Management of intractable chronic cough during awake craniotomy: illustrative case

BACKGROUND Chronic cough is a common but challenging clinical condition that can adversely affect the safety of awake surgical endeavors such as awake craniotomy (AC). This case lesson highlighted a patient with severe refractory chronic cough undergoing AC for resection of a recurrent left frontal, insula, anterior temporal anaplastic ependymoma of the eloquent cortex. OBSERVATIONS The patient was successfully managed using a multifaceted medical treatment regimen combined with preoperative and intraoperative cough suppression therapy with a speech-language pathologist. The patient coughed only once intraoperatively and had a positive outcome. LESSONS Chronic cough is often multifactorial and requires a multifaceted treatment approach. Despite this challenge, select patients can successfully be navigated through AC with appropriate treatment for their condition. A review of neurogenic cough and modern treatments, which were used in this patient and would be helpful to neurologists or neurosurgeons, are also discussed.

An Overview of Intracranial Ependymomas in Adults

Ependymomas are rare primary central nervous system tumors. They can form anywhere along the neuraxis, but in adults, these tumors predominantly occur in the spine and less frequently intracranially. Ependymal tumors represent a heterogenous group of gliomas, and the WHO 2016 classification is based essentially on a grading system, with ependymomas classified as grade I, II (classic), or III (anaplastic). In adults, surgery is the primary initial treatment, while radiotherapy is employed as an adjuvant treatment in some cases of grade II and in all cases of anaplastic ependymoma; chemotherapy is reserved for recurrent cases. In recent years, important and interesting advances in the molecular characterization of ependymomas have been made, allowing for the identification of nine molecular subgroups of ependymal tumors and moving toward subgroup-specific patients with improved risk stratification for treatment-decisions and future prospective trials. New targeted agents or immunotherapies for ependymoma patients are being explored for recurrent disease. This review summarizes recent molecular advances in the diagnosis and treatment of intracranial ependymomas including surgery, radiation therapy and systemic therapies.

A-43 Pediatric Infratentorial Anaplastic Ependymoma: A Case Study

Objective Ependymoma is the third most common malignant pediatric brain tumor and often presents posteriorly. Understanding of long-term outcomes of pediatric posterior fossa tumor and treatment is required to maximize developmental trajectory. This case illustrates cognitive correlates of pediatric posterior fossa tumor and treatment in an adolescent without cerebellar mutism or psychiatric co-morbidities. Repeated post-treatment assessments allowed for characterization of initial effects of treatment over time. Method The patient, diagnosed with an infratentorial anaplastic ependymoma at age 15, presented for initial neuropsychological evaluation status-post craniotomy, ventricular drain placement, posterior fossa tumor resection, radiotherapy, and chemotherapy. Recovery was complicated by cranial nerve palsies, dysphasia, and dysarthria. Repeat assessment was conducted 13 months later. Interim treatment included three cycles of maintenance chemotherapy. Aside from moderate dysarthria, the patient denied cognitive concerns each evaluation. Results Attention, working memory, planning, problem solving, language, reasoning, and motor speed/dexterity were within expectations at initial evaluation without deficits in academic or socioemotional functioning. Visual encoding/organization and processing speed were below expectations. Findings at follow-up revealed marked declines in bilateral motor speed and dexterity. Conclusion Weaknesses in visual organization and motor functioning are consistent with expected neurocognitive correlates of pediatric posterior fossa tumor and resection, particularly with cerebellar involvement. Additional motoric slowing is associated with white matter changes and often observed after cancer treatment. Longitudinal follow-up illustrated the potential for neurocognitive resilience in pediatric posterior fossa tumor treatment in the face of significant motor dysfunction. Nevertheless, late-effects of radiation and chemotherapy may manifest in early adulthood and must be monitored in pediatric patients.

Anaplastic ependymoma

The authors present a clinical case of anaplastic ependymoma in a 6-year-old child with the clinical manifestations of intracranial hypertension syndrome with evolutionary hydrocephaly and cerebellar-atactic syndrome. CT and brain MRI investigation as well as histology were essential in establishing multimodal treatment. 11 references, 3 pictures.

Multifocal Intradural Extramedullary Anaplastic Ependymoma With Intracranial Involvement at Presentation: A Case Report

Background and Importance: Ependymomas are a rare malignant neoplasm. Multifocal intradural extramedullary anaplastic ependymomas are even more of a rare entity with much of the current knowledge derived from case reports. We presented a case of a multifocal intradural extramedullary anaplastic ependymoma with intracranial involvement at presentation. Case Presentation: A 53-year-old male presented with urinary symptoms. Magnetic resonance imaging revealed two lesions along the spinal cord and two lesions, intracranially. Histopathological examination was consistent with the World Health Organization grade III anaplastic ependymoma. The patient was treated with the gross total resections of spinal cord lesions, followed by radiation therapy to the resection cavities and intracranial lesions. At the 10-month follow-up visit, he reported almost complete resolution of symptoms, and magnetic resonance imaging revealed no recurrence. Conclusion: Despite their rarity, ependymomas should be considered as the differential diagnosis when evaluating spinal tumors. Gross total resection followed by targeted radiotherapy appears to be an effective treatment modality for high-grade lesions.

PAX6 is frequently expressed in ependymal tumours and associated with prognostic relevant subgroups

AimsAn ependymoma shows divergent morphological and molecular features depending on their location. The paired box 6 (PAX6) transcription factor is a putative tumour suppressor and drives cancer cells towards a stem cell-like state. A transcriptome study reported high PAX6 expression in ependymal tumours, but data on protein expression are lacking.MethodsWe, therefore, analysed PAX6 expression by immunohistochemistry in 172 ependymoma samples and correlated its expression to histology, WHO grade, anatomical location and molecular subgroups.ResultsMean PAX6 nuclear expression in ependymoma was 27.5% (95% CI 23.3 to 31.7). PAX6 expression in subependymoma (mean: 5%) was significantly lower compared with myxopapillary (30%), WHO grade II (26%) and anaplastic ependymoma (35%). Supratentorial ependymomas also displayed significant lower PAX6 levels (15%) compared with spinal cord tumours (30%). Expression levels in YAP1-fused ependymoma (41%) were higher compared with REL-associated protein (RELA)-fusion positive tumours (17%), while PAX6 expression was similar in posterior fossa group A (33%) and B (29%) ependymomas. Kaplan-Meier analysis in RELA-fusion positive ependymomas and posterior fossa group B showed a significant better outcome for PAX6 at or above the cut-off of 19.45% compared with tumours with PAX6 below the cut-off.ConclusionsWe demonstrate that PAX6 is frequently expressed in human ependymal tumours and immunohistochemistry may be helpful in determining prognostic relevant subgroups.