bluuebunny/crossref_metadata_2025 huggingface dataset:
P19.03.A CHARACTERIZATION OF THE IMMUNE MICROENVIRONMENT IN ATYPICAL MENINGIOMAS
Authors: Del Bene M , Agistri F , Patanè M , Carone G , D’Angeli E , Porto E , Stroppa A , Calatozzolo C , Di Meco F , Pollo B
Published: October 2024

BACKGROUND

Meningiomas are the most frequent primary intracranial tumors. The high incidence of grade 2 meningiomas (or atypical meningiomas) and their tendency to recur make them a challenge for patients and healthcare systems. Treatment is based primarily on surgical resection, which may be followed by radiotherapy and, seldom and only in extreme cases, chemotherapy. The most controversial topic regarding the treatment concerns the use of adjuvant radiotherapy. To evaluate if a better knowledge of the immune microenvironment of meningioma may help in planning treatments, we carried out a study on the tumor immune infiltrate (TII) looking for a role as a prognostic/predictive marker, suggesting tailored therapies. With this aim our study proposes the development of a panel including demographic, clinical, and pathological data, focusing on the TII, with the highest goal of adding a tool useful for guiding the indication and timing of adjuvant treatments.

MATERIAL AND METHODS

For this purpose, we carried out a histological review of 53 atypical meningiomas following The 2021 World Health Organization (WHO) Classification of CNS Tumors. Through an immunohistochemical analysis, with an expert pathologist, we evaluated the presence of different classes of immune infiltrates. The panel includes different immune infiltrate markers, microglia, monocytes and macrophages biomarkers and NK cells. We also evaluated the loss of H3K27-me3 as prognostic marker of aggressiveness.

RESULTS

27/53 samples showed a massive presence of lymphocytic infiltrate, mostly CD3+ and CD8+ T cells, which is widespread in the tumor and not only in the interface with the healthy nervous tissue. In a large number of cases, these data were also correlated to the presence of PD1 (Programmed death-1). CD4+ T lymphocytes and CD20+ B lymphocytes in the majority of cases were poorly represented, while microglia and macrophages were widely present in tumors.Furthermore, we noticed a discrete or high presence of NK cells, widespread throughout the tumor mass. CD11b+ monocytes were mostly expressed in blood vessels, in adjacent nervous tissue (when present in the sample) and in the interface between the latter and the tumor. Suppressive myeloid immune cells CD14+ were found in most samples, with a degree of labeling ranging from weak to strong.

CONCLUSION

The data we obtained lead us to assume that atypical meningiomas present a “hot” tumor microenvironment characterized by a high immune infiltrate, however, the meaning and the role of this profile will be correlated with clinical data, investigating the impact on the tumor progression. Moreover, we are also looking to understand if meningiomas with abundant TII may be potentially responsive to immuno-oncological therapies.
P19.14.A TARGETING TUMOR-ASSOCIATED MACROPHAGES IN THE IMMUNE-MICROENVIRONMENT OF MENINGIOMAS
Authors: Lotsch C , Warta R , Dao Trong P , Krieg S , Sahm F , Unterberg A , Herold-Mende C
Published: October 2024

BACKGROUND

Meningiomas represent the most common primary brain malignancies in adults with a subset of tumors exhibiting aggressive clinical behavior. Immunotherapy might present a new treatment strategy but is highly dependent on the immunological composition of the tumor microenvironment. Our previous data have shown that tumor-associated macrophages (TAMs) make up the main immune cell population in meningiomas with a significant negative impact on patient outcome. In this study, we investigated whether TAMs from meningioma tissue could be reprogrammed to an immunologically active and tumoricidal phenotype.

MATERIAL AND METHODS

For this purpose, CD11b+ sorted macrophages derived from tumor samples from > 40 patients including a substantial number of clinically aggressive meningiomas were treated with small molecule inhibitors targeting the colony-stimulating factor-1 receptor (CSF-1R). In a first analysis, the direct treatment response of CD11b+ patient-derived macrophages has been investigated by various techniques including flow cytometry and bulk RNA-sequencing of treated TAMs and further analysis of the macrophage-conditioned media after treatment. In addition, we studied the influence of CSF-1R-targeted macrophage treatment on the phenotype and functional activity of T cells to assess a potential indirect treatment response in the tumor microenvironment.

RESULTS

Our data revealed that CSF-1R-targeted treatment of CD11b+ TAMs induced significant changes in the protein and gene expression of macrophage polarization markers towards a more immunologically active state and a significantly higher metabolic nitric oxide production as another sign of immunological activation. Subsequent analysis of indirect effects on T cells showed not only a significantly increased expression of the T cell activation marker CD69+, but also a significantly increased tumor cell killing by autologous T cells after macrophage-targeted treatment.

CONCLUSION

Together these data suggest both a direct and indirect CSF-1R-targeted macrophage treatment response in the local tumor microenvironment and give first promising results on the efficacy of macrophage-targeted immunotherapy in human meningiomas.
P19.15.B TERT PROMOTOR MUTATIONS IN ATYPICAL MENINGIOMAS
Authors: Meta R , Dai H , Sjursen W , Torp S
Published: October 2024

BACKGROUND

In human meningiomas TERT promotor mutations have been shown to be associated with a potentially worse prognosis, thus favouring a CNS WHO grade 3. In this study we wanted to investigate whether this genetic event could distinguish between atypical meningiomas with variable clinical course.

