BACKGROUND: Vestibular schwannomas - benign tumours originating from the vestibular nerve - are rare during pregnancy. The intricate interplay between the gravid uterus, maternal physiology and neoplastic growth imposes complexities that demand a careful and tailored approach.
METHODS: This article reports a case of a pregnant woman in her 30 s diagnosed with a large vestibular schwannoma exhibiting brainstem compression, peritumoral oedema and cranial nerve encasement at 36 + 5 weeks of gestation. A multi-disciplinary team collaborated to devise a treatment plan considering the delicate balance between fetal well-being and the urgent need for intervention. A conservative approach involving close monitoring, corticosteroid therapy to manage peritumoral oedema, and detailed fetal assessments was initially employed. As the patient neared full term, a carefully planned caesarean section was performed, followed by a successful craniotomy to resect the vestibular schwannoma. Both the mother and the newborn showed favourable outcomes postoperatively. In addition, a literature review of cases of vestibular schwannoma in pregnancy was undertaken to inform optimal management strategies and enhance understanding of this complex scenario.
CONCLUSIONS: This case highlights the complexity of managing vestibular schwannomas in pregnant women, and underscores the importance of a tailored, collaborative approach. The condition was resolved successfully, emphasizing the significance of timely diagnosis, meticulous planning and a patient-centred approach in these rare and intricate cases.

Sporadic vestibular schwannomas (VSs) are rare in children. When occurred in the pediatric population, they usually appear bilaterally and are related to neurofibromatosis type 2 (NF2). The current study reports a 4-year-old boy without family history of VS or NF2 who presented with a large (5.7-cm) VS involving the right cerebellopontine angle and internal auditory canal. Through seven-staged surgical interventions and two stereotactic γ‑knife radiosurgery, the disease was stabilized. At 2-year follow-up, the child had right ear hearing loss, grade IV facial palsy, and normal motor function and gait. No definite evidence of gene mutation regarding NF2 can be identified after sequence analysis and deletion/duplication testing. This case highlights the significance of considering the possibility of sporadic VSs, even in very young children. It emphasizes the importance of not overlooking initial symptoms, as they may indicate the presence of a large tumor and could potentially result in delayed diagnosis.

Schwannomas, also known as neurilemomas, are peripheral nerve sheath neoplasms. They can be sporadic or associated with genetic syndromes including neurofibromatosis type 2 (NF2). Schwannomas may lead to symptoms by exerting pressure on nearby structures, such as nerve and muscle fibers. In this study, we present the case of a 22-year-old female with a history of NF2 who, upon examination, presented with a visibly enlarged salmon-colored mass involving the left inferior rectus that she had since the age of 12 years. Ocular examinations revealed a small left hypertropia and exotropia in all gazes. Magnetic resonance imaging confirmed bilateral involvement of the inferior rectus muscles. She had a partial excisional biopsy of the mass involving the left inferior rectus muscle that confirmed the presence of schwannoma. This case highlights the importance of comprehensive evaluation of sensory and motor functions as well as considering orbital schwannomas in cases of strabismus, especially within the context of neurofibromatosis.

BACKGROUND: Ependymomas are the third most common central nervous system tumor in the pediatric population; however, spinal ependymomas in children are rare. Ependymomas affecting the spinal cord most frequently occur in adults of 20-40 years of age. The current World Health Organization classification system for ependymomas is now composed of ten different entities based on histopathology, location, and molecular studies, with evidence that the new classification system more accurately predicts clinical outcomes.
METHODS: We present the case of a 16-year-old Caucasian female patient with a history of type 2 neurofibromatosis with multiple schwannomas, meningioma, and spinal ependymoma. Chromosome analysis of the harvested spinal ependymoma tumor sample revealed a 46,XX,-6,+7,-22,+mar[16]/46,XX[4] karyotype. Subsequent OncoScan microarray analysis of the formalin-fixed paraffin-embedded tumor sample confirmed + 7, -22 and clarified that the marker chromosome represents chromothripsis of the entire chromosome 6 with more than 100 breakpoints. Fluorescent in situ hybridization and microarray analysis showed no evidence of MYCN amplification. The final integrated pathology diagnosis was spinal ependymoma (central nervous system World Health Organization grade 2 with no MYCN amplification.
CONCLUSIONS: This case adds to the existing literature of pediatric patients with spinal ependymomas and expands the cytogenetic findings that may be seen in patients with this tumor type. This case also highlights the value of cytogenetics and microarray analysis in solid tumors to provide a more accurate molecular diagnosis.

UNASSIGNED: Vestibular schwannoma (VS) is a benign tumor that develops from Schwann cells of the eighth cranial pair, mainly in the cerebellopontine angle.
UNASSIGNED: We report the case of a 30-year-old female patient who developed left otalgia associated with neglected tinnitus, the evolution of which was marked by the development of a static cerebellar syndrome and a behavioral disorder, whose brain MRI revealed a locally advanced process in the cerebellopontine angle at the expense of the vestibulocochlear nerve, in favor of a VS, complicated by involvement of the tonsils, which unfortunately led to the patient\'s death.
UNASSIGNED: VS, formerly known as acoustic neuroma, is an extra-axial intracranial tumor that accounts for over 80% of pontocerebellar angle tumors, and is secondary in the majority of cases to inactivation of the neurofibromatosis type 2 (NF2) tumor suppressor gene, either by mutation of the NF2 gene or loss of chromosome 22q. In the majority of cases, it is unilateral and solitary, but in almost 8% of cases, it is associated with NF2. Cerebral MRI is the examination of choice for the detection, characterization, and diagnosis of VS without the need for biopsy, mainly with T1-weighted sequences before and after gadolinium injection. Treatment is based essentially on surgery or radiosurgery, depending on the size, impact, and expertise of the treatment team.
UNASSIGNED: VS remains an important intracranial tumor entity, which can be life-threatening in cases of advanced local invasion.

