UNASSIGNED: Malignant peripheral nerve sheath tumors (MPNSTs) are rare malignant soft-tissue sarcomas arising from peripheral nerves. Little data exist regarding MPNST originating intracranially. Here, we present a 7th/8th nerve complex MPNST, discuss the treatment strategy and patient outcome, and provide a comprehensive review of existing literature.
UNASSIGNED: Using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, PubMed and crossed references were queried, yielding 37 publications from 1952 to the present. Fifty-three cases of primary intracranial and extra-axial MPNST were identified.
UNASSIGNED: We additionally report a 40-year-old female presented with acute onset dizziness and subsequent hearing loss with associated right-sided facial numbness. Magnetic resonance imaging revealed a 0.5 cm × 1.7 cm enhancing lesion within the right internal auditory canal extending into the cerebellopontine angle. The patient was initially treated with retro sigmoid craniotomy for tumor resection followed by a trans labyrinth approach for residual tumor resection. She completed adjuvant fractionated radiation therapy and underwent facial nerve transfer to restore complete hemifacial paralysis. The most common cranial nerves involved were V and VIII (43.4% each), with 66% of patients male and 34% female. The average age was 43.4 ± 17.4 years. The mean survival time for reported non-survivors after tissue diagnosis was 15 ± 4 months. Two-year survival for patients receiving gross total resection was 33.3% versus 22.8% with subtotal resection.
UNASSIGNED: MPNSTs comprise a group of highly aggressive neoplasms that rarely arise intracranially. Gross total surgical resection should be pursued when feasible.

Venous sinus thromboses (VSTs) are rare complications of neurosurgical procedures in the proximity of the dural sinuses. Surgery of the posterior cranial fossa (PCF) and particularly of the cerebellopontine angle (CPA) shows increased risk of VST. VST management is challenging because anticoagulant therapy must be balanced with the risk of postoperative bleeding. We performed a systematic review and meta-analysis to summarize the most important neuroradiologic and clinical aspects of VST after PCF/CPA surgery.
We performed a comprehensive literature search to identify articles reporting data on VST after PCF/CPA surgery. We selected only comparative studies providing adequate neuroimaging assessing VST and a control group.
We included 13 articles reporting 1855 patients. VST occurred in 251/1855 cases (estimated incidence, 17.3%; 95% confidence interval [CI], 12.4%-22.2%). Only presigmoid approach (odds ratio [OR], 2.505; 95% CI, 1.161-5.404; P = 0.019) and intraoperative sinus injury (OR, 8.95; 95% CI, 3.43-23.34; P < 0.001) showed a significant association with VST. VST-related symptoms were reported in 12/251 patients with VST (pooled incidence, 3.1%; 95% CI, 1%-5.2%). In particular, we found a significantly increased OR of cerebrospinal fluid leak (OR, 3.197; 95% CI, 1.899-5.382; P < 0.001) and cerebrospinal fluid dynamic alterations in general (OR, 3.625; 95% CI, 2.370-5.543; P < 0.001). Indications for VST treatment were heterogeneous: 58/251 patients underwent antithrombotics, with 6 treatment-related bleedings. Recanalization overall occurred in 56.4% (95% CI, 40.6%-72.2%), with no significant difference between treated and untreated patients. However, untreated patients had a favorable outcome.
VST is a relatively frequent complication after PCF/CPA surgery and a presigmoid approach and intraoperative sinus injury represent the most significant risk factors. However, the clinical course is generally benign, with no advantage of antithrombotic therapy.