METHODS

In our archives we found twenty human meningiomas among which 10 cases experienced recurrence and 10 cases did not. Formalin-fixed and paraffin-embedded (FFPE) material was analysed by means of Sanger sequencing to assess the occurrence of TERT promotor mutations.

RESULTS

No mutations were found in 17 cases. In 3 cases the DNA quality was too poor for sequencing.

CONCLUSION

We found no TERT promotor mutations in our series of atypical meningiomas, so the prognostic value of this genetic event in these tumours requires further studies. Since there were cases with poor DNA quality, such sequencing analyses on FFPE meningioma tissue may be problematic and may therefore represent a limitation in retrospective studies.
P20.04.A ATYPICAL TERATOID/RHABDOID TUMOR ARISING FROM THE THIRD VENTRICLE IN YOUNG ADULT
Authors: Hwang J , Lee C , Yoon S , Park K , Park S
Published: October 2024

BACKGROUND

Atypical teratoid/rhabdoid tumor (AT/RT) Is a high-grade CNS malignancy predominantly seen in infants and children. AT/RTs occur throughout the neuraxis but have high predilection for midline structure such as pineal gland and brainstem. Suprasellar location is rare and pure third ventricular AT/RT is exceptional. We report a case of AT/RT in a young adult with third ventricle origin. This is the second documented case of adult third ventricle AT/RT.

MATERIAL AND METHODS

A 24-year-old male presented with two-week-long dizziness and diplopia. Magnetic resonance imaging (MRI) of the brain showed a heterogeneously enhancing large lesion in the third ventricle with isointense on T1WI and T2WI. Supratentorial ventricular space was enlarged with Evan’s ration of 33%. He underwent anterior transcallosal transchoroidal approach with gross tumor resection.

RESULTS

Postoperative course was uneventful with no hormonal deficiency. CSF diversion was not needed. Histopathological examination revealed AT/RT, WHO grade 4, with loss of SMARCB1 (INI 1) protein. H3 mutation was not found. He later underwent cranio-spinal irradiation with local booster followed by high dose chemotherapy (carboplatin, thiotepa and etoposide) with autologous stem cell transplantation. No recurrence was found on follow-up MRI at 10 months after operation.

CONCLUSION

AT/RT is a rare primary CNS tumor but their incidence among adults is being increased. We report a case of young adult AT/RT with very rare third ventricle location. AT/RT maybe considered as a differential diagnosis for suprasellar-third ventricle lesion in young adults.
P20.06.B CHOROID PLEXUS TUMORS IN ADULT: RETROSPECTIVE MONO-INSTITUTIONAL STUDY
Authors: Anghileri E , Gaviani P , Amato A , Pollo B , Marchetti M , Doniselli F M , Eoli M , Redaelli V , Botturi A G L , Di Meco F , Ferroli P , Farinotti M , de Oliveira Muniz Koch L , Paterra R , Silvani A
Published: October 2024

BACKGROUND

Choroid plexus tumors (CPT) are rare entities, mainly affecting childhood, including three grade subtypes.

MATERIAL AND METHODS

A mono-institutional retrospective series of adult CPT patients was collected. The database contains demographic and clinical details, diagnostic (radiological and histological/molecular), therapeutic, recurrence information, and survival data.

RESULTS

We described twenty-four adult CPT, with a median age of 43.29 years [Interquartile range (IR) 37.31-53.30]. Clinical onset (n=20/24) included cerebellar signs (n=11), intracranial hypertension signs (n=8), cranial nerves impairment (n=5), seizures (n=1), spinal signs (n=1) (multiple symptoms in some patients). Remarkably, in 17% the radiological detection was occasional.The tumors were mostly located in the ventricular system at first diagnosis: 79.2% were infratentorial, but any other locations can occur, including the spine (one case); meningeal involvement was present in one case. Only one case presented a pre-surgical hydrocephalus. CPT histological grade ranged from grade 1 (n=17), grade 2 (n=6), and grade 3 (n=1). TERTp mutations were detected in 17.6% (n=3/17). TP53 mutation in 5.9% (n=1/17). Complete resection was achieved in n=16/24 (66.7%), gross-total resection in n=3/24 (12.5%), partial resection in n=3/24 (12.5%), biopsy in one case, and no information on surgery extension in one case. 76% of the cases (n=16/21) experienced clinical worsening suddenly after surgery for different reasons, and mostly gradually recovered. Adjuvant therapy was performed only for grade 2 and 3 tumors, and included conformational radiotherapy (RT) in one disseminated grade 2 and in one grade 3 case. At recurrence, surgery, RT, and chemotherapy had been considered. The median Overall Survival from surgery was 108.1 months [IR 62.5-143.4].

CONCLUSION

We could confirm that CPT can occur in adults, and are mostly grade 1 tumors and located in the ventricular system. The surgical approach is the gold standard, although a substantial number of clinical worsening occurred, but often transient. Adjuvant treatment is limited to higher grade CPT; however, no consensus has already been achieved about adjuvant therapy.

FUNDING

RRC Italian Ministry of Health
P20.08.B CLINICAL SEVERITY GRADING OF NF2-RELATED SCHWANNOMATOSIS
Authors: Lawson McLean A , Löschner D , Farschtschi S , Dengler N , Neumeister A , Senft C , Rosahl S
Published: October 2024

BACKGROUND

Nf2-related schwannomatosis (NF2) is a genetic disorder linked to tumours in the nervous system, but the extent of radiological tumour burden doesn’t always align with the diverse disabilities patients face. Additionally, there lacks a validated clinical classification for assessing disease severity.This study proposes a clinical classification comprising three severity grades, aiming to enhance communication among caregivers regarding individual patients and aid in clinical management.

