined as anastomoses between cerebral or meningeal arteries and dural venous sinuses or cortical veins. With the development of new agents and devices, endovascular embolization has been considered safe and effective in a majority of cases. However, cases that require direct surgery do exist. Herein, the authors present 3 cases of intracranial AVFs that presented with hemorrhage and were treated with direct surgery, achieving complete obliteration and favorable outcomes.

OBSERVATIONS

Intracranial AVFs that present with hemorrhage require immediate and complete obliteration. When AVFs involve the dural sinus, transvenous embolization is usually the first choice of treatment. AVFs with single cortical venous drainage are best treated with interruption of the draining vein close to the fistula. Transarterial embolization can be a curative treatment if there are no branches supplying cranial nerves or an association with pial feeders. In cases in which endovascular treatment is technically challenging or has resulted in incomplete occlusion, surgical treatment is indicated.

LESSONS

Despite the recent rise in endovascular treatment, it is important to recognize situations in which such treatment is not suitable for intracranial AVFs. Direct surgery is effective in such cases to offer the best possible outcome.

Open access

Trigeminal neuralgia secondary to osteoma and vascular compression: illustrative case

Chenglong Cao, Mingwu Li, Min Wu, and Xiaofeng Jiang

BACKGROUND

Trigeminal neuralgia (TN) is a common neurosurgical issue that has a detrimental impact on patients’ quality of life. Osteoma at the petrous apex is a rare etiology of TN. Here, the authors present a case involving the co-occurrence of petrous osteoma and a vascular loop around the trigeminal nerve. Both exerted pressure or compression on the exit of the trigeminal nerve.

OBSERVATIONS

A 46-year-old male presented with a 3-year history of persistent severe pain in the right side of his face. Magnetic resonance tomographic angiography of the trigeminal nerve revealed an abnormal signal in the right prepontine cistern, along with a vascular loop accompanying the right trigeminal nerve. A computed tomography scan of the skull indicated a nodular calcified density. The combined anterior transpetrosal approach for petrous osteoma and microvascular decompression (MVD) for the offending vessel were successfully performed. The patient was discharged without any complications or facial pain.

LESSONS

Although extremely rare, TN simultaneously secondary to petrous osteoma and offending vessels should be considered in the diagnosis. In this case, the combined surgical removal of petrous osteoma and MVD for the offending vessels proved to be an effective treatment for TN secondary to osteoma and vascular compression.

Restricted access

THE 2023 PRESIDENTAL ADDRESS. Celebrating advocacy: neurosurgeons as advocates

Ann Stroink

The central focus of the 2023 annual gathering of the American Association of Neurological Surgeons in Los Angeles revolved around the concept of neurosurgeons as advocates. Beyond their roles in clinical practice and patient care, neurosurgeons frequently unite in their commitment to advocacy. This shared dedication empowers them to thrive in areas such as innovation, teaching, advanced research, and comprehensive training to shape the future of the neurosurgical field. The substantial outcome of this approach is the establishment of an environment dedicated to delivering the utmost quality of care to neurosurgery patients.

Free access

Applications of enhanced recovery after surgery protocols for unruptured anterior circulation aneurysms in tertiary-level healthcare institutions: a national study

Fatih Yakar, Batuhan Bakirarar, Çağrı Elbir, Emrah Egemen, Şahin Hanalioğlu, Ümit Akın Dere, Serkan Civlan, Çağhan Tönge, Barış Albuz, Mehmet Erdal Coşkun, and Mehmet Erhan Türkoğlu

OBJECTIVE

Enhanced recovery after surgery (ERAS) protocols are standardized perioperative care that reduce patients’ stress response during hospitalization and improve hospitalization time, complication rates, costs, and readmission rates. This study aimed to investigate the application rate of protocols for elective craniotomy in the surgery of unruptured anterior circulation aneurysms (AnCAs) at tertiary-level healthcare (TLH) institutions in Türkiye and its effect on the outcomes of the patients.

METHODS

An electronic survey was sent to all Turkish TLH institutions (n = 127) between May and June 2023. The number of institutions participating in the survey was 38 (30%). The institutions were subdivided according to three main factors: institution type (university hospital [UH] vs training and research hospital [TRH]), annual case volume (low [≤ 20 aneurysms] vs high [> 20 aneurysms]), and institution accreditation status (accredited vs nonaccredited).

RESULTS

Overall, 55.3% (n = 21) of the institutions participating in the study were UHs. The rates of those that were accredited and had a high case volume were 55.3% (n = 21) and 31.6% (n = 12), respectively. It was determined that the accredited clinics applied preoperative protocols at a higher rate (p = 0.050), and the length of stay in the postoperative period was shorter in the clinics that used the intraoperative protocols (p = 0.014).

CONCLUSIONS

The length of stay in the postoperative period is lower in TLH institutions in Türkiye that highly implement intraoperative protocols. Furthermore, this is the first study in the literature evaluating protocols for elective craniotomy in unruptured AnCAs.