UNASSIGNED: Medulloblastoma, a highly malignant embryonal tumor predominantly found in the pediatric population, typically arises within the cerebellum. This case report holds particular importance due to the rarity of medulloblastoma within the cerebellopontine angle (CPA). The distinct anatomical challenge posed by the CPA complex neurovascular structures, along with the absence of pathognomonic clinical or radiographic features, highlights the unique diagnostic and management challenge of this case.
UNASSIGNED: A 5-year-old boy presented with mild, progressively worsening headaches on CT/MRI imaging, which revealed a solid mass in the left CPA. Radiologically, the lesion closely resembled a CPA meningioma. The patient underwent a left retrosigmoid suboccipital craniectomy, utilizing a modified park bench position and careful burrhole creation. Intraoperatively, the tumor exhibited well-defined margins, firm adherence to cranial nerves, and complex tissue characteristics. Postoperatively, histopathological analysis identified nodular medulloblastoma, WHO grade IV, with immunohistochemical markers confirming its subtype.
UNASSIGNED: This case highlights the critical role of surgical intervention in addressing rare tumors, emphasizing the need for multidisciplinary collaboration in both diagnosis and management to achieve a favorable outcome. Uncommon tumor locations, such as the CPA, require tailored approaches, and the utilization of advanced diagnostic techniques, including immunohistochemistry, aids in accurate subtype classification.
UNASSIGNED: This case highlights the critical role of surgical intervention in addressing rare tumors, emphasizing the need for multidisciplinary collaboration in both diagnosis and management to achieve a favorable outcome.

Paragangliomas (PGLs) are rare neuroendocrine tumors. Sometimes, these tumors secrete excessive catecholamines, which results in the manifestations of various signs and symptoms, usually with a triad of hypertension, tachycardia, and headache. We report the case of a 42-year-old woman presenting with uncontrolled hypertension, right facial palsy, vomiting, and disturbed gait. Diagnosis for PGL was confirmed on postoperative histological examination of the excised mass and correlated with preoperative clinical and radiological findings. Tumor excision was done via a suboccipital craniotomy approach. Our case presents the typically severe features of a jugulotympanic PGL, but most importantly, it highlights the necessity of biochemical diagnosing, thorough probing of the causes of hypertension, and a multi-disciplinary approach in dealing with these tumors. Moreover, the case emphasizes necessitating the use of preoperative embolization in vascular tumors of the head and neck to avoid a hemorrhagic crisis during surgery. Unfortunately, due to a lack of adequate hospital funds, the surgeon had to proceed without preoperative embolization. Despite such a risk, the excision was a success.

BACKGROUND: Epidermoid cysts, originating from ectodermal cell remnants during embryogenesis, are rare, slow-growing tumours commonly found in the cerebellopontine angle, fourth ventricle, and sellar/parasellar regions. This retrospective study explores clinicopathological features, demographics, and radiological details of fifteen central nervous system epidermoid cyst cases over three years.
METHODS: Histologically confirmed cases undergoing surgical excision from July 2020 to July 2023 were retrospectively evaluated. Patient age, clinical presentations, radiological features, and histopathological findings were analyzed using descriptive statistics and aligned with the PROCESS criteria.
RESULTS: Fifteen cases, spanning various age groups and clinical presentations, were included. There were four cases each in the posterior fossa and cerebellopontine angle, three in temporal region, two in intraspinal region, and one each in the retromastoid intra-diploic and third intra-ventricular region. Lesions exhibited diverse distribution, gross findings, and microscopic features. Radiological imaging exhibited well-defined hypo-attenuated masses on computed tomography and contributed to the preoperative diagnosis in eight cases. Despite challenges in complete excision due to adherence, 13 cases underwent successful resection. Chemical meningitis, a common postoperative complication, underscores the importance of meticulous surgery.
CONCLUSIONS: This study highlights clinicopathological characteristics, challenges in surgical management, and postoperative complications of central nervous system epidermoid cysts. Comparative analysis with existing literature emphasizes the uniqueness of epidermoid cysts, differentiating them from craniopharyngiomas and dermoid cysts. Despite the retrospective design, valuable insights are provided, emphasizing the need for prospective studies. Effective management involves complete excision, ensuring long-term remission and emphasizing the distinctive nature of these intriguing intracranial lesions.