MATERIAL AND METHODS

The records of 196 patients were reviewed to identify the most common diagnoses with significant impacts on daily tasks, social interactions, and life expectancy, resulting in seven main categories. Each category was assigned one point. Using a training set, three severity grades were established: grade 1 (mild NF2) with 0 points, grade 2 (moderate NF2) with less than 3 points, and grade 3 (severe NF2) with 3 or more points. The grading system was then evaluated for inter-rater reliability and clinical significance.

RESULTS

Substantial inter-rater reliability was observed among three raters with varying levels of clinical experience (Fleiss’ kappa = 0.62). The severity grades showed significant correlations with hospitalization rates, number of operations, and reliance on implants such as cochlear implants, auditory brain-stem implants, or ventriculoperitoneal shunts.

CONCLUSION

This simple and rater-independent scoring system comprising three grades effectively reflects the clinical disease severity of NF2 patients. This system may facilitate communication among medical personnel of diverse backgrounds and experience levels and provides a valuable clinical tool for decision-making and research in NF2 management.
P20.13.B LHERMITTE-DUCLOS DISEASE - AN ILLUSTRATED SURGICAL CASE REPORT
Authors: Casimiro C D , Carneiro M , Monteiro J , Dias F
Published: October 2024

BACKGROUND

Dysplastic gangliocytoma of the cerebellum/Lhermitte-Duclos disease is a rare pathologic condition of uncertain etiology, characterized by the presence of a slow growing cerebellar tumor with very typical appearance on MRI and benign histopathological features. In adults it is part of the Cowden syndrome, an autosomally dominant genetic disorder characterized by the development of multiple body hamartomas and also an increased likelihood of malignant neoplasms.

METHODS

Report of a surgical case.

RESULTS

We report the rare case of a 31 years old female that presented with a history of headache, episodes of vomiting and progressive visual impairment since the previous year. The patient had several malformative features such as macrocephaly, retrognathism and short hands. The neurological examination was positive for mild appendicular dysmetria and bilateral papilledema. A CT scan showed an enlargement of the supratentorial ventricular system and a hypodense left cerebellar hemispheric lesion with moderate mass effect. A subsequent MRI showed an intrinsic expansive hemispheric lesion, with striated folial pattern (“tigroid” appearance), hyperintense on T2 and with no contrast enhancement; there was moderate cerebellar amygdala ectopy, a constitutive reduced amplitude of the posterior fossa and an extensive cervico-dorsal syrinx, features compatible with a Chiari type 1 malformation. The patient underwent surgery for lesion removal via a sub-occipital craniotomy and EVD placement, later removed without need for shunting. Post operative MRI was negative for complications but raised the question about a small antero-lateral residue. Pathology showed a dysplastic gangliocytoma (WHO grade 1) compatible with Lhermitte Duclos (which in adults is pathognomonic of Cowden Syndrome). During follow up the patient developed neck stiffness due to cervical muscle contracture which progressively improved. Headache and visual impairment also progressively improved. Given this diagnostic the patient is now under extensive clinical and serial imaging follow up. MRI at 3 months after surgery showed mass effect resolution on the posterior fossa, with improvement of the cerebellar tonsils ectopy and a smaller residual syrinx.

CONCLUSION

We report a rare case of Lhermitte Duclos disease that was diagnosed and treated successfully with surgery. The subsequent improvement of ventriculomegaly, the Chiari-like findings, and syrinx was remarkable, without the need for additional posterior fossa decompression surgery. A close and long follow up is needed in order to anticipate and diagnose the possible manifestations of this rare syndrome.
P20.14.A METASTATIC EPITHELIOD SOLITARY FIBROUS TUMOR
Authors: Wang R , Pejhan S , Ang L , Megyesi J
Published: October 2024

BACKGROUND

Epitheliod solitary fibrous tumor is a rare central nervous system tumor that can rarely metastasize. An unusual case is illustrated.

MATERIAL AND METHODS

A 76 year old man underwent resection of a lower cervical spinal cord (C5-C7) tumor that was called hemangiopericytoma at time of resection. He was treated with radiotherapy. Twenty-four years later he presented with headache and gait ataxia and neuroimaging revealed a 4th ventricular tumor. He underwent surgical resection via a suboccipital craniectomy.

RESULTS

Pathology showed a hypercellular tumor composed of epitheliod cells with eosinophilic cytoplasm and moderately pleomorphic enlarged oval nuclei containing finely dispersed chromatin and prominent eosinophilic nucleoli in some nuclei. The tumor cells were arranged in nests and sheets separated by thin walled branching to thick walled hyalinized vessels with frequent areas of hemorrhage. Tumor cells showed high mitotic activity (about 8 mitoses/mm squared). The majority of tumor cells were positive for CK7, synaptophysin, E-cadherin, and rarely for CK20 and D2-40. Tumor cells were negative for EMA, transthyretin, myogenin, desmin, progesterone receptor, NeuN, Olig-2, TTF-1, GATA3, PAX8, OCT3/4, SALL4, CD117, S100 and GFAP. Vasculature was highlighted by PAS, reticulin, CD31, ERG, and CD34. INI-1 was intact in the nuclei. Final diagnosis was epitheliod solitary fibrous tumor, at least CNS WHO grade 2. This was consistent with the pathology from 24 years prior.