Neurofibromatosis type 2 (phacomatosis) is a rare inherited autosomal dominant condition defined by the development of numerous central neuronal tumors. In addition to classic intracranial schwannomas, intracranial and spinal meningiomas, and intramedullary ependymomas, it can be associated with a few cutaneous abnormalities. In this report, we discuss the case of a 21-year-old female who was examined for persistent headache with cutaneous masses and bilateral hearing loss. Magnetic resonance imaging of the cranium and the whole spine detected multiple meningiomas, intracranial, and intramedullary tumors.

(1) Background: This study aimed to evaluate the efficacy and treatment-related toxicity of proton radiotherapy (PRT) for vestibular schwannoma (VS) in patients with neurofibromatosis type 2-related schwannomatosis (NF2). (2) Methods: Consecutive NF2 patients treated with PRT for VS between 2004 and 2016 were retrospectively evaluated, focusing on tumor volume, facial and trigeminal nerve function, hearing, tinnitus, vestibular symptoms, and the need for salvage therapy after PRT. (3) Results: Eight patients were included (median age 36 years, 50% female). Median follow-up was 71 months. Five (63%) patients received fractionated PRT and three (38%) received PRT radiosurgery for VS. Six patients (75%) received prior VS surgery; three also received bevacizumab. Six patients (75%) did not require salvage therapy after PRT. Two patients (25%) with residual hearing lost it after PRT, and six had already lost ipsilateral hearing prior to PRT. Tumor and treatment-related morbidity could be evaluated in six patients. Following PRT, conditions that occurred or worsened were: facial paresis in five (83%), trigeminal hypoesthesia in two (33%), tinnitus in two (33%), and vestibular symptoms in four patients (67%). (4) Conclusion: After PRT for VS, the majority of the NF2 patients in the cohort did not require additional therapy. Tumor and/or treatment-related cranial nerve deficits were common. This is at least partly explained by the use of PRT as a salvage treatment after surgery or bevacizumab, in the majority of cases. There remains the further opportunity to elucidate the efficacy and toxicity of proton radiotherapy as a primary treatment.

Schwannoma is a slow-growing nerve sheath tumour comprising differentiated neoplastic Schwann cells. The plexiform variant of schwannoma grows in a plexiform or multinodular pattern and may be conventional or of cellular type. Clinically, they manifest as single, skin-coloured tumours along the distribution of peripheral or cranial nerves. This tumour usually ranges between 2 and 4 cm in size, common sites of localization being the head or the flexor aspect of the extremities. The tumour may be associated with neurofibromatosis Type 2 (but not with neurofibromatosis Type 1). The majority are biphasic tumours containing compact areas (Antoni A tissue) showing occasional nuclear palisading (Verocay bodies), alternating with loosely arranged foci (Antoni B tissue). Here, we report a case of a 37-year-old female patient, with complaints of swellings over the right forearm, right wrist and right hand. Biopsy specimens were sent from each of the swellings for histopathological evaluation. The final impression of plexiform schwannoma was made based on the microscopical examination of hematoxylin-and-eosin-stained sections.

A primary intracochlear schwannoma (ICS) is a unique type of vestibular schwannoma (VS); the tumor originates from the terminal branches of the cochlear nerve and is confined to the cochlea. An ICS is the most common subtype of schwannoma in the inner ear. As an ICS is clinically rare, diagnosis and treatment remain challenging. We report a rare case of cochlear implantation (CI) in a patient with neurofibromatosis type 2 and an ICS. The patient exhibited bilateral, profound, sensorineural hearing loss. The tumor on one side was a common VS treated via tumor and acoustic nerve resection and that on the other side an ICS. To ensure auditory rehabilitation via CI, we performed CI while removing part of the ICS via an enlarged round window. Auditory rehabilitation was satisfactory. Thus, ICS patients, especially those who urgently require auditory rehabilitation, can undergo simultaneous CI and (total or partial) tumor removal. However, the long-term results require close observation.

BACKGROUND: Neurofibromatosis type 2 (NF-2) is an inherited disease, linked with abnormalities in the NF-2 gene, which is located on chromosome 22 and involved in merlin production. Many craniospinal tumors are common in individuals with NF-2. We present a case of NF-2 with the rapid symptomatic progression of multiple craniospinal tumors.
METHODS: A 12-year-old male complained of headache and hearing impairment in the right ear for 7 months. Brain magnetic resonance imaging (MRI) revealed a right frontal meningioma, bilateral vestibular and trigeminal schwannomas, and a brainstem tumor. He was diagnosed with NF-2 and underwent brain surgery and radiotherapy for chordoid meningioma. He complained of right leg motor weakness 5 months post-surgery. The spine MRI showed multiple heterogeneously enhanced masses spreading over the entire spinal cord. The symptomatic intradural extramedullary mass at the cervicothoracic area was removed and the histological finding was schwannoma. His leg motor weakness was relieved after surgery. At the 6-month follow-up, brain MRI revealed the progression of the vestibular schwannoma, trigeminal schwannoma, and brainstem tumor. The patient was treated with bevacizumab (5 mg/kg) every 2 weeks for 6 months. For 2 years, all of the craniospinal tumors were stable without neurological deterioration after the completion of chemotherapy.
CONCLUSIONS: Meningiomas and schwannomas grow slowly in most patients with NF-2, but these multiple craniospinal tumors can show sudden rapid growth and manifest as neurological symptoms in a pediatric patient. These tumors could be controlled with local symptomatic and systemic bevacizumab treatments.