The role of surgical management of arachnoid cyst (AC) of the cerebellopontine angle (CPA) is uncertain. This topic has remained controversial with varying contradictory recommendations in the literature, which is limited to mostly case reports. We aimed to provide a comprehensive summary and analysis of symptoms, operative techniques, outcomes, and recurrence of all available surgical cases of AC of the CPA to date.
A systematic literature search was performed in May 2022 querying several scientific databases. Inclusion criteria specified all studies and case reports of patients with AC located at the CPA for which any relevant surgical procedures were performed.
A total of 55 patients from the literature and 5 treated at our institution were included. Mean patient age was 29 years (range, 0.08-79 years), with nearly twice (1.7×) as many female as male patients (37 female, 22 male). Headaches (35%), hearing loss (30%), vertigo (22%), and ataxia (22%) were the most common presentations. Following surgery, 95% experienced symptom improvement, with complete resolution in 64%. Of patients with hearing loss, 44% reported a return to normal. The rate of mortality was 1.69%, and 10% of tumors recurred (mean follow-up 2.3 years [range, 0-15 years].
Symptomatic AC of the CPA is rare. It exhibits a proclivity for females and commonly manifests with headache, hearing loss, vertigo, and ataxia. While careful selection for surgical candidacy is needed and intervention should be reserved for patients with severe symptoms, surgical decompression is an effective tool for symptom alleviation and recovery.

暂无摘要。

Background  Despite advances in multimodal oncologic therapies and molecular genetics, overall survival (OS) in patients with high-grade astrocytomas remains poor. We present an illustrative case and systematic review of rare, predominantly extra-axial World Health Organization (WHO) grade 4 astrocytomas located within the cerebellopontine angle (CPA) and explore the impact of anatomic location on diagnosis, management, and outcomes. Methods  A systematic review of adult patients with predominantly extra-axial WHO grade 4 CPA astrocytomas was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines through December 2022. Results  Eighteen articles were included comprising 21 astrocytomas: 13 exophytic tumors arising from the cerebellopontine parenchyma and 8 tumors originating from a cranial nerve root entry zone. The median OS was 15 months with one-third of cases demonstrating delayed diagnosis. Gross total resection, molecular genetic profiling, and use of ancillary treatment were low. We report the only patient with an integrated isocitrate dehydrogenase 1 (IDH-1) mutant diagnosis, who, after subtotal resection and chemoradiation, remains alive at 40 months without progression. Conclusion  The deep conical-shaped corridor and abundance of eloquent tissue of the CPA significantly limits both surgical resection and utility of device-based therapies in this region. Prompt diagnosis, molecular characterization, and systemic therapeutic advances serve as the predominant means to optimize survival for patients with rare skull base astrocytomas.

Epidermoid cysts represent roughly 1% of all intracranial tumors. They are frequently located in the cerebellopontine angle but rarely extend to the supratentorial brain. Epilepsy is an extremely uncommon manifestation of this neoplasm. We suggest the surgical management of a 35-year-old male who presented with a six-month history of intractable temporal lobe epilepsy. His seizures were characterized by a focal onset in the form of déjà vu experiences, followed by a secondarily generalized tonic-clonic seizure. Imaging revealed a heterogeneous cystic mass in the right cerebellopontine angle, extending supratentorially causing a mass effect on the mesial temporal region. Gross total resection was achieved through a combined subtemporal-retrosigmoid approach. Histopathology revealed an epidermoid cyst. The patient was entirely seizure-free at the three-month follow-up. Epidermoid cysts may present with epileptic seizures. Seizure freedom can be achieved with surgical management in most cases. The patient\'s symptoms, imaging findings, and epileptogenic focus must be considered to select the appropriate surgical strategy.

BACKGROUND: Cerebellopontine angle lipoma is a rare tumor that composes less than 1% of all CPA tumors. There has been no recorded case of unilateral CPA/IAC lipoma with sudden contralateral deafness yet.
METHODS: We report a 52-year-old man diagnosed with right cerebellopontine angle lipoma and combined left total deafness. Pure-tone audiometry revealed total sensorineural deafness in his left ear and moderate sensorineural deafness in the right ear. The patient was treated with glucocorticoids, batroxobin, and other symptomatic treatments. There was no substantial improvement in hearing after 14 days\' treatment.
CONCLUSIONS: We chose conservative treatment for him. It is advised to wear hearing aids in the right ear and to undergo regular imaging monitoring.
CONCLUSIONS: Treatment options for such patients should be chosen by taking into account the degree of bilateral hearing loss, the size and location of the tumor, the possibility of preserving hearing during surgery, the functional level of the patient\'s facial nerve, and other factors.