CONCLUSION

Solitary fibrous tumor is known for recurrence and metastasis a long time after initial diagnosis. However, this case highlights the delayed metastatic spread of a relatively rare subtype of solitary fibrous tumor (epitheliod) to a rare location (intraventricular, specifically the 4th ventricle). The literature will be reviewed.
P20.15.B MOLECULAR CHARACTERIZATION OF ADULT NON-GLIOBLASTOMA CENTRAL NERVOUS SYSTEM (CNS) TUMORS TO IDENTIFY POTENTIAL TARGETTABLE ALTERATIONS
Authors: Padovan M , Bosio A , Cella E , Maccari M , Cerretti G , Corrà M , Caccese M , Lombardi G
Published: October 2024

BACKGROUND

NGS panels in oncology offer personalized therapies based on genomic alterations, but data on their clinical use and efficacy for non-glioblastoma CNS tumors is limited.

MATERIAL AND METHODS

This study aimed to explore the molecular landscape of non-glioblastoma CNS tumors in patients (pts) who underwent FoundationOne®CDx testing between 11/2019 and 04/2023 at Veneto Institute of Oncology, Padua (Italy), while also assessing access to TT. Analysis was conducted on formalin-fixed paraffin-embedded tumor samples, comprising a cohort of 176 adult CNS tumor pts. Brain tumors were classified per WHO 2021.

RESULTS

Our cohort was constituted by: 19 Grade 2 IDH mut astrocytomas (A); 35 G3 IDH mut A; 34 G4 A; 29 oligodendrogliomas; 4 diffuse midline gliomas; 3 gangliogliomas; 3 pleomorphic xanthoA; 30 meningiomas; 4 medulloblastomas; 5 ependymomas; 2 neuroblastomas; 3 schwannomas; 4 pituitary adenomas; 1 hemangiopericytoma.The most frequent targettable molecular alterations (ESCAT ESMO Scale for Clinical Actionability of molecular Targets IIB-IIIB) were: PIK3CA/B mutations (14.2%), NF1 and NF2 mutations (10.2 and 13%, respectively), BRCA 1-2 mutations (8%), POLE mutation (7.4%), high tumor mutational burden (TMB) (>10 mut/megabase) (6.8%), PDGFRA alterations (6.2%), BRAF non-V600E alterations (5.1%), RET and ROS1 mutations (3.4% and 2.8% respectively), MDM2 amplification (2.3%), FGFR1-2-3 alterations and H3K28M (1.7%), MET amplification (1.7%), ALK rearrangements (1%). NTRK fusions and BRAF V600E mutations have not been detected. 3 of 4 medulloblastomas pts exhibited a PTCH1 mutation.4 pts received TT at recurrence, within clinical trials: one with grade 3 meningioma and ALK rearrangement treated with alectinib, one with PTCH1 mutant medulloblastoma treated with vismodegib, and two with high TMB treated with nivolumab/ipilumumab. Immune checkpoint inhibitors have shown remarkable activity in a meningioma patient, who is undergoing treatment for 12 months and has achieved a complete response according to RANO criteria, while another has had stable disease with alectinib for 7 months. In other cases TT did not demonstrate activity in controlling disease.

CONCLUSION

The incidence of targettable molecular alterations in adult CNS tumor patients was lower than in GBM. Nevertheless, in a few selected cases TT have the potential to increase treatment options at recurrence and improve outcomes.
P20.17.B PRIMARY CEREBRAL T-CELL LYMPHOMAS DISPLAY AN ATYPICAL PRESENTATION: A LOC STUDY
Authors: NDIAYE M B , Jabeur W , Mokhtari K , Gaulard P , Choquet S , Nichelli L , Mathon B , Le Garff Tavernier M , Soussain C , Moluçon-Chabrot C , Gressin R , Ghesquières H , Houot R , Tricot S , Waultier D , Ahle G , Schmitt A , Emin A , Bonnet A , Xuan K H , Houillier C
Published: October 2024

BACKGROUND

Primary cerebral T-cell lymphomas are very rare and remain poorly known as opposed to diffuse large B-cell lymphomas (DLBCL) that account for more than 90% of all primary central nervous system lymphomas (PCNSL). The aim of the present study was to describe the characteristics of primary T-cell lymphomas, as well as their treatment and outcomes.

MATERIAL AND METHODS

We retrospectively selected from the French LOC (“Lymphomes oculo-cérébraux”) database the cases of primary T-cell lymphomas diagnosed between January 2011 to December 2023.

RESULTS

Of the 2966 patients diagnosed with PCNSL between January 2011 and February 2024, 16 patients (0.54%), including 9 men, were diagnosed with primary T-cell lymphoma. Their median age was 60 years [25-80], their median KPS was 70 [20-90]. The mean delay between first symptoms and diagnosis was 78 days [13-365]. Four patients had a history of auto-immune disease and 2 patients were under immunosuppressive therapy. Six patients harboured a classical aspect of brain lymphoma on MRI (tumor with intense and homogeneous contrast enhancement). In ten patients, the MRI presentation was atypical and other diagnoses were suspected before brain biopsy: high-grade gliomas in six immunocompetent patients with necrotic/hemorrhagic lesions, cerebral vasculitis in 2 patients, gliomatosis cerebri in one patient and cerebral metastasis in one patient. The histological diagnosis was NOS peripheral T-cell lymphoma in 8 patients, anaplastic large-cell lymphoma in 4 patients (2 ALK negative, 1 ALK positive, 1 undetermined), EBV-negative NK-T-cell in 1 patient, T-cell lymphomatosis cerebri in 1 patient (2 missing data). Clonality assessment by PCR found a TCR clonal rearrangement in 5/7 cases.Fifteen of 16 patients were treated with high dose methotrexate-based chemotherapy as induction therapy in first-line treatment. Six patients received intensive chemotherapy with autologous stem cell transplantation during their follow-up Nine patients relapsed, either during first-line treatment (N=3) or during the follow-up after treatment (N=6). The median follow-up was 32 months. The 2-year PFS rate was 46% (95% CI: 26-81) The 2-year OS rate was 67% (46-96) Conclusion: T-cell lymphoma represents a rare and severe form of PCNSL. It often occurs in patients with a different background than in DLBCL: younger patients, history of autoimmune disease or immunosuppression. The diagnosis can be challenging as the radiological presentation is most often atypical, often mimicking high grade gliomas or vasculitis, leading to a delayed diagnosis.
P20.18.A PRIMARY MENINGEAL MELANOCYTIC TUMORS (PMMT): A REVIEW FROM THE ULTRA RARE BRAIN TUMORS DOMAIN OF THE EURACAN
Authors: Pellerino A , Verdijk R M , Bertero L , Nichelli L , Andratschke N H , Idbaih A , Goldbrunner R
Published: October 2024

INTRODUCTION

PMMT are ultra rare entities with distinct histological and molecular features as compared with other melanocytic or pigmented intracranial lesions.

METHODS

The EUropean Network for RAre CANcers (EURACAN) Task Force on Ultrarare Brain Tumors (domain 10) has perfomed a literature revision from January 1985 to December 2023 regarding the evidence of epidemiologic and clinical characteristics, histological and molecular features, radiological findings, and activity of local and systemic therapies.

RESULTS

Molecular analysis can detect specific mutations, including SF3B1, EIF1AX, BAP1, NRAS and BRAF, that are found in PMMT and not in other melanocytic lesions. Neuroimaging shows the leptomeningeal involvement with or without parenchymal lesions with meningeal base implant, delineate the extension with respect to adjacent structures, but cannot reveal the aggressiveness of the disease. Gross-total resection is the first choice in case of circumscribed meningeal melanocytoma and melanoma; conversely, meningeal biopsy may be reserved for patients with diffuse and multinodular leptomeningeal spread to achieve a definitive diagnosis. High-dose radiotherapy is rarely indicated in diffuse melanocytic tumors except as palliative treatment to alleviate symptoms. A definitive advantage of a specific systemic treatment over other could not be concluded, as most of the data available derive from case reports or small cohorts.

CONCLUSIONS

As PMMT are extremely rare, weak correlations between clinical characteristics, molecular profile, radiological findings at diagnosis and progression, and poor evidence on the best therapeutic approach are available thus far. There is the need to develop shared platforms and registries to capture more knowledge regarding these ultra rare entities
P20.19.A RARE CASE OF PRIMARY BRAIN AMYLOIDOMA
Authors: Ivan R , Signate A , Puget S , Sirbu C A
Published: October 2024

BACKGROUND

Cerebral amyloidoma, composed of insoluble fibrillary proteins, is one of the rarest forms of amyloid deposition. It resembles a tumor, with a variable clinical presentation that can include vision loss, behavioral changes, cognitive issues, and recurrent headaches. It has a slow evolution pattern, without characteristics of dissemination.

MATERIAL AND METHODS

We present the case of a 56-year-old patient, who presented with a sensory loss on the right side of the body, progressive memory troubles of one-year duration, and later a left homonym hemianopsia. She underwent a cerebral MRI that showed a right parietal-temporal heterogenous lesion, with contrast enhancement as well as a right parieto-occipital smaller lesion, with a differential diagnostic of glial tumor or cerebral lymphoma. A stereotactic biopsy of the mass was made by the neurosurgeon with a subsequent anatomopathological examination.

RESULTS

The histopathological result was consistent with a cerebral amyloidoma AL with lambda chains, without an associated lymphatic lesion. The systemic extension was also evaluated, without signs of systemic amyloidosis (salivary gland biopsy, thoracic abdominal pelvic scan, myelogram, and a urinary analysis). Hematological and ophthalmological consults were also made.

CONCLUSION

Given the age and the progressive clinical course, the treatment proposed was with Revlimid and Dexamethasone, along with a periodical surveillance with cerebral imaging and neurological consultations. The evolution remained stable at the moment of the publication of this report.
P21.03.B PSYCHOLOGICAL FACTORS AND LONG-TERM TINNITUS HANDICAP IN VESTIBULAR SCHWANNOMA PATIENTS AFTER RETROSIGMOID MICROSURGERY - A CROSS-SECTIONAL STUDY
Authors: Rutenkröger M , Scheer M , Rampp S , Strauss C , Schönfeld R , Leplow B
Published: October 2024

BACKGROUND

This study aimed to investigate the connection between psychological factors and postoperative tinnitus in vestibular schwannoma (VS) patients following retrosigmoid microsurgery.

MATERIAL AND METHODS

Ninety-three VS patients participated, completing questionnaires on demographics, tinnitus severity (THI-12), personality traits (TIPI-G), dizziness impact (DHI), perceived health benefits (GBI),somatisation tendencies (SOMS-2), and psychological distress (HADS-D). Our analysis involved Mann-Whitney U-tests, Spearman’s rank-order correlations, and false discovery rate correction.

RESULTS

Most participants reported postoperative tinnitus (77/93), with 41 experiencing it preoperatively. Emotional stability correlated negatively with tinnitus presence, while tinnitus severity was associatedwith emotional distress. Preoperative somatisation tendencies were also positively linked to tinnitus severity. Postoperative Tinnitus was further linked to reduced perceived health benefits and increased anxiety and depression levels. Notably, age and gender showed no significant associations.

CONCLUSION

This study uncovers the interplay between postoperative tinnitus and psychological factors in VS patients, highlighting emotional and cognitive dimensions. Tailored psychological interventions addressing tinnitus‘s psychosomatic impact may enhance patients quality of life.
P22.04.B BALANCING COMMUNICATION CHALLENGES IN NEURO-ONCOLOGY: A CROSS-SECTIONAL INTERVIEW STUDY WITH NEURO-ONCOLOGY HEALTHCARE PROFESSIONALS
Authors: Legge D M , Jeon M , Banks H , Shaw J , He S , Carlick T , Breen L J , Laidsaar-Powell R , Halkett G K B , Dhillon H
Published: October 2024

BACKGROUND

Primary brain tumours are associated with a significant symptom burden and high supportive care needs, presenting in varied and unique ways along the disease trajectory. Timely and comprehensive patient navigation is vital to ensuring both people with brain tumours (PwBT) and their caregivers have the resources and support needed to negotiate the multidisciplinary team, treatment pathways and survivorship care. We explored HCPs’ experiences and perceptions of the particular challenges in communicating with PwBT and their families. Material and Methods:This study formed part of a qualitative exploratory study investigating the roles, training and support needs of healthcare professionals involved in neuro-oncology care coordination (NOCC) for PwBT and their families. Semi-structured interviews were conducted with HCPs via videoconferencing and were audio-recorded and transcribed. Questions focused on communication experiences and challenges in the NOCC role. Themes were developed in the data using interpretive description with three coders using NVivo v14 software.

RESULTS

Interviews were conducted with 12 HCPs between June and November 2023, lasting a median of 58 (range: 43-82) minutes. Participants were from Australia and Aotearoa New Zealand with a median of 13 years of neuro-oncology clinical experience across medical, nursing, and allied health disciplines. An overarching meta-theme of ‘communication is key’ was identified and supported by three themes describing the elements of communication in neuro-oncology: 1) In safe hands; describing the way care is navigated for patients and families, and the importance of providing a safety net during diagnosis and early treatment; 2) Delicate balance; recognising elements of communication needing to be balanced during each interaction. Balancing hope with reality, family with patient needs, process with time. 3) Creative communication; describing the importance of creative and adaptable communication styles required for NOCC.

CONCLUSION

Our results highlight the unique challenges and complex nature of communicating with PwBT and their families and the pivotal nature of communication in NOCC. The findings will support the development of training and support for health professionals already working within neuro-oncology and assist in defining the core skill set and training needs of health professionals seeking to move into this area of practice.
P22.13.B QUALITY OF LIFE DISPARITIES AMONG VESTIBULAR SCHWANNOMA PATIENTS UNDER DIFFERENT TREATMENT REGIMENS: A SYSTEMATIC REVIEW
Authors: Rutenkröger M , Brandes L , Führes H
Published: October 2024

BACKGROUND

Evaluating the quality of life in vestibular schwannoma patients is crucial for gaining insights into the broader impact of the condition and its treatments. Understanding how vestibular schwannoma affects physical, emotional, and social well-being is essential for comprehensive patient care. Quality of life assessments illuminate the unique challenges faced by patients, including hearing loss and balance issues, guiding healthcare professionals in tailoring treatment plans to improve overall well-being. Furthermore, such evaluations contribute to advancing medical knowledge and developing more patient-centered approaches to managing vestibular schwannoma.

MATERIAL AND METHODS

We conducted a systematic literature search across four databases— MEDLINE, Web of science, Cochrane Library and PSYNDEX—to identify pertinent studies reporting disease-specific quality of life measures (PANQOL) in patients with vestibular schwannoma. Stringent blinding procedures were implemented during the screening stage, yet determinations regarding study eligibility and data extraction were carried out independently by two reviewers. Our overarching goal is to systematically synthesize available data, offering a comprehensive overview of the variations in quality of life among individuals undergoing diverse treatment modalities for this condition. Should a sufficient volume of data be accessible, we intend to progress to a comprehensive meta-analysis.

RESULTS

During our screening process, we initially identified 548 articles, of which 317 have progressed to the ongoing stage of abstract screening. This extensive process aimed to include studies specifically reporting disease-specific quality of life measures in vestibular schwannoma patients.

CONCLUSION

We anticipate presenting comprehensive results from our systematic review at the upcoming conference. The results aim to enhance our comprehension of the multifaceted impact of vestibular schwannoma on patients and establishes a foundation for the development of more personalized, patient-centered strategies in managing patient education and post-treatment care for this condition.
P22.15.B THE DEVELOPMENT OF C-SMART: A NOVEL COGNITIVE REHABILITATION AND MINDFULNESS INTERVENTION FOR PATIENTS WITH BRAIN TUMORS
Authors: Braun S E , Zarrella G , Fox A , Loughan A , Lanoye A , Parsons M , Beach W , Palesh O , Moeller F
Published: October 2024

BACKGROUND

Patients diagnosed with brain tumors experience significant cognitive sequelae, yet they remain underrepresented in behavioral intervention research. We present the development and initial results from the pilot study of C-SMART (Cognitive Strategies, Mindfulness, and Rehabilitation Therapy), an integrated cognitive rehabilitation and mindfulness intervention for patients with brain tumor and mild cognitive decline. We incorporated key stakeholders and content experts’ opinions as a critical first step in developing empirically supported treatments for these underserved patients. We also present preliminary feasibility and acceptability data from an ongoing single-arm pilot study.

METHODS

Following the NIH Stage Model for Behavioral Intervention Development, a Stage Ia study convened two expert advisory boards (EABs), one comprised of brain tumor patients and their caregivers (n=4) and another of neuro-oncology professionals (n=5), to review evidence-based cognitive rehabilitation and mindfulness intervention manuals in other medical populations, identify topic applicability, determine medical relevance, and discuss necessary adaptations for patients with brain tumor. Then, a Stage 1a pilot trial was opened to investigate feasibility (rates of screening, eligibility, enrollment) and acceptability (satisfaction and intervention recommendations) of the resultant, newly developed C-SMART manual and research procedures.

RESULTS

Both EABs recommended adaptations to existing cognitive rehabilitation and mindfulness manuals to meet the distinct needs of patients with brain tumor. As a result, C-SMART’s key components include: 1) neurocognitive evaluation to inform the chosen cognitive rehabilitation strategies, 2) tailored goal-setting to each individual, 3) self-compassion training in the face of cognitive slips, 4) neuropsychological education and strategy training with consideration of disease and treatment characteristics, 5) mindfulness practice prior to strategy use, and 6) caregiver participation. To date, 69% of 16 interested individuals were screened (n=11), 82% were eligible (n=9), and, of those, 89% enrolled (n=8), with 75% also enrolling a caregiver (n=6). Currently, 50% are off trial with a completion rate of 80%. Those who have completed C-SMART have found it beneficial (100%) and would recommend to others (100%).

CONCLUSION

C-SMART is the first consensus-supported, individualized, mindfulness-based, cognitive rehabilitation intervention in neuro-oncology. Initial results support feasibility and acceptability of C-SMART for those diagnosed with brain tumors. The current preliminary results encourage the continued optimization and investigation of C-SMART.
P22.16.A THE EXPERIENCE OF LIVING WITH MALIGNANT MENINGIOMA
Authors: Maier A D , Nordentoft S , Mathiesen T , Guldager R
Published: October 2024

BACKGROUND

Meningiomas are the most common, primary intracranial tumor and most are benign. Little is known of the rare patient group living with a malignant meningioma, comprising 1-3% of all meningiomas. Our aim was to explore how patients perceived quality of daily life after a malignant meningioma diagnosis.

MATERIAL AND METHODS

This qualitative explorative study was composed of individual semi-structured interviews. Eligible patients (n = 12) were selected based on ability to participate in an interview, from a background population of 23 patients diagnosed with malignant meningioma at Rigshospitalet from 2000 to 2021. We performed an inductive thematic analysis following Braun and Clarke’s guidelines.

RESULTS

Eight patients were interviewed. The analysis revealed 4 overarching themes: (1) perceived illness and cause of symptoms, (2) identity, roles, and interaction, (3) threat and uncertainty of the future, and (4) belief in authority. The perceived quality of daily life is negatively impacted by the disease. Patients experience a shift in self-concept and close interactions, and some struggle with accepting a new everyday life. Patients have a high risk of discordant prognostic awareness in relation to health-care professionals.

CONCLUSION

We provide a much-needed patient-centered perspective of living with malignant meningioma: quality of life was affected by perception of threat and an uncertainty of the future. Perception of illness and the interpretation of the cause of symptoms varied between subjects, but a common trait was that patients’ identity, roles, and interactions were affected. Shared decision-making and a strengthened continuity during follow-up could aid this rare patient group.
P22.17.B THE NEURO-ONCOLOGICAL CAREGIVER ROLES AND RESPONSIBILITIES - A QUANTITATIVE SURVEY
Authors: Smith V R , Kjærgård S S , Piil K
Published: October 2024

BACKGROUND

In Denmark, cancer treatment is increasingly offered on an outpatient basis. This adds a responsibility on the caregivers, who often handle treatment agreements, provide 24/7 care, and offer practical support within the home environment. Especially, caregivers of individuals with brain cancer due to the disease’s unique characteristics, including cognitive and physical alterations. Qualitative exploration of the caregiver role has been undertaken, but there remains a notable absence of quantitative measurements regarding the burden faced by neuro-oncological caregivers. Therefore, this study aimed to quantitatively investigate the impact of caregiving on individuals caring for brain cancer patients.

MATERIAL AND METHODS

A cross-sectional study was conducted. The questionnaire used was the validated and standardized questionnaire “Caregiver roles and responsibilities scale” (CRRS). It consists of 51 questions grouped into 6 core areas: Support and Impact, Lifestyle, Emotional Health and Well-being, Self-care, Financial situation, and Work and Career. Responses are scored on a 5-point Likert scale ranging from 0 (“not at all”) to 4 (“very much”). The CRRS questionnaire has an official scoring guideline that calculates a score ranging from 0-152, in which 0 accounts for the most burdened, and 152 the least burdened. However, there isn’t a specific cut-off score to indicate when the CRRS score should raise concern. The questionnaire was distributed for two weeks to all caregivers attending the neuro-oncology outpatient clinic at Rigshospitalet.

RESULTS

Included were 27 primary caregivers (out of 30 eligible). 64% were female, and age was categorized in under 65 (younger) and over 65 (older); with a range from 39 to 80 years old. Data indicated that the younger caregivers (CRRS score = 87), those being in the initial 6 months of the disease (CRRS score = 85), and those receiving minimal to no practical assistance from family and friends (CRRS score = 90) were at a higher risk of burden, compared to those being older (CRRS score = 103), in the stage of 12-24 month post-diagnose (CRRS score = 106) and who received moderate to substantial support (CRRS score = 101). 18% of women rated their physical health as impaired to a large degree due to the patient’s disease, and none of the men noted this.

CONCLUSION

The CRRS questionnaire could serve as a valuable screening tool to identify the specific support required by individual caregivers. The study indicates that female caregivers under the age of 65, particularly within the initial 6 months of the disease onset, and those lacking practical support from friends and family, tend to experience elevated levels of burden. The study’s findings do not specify the frequency or timing for administering the CRRS questionnaire as a screening tool, initial steps could involve integrating the screening process within the first six months of caregiving.
P22.18.A THE SEVERITY OF ANXIETY AND DEPRESSION SYMPTOMS AND QUALITY OF LIFE IN PATIENTS WITH LOW-GRADE GLIOMAS
Authors: Olejnik A , Bala A , Dziedzic T , Marchel A , Kunert P
Published: October 2024

BACKGROUND

Depression, anxiety, and reduced quality of life are frequent problems in patients with brain tumors. Objective of the current study was to examine quality of life level and its correlation with different clinical and demographic factors among patients diagnosed with Low Grade Gliomas (LGG).

MATERIAL AND METHODS

The study involved 55 individuals with a possible diagnosis of low-grade glioma based on MRI examination. The final analysis included only the data collected from 48 patients whose diagnosis of LGG was confirmed by histological and genetic examination, as well as 48 demographically matched healthy control individuals. Before the surgical procedure, when neither the histological nor the genetic diagnosis had been established, each patient filled out a questionnaire to ensure the outcomes would not be affected by this information. The Hospital Anxiety and Depression Scale (HADS), the World Health Organization Quality of Life questionnaire (WHO-QoL-BREF), and a demographic-clinical questionnaire were used in the study.

RESULTS

Intergroup comparisons indicated that patients with LGG reported a lower quality of life than healthy individuals (p < 0.05). According to the HADS scale responses, patients with LGG experienced significantly more severe depression and anxiety symptoms, than healthy individuals (p < 0.001). Correlational analysis revealed a significant negative correlation between almost all subscales of quality of life and the severity of depression symptoms: QoL - physical scale (p < 0.05), QoL - psychological scale (p < 0.05), QoL - social scale (p < 0.05). Additionally, older patients reported a poorer quality of life compared to younger patients. The gender of the participants did not correlate with the quality of life.

CONCLUSION

Patients with LGG experience a decline in quality of life. Objective neurological deficits, epilepsy, as well as awareness of the cancer diagnosis, may be associated with the occurrence of depressive and anxiety symptoms, such as low mood, loss of interest, difficulties in social functioning. Acknowledging these factors and integrating them into the care of patients with LGG is essential for providing sufficient psychological support and improving their psychological well-being. Further research is necessary to assess the quality of life of patients after surgery, to exclude the impact of stress associated with waiting for the procedure, as well as to assess potential changes in quality of life during the recovery period.
P23.04.A DIZZY, DODDERY AND DISCOMBOBULATED: EXPLORING SYMPTOMS OF DIZZINESS AND IMBALANCE IN VESTIBULAR SCHWANNOMA TREATABLE WITH SRS AT A TERTIARY CANCER CENTRE
Authors: Jethwa J , Williams L , Davenport S , Adonis A
Published: October 2024

BACKGROUND

Vestibular schwannoma (VS), a brain tumour of the vestibulocochlear nerve, causing auditory and vestibular symptoms. Dizziness and associated fatigue, cognitive changes and anxiety are shown to have the biggest impact on quality of life and ability to work. The National Institute for Health and Care Excellence (2021) and Tessa Jowel Brain Cancer Mission (2024) recommend patients with brain tumour have early and ongoing access to specialist therapy assessment and rehabilitation. However, assessment of vestibular symptoms is not included in VS guidelines, nor is it included in routine assessment for those with VS at The Royal Marsden Hospital (RMH). Consequently, symptom burden for those with VS is unclear at RMH. Without a defined rehabilitation pathways, this affects equity of access to vestibular rehabilitation locally. The aim of this study was therefore to understand vestibular symptoms in VS, describe the patient profile, and identify improvements to local VS services.

MATERIAL AND METHODS

An observational study assessed vestibular function in VS using a symptom questionnaire, Dizziness Handicap Inventory, oculomotor examination and MiniBESTest. Case-note audits identified demographics, frequency of rehabilitation referral, and rehabilitation waiting times.

RESULTS

Vestibular symptoms (dizziness, oculomotor impairment, and imbalance) and secondary symptoms (anxiety, fatigue and cognitive changes) were frequent. The majority caused mild or moderate functional impact on work or recreation. Rehabilitation need correlated significantly with a DHI score >20 combined with moderate-severe functional impact. Rehabilitation waiting times significantly improved with early physiotherapy assessment. Patients reported many benefits of physiotherapy as part an MDT clinic. RMH VS population was older than the catchment population. There was no gender or race difference.

CONCLUSION

Vestibular symptoms are common and impactful in VS. Prevalence of secondary symptoms appear common and warrant formal investigation. Vestibular symptoms, secondary symptoms, and the functional impact of these should be assessed as standard practice and included in future guidelines. A DHI score >20 combined with moderate/severe functional impact identified rehabilitation need, but should not replace physiotherapy as part of MDT clinic. Demographic findings showing no difference in VS incidence according to race are contrary to previous findings of increase incidence in Caucasian populations.