Brain tumors diagnosed during pregnancy are exceptionally rare, and their clinical progression is not yet fully understood. Managing intracranial tumors during pregnancy necessitates a specialized approach, balancing neuro-oncological considerations with obstetric concerns to evaluate therapeutic options effectively.Tumor Treating Fields (TTF) therapy is a non-invasive, regionally-applied, anti-cancer treatment modality approved for adults with newly diagnosed and recurrent glioblastoma. To date, there is no data on the safety and efficacy of TTF therapy in pregnant patients harbouring malignant brain tumors. To describe a case of TTF-use during pregnancy and provide a first insight into its feasibility. We describe the first reported case of a pregnant women treated with TTF. Medical records and imaging data were analysed. Relevant literature concerning the management of malignant brain tumors in pregnancy was reviewed. The patient diagnosed with a posterior fossa high-grade anaplastic ganglioglioma (CNS WHO grade 3-4) received surgical resection followed by radiotherapy with fetal shielding combined with TTF therapy during pregnancy. Since TTF therapy represents a topical treatment without systemic application, we suggest it as a feasible option for pregnant patients diagnosed with malignant brain tumors.

BRAF V600E mutations occur in many pediatric malignancies, including ~5% to 10% of pediatric high-grade gliomas (HGGs). Despite efforts over the decades, the prognosis of pediatric HGG remains dismal, with low survival rates. Dabrafenib has shown efficacy in pediatric patients with BRAF V600 mutation-positive malignancies in a phase 1/2a study. This report combines data from all pediatric patients with HGG in both dose escalation (part 1) and tumor-specific dose expansion (part 2) parts of the study, including patients with HGG for the first time in phase 2a. Patients aged 1 to < 18 years with BRAF V600 mutation-positive HGG who had refractory or progressive disease after standard therapy received oral dabrafenib 3.0 to 5.25 mg/kg/day (part 1) or the recommended dose (part 2) of 5.25 mg/kg/day (age < 12 years) or 4.5 mg/kg/day (age ≥ 12 years) as 2 equal doses twice daily. The primary objectives were safety and tolerability of dabrafenib (part 1; previously published) and clinical activity (part 2), defined as ORRs reported by investigator assessment and independent review using RANO 2010 criteria. Overall, 35 pediatric patients with HGG were treated. Histologic diagnosis included pleomorphic xanthoastrocytoma (n = 8), glioblastoma (n = 7), anaplastic astrocytoma (n = 6), anaplastic ganglioglioma (n = 4), and other gliomas (n = 10). The ORRs were 29% (95% CI, 14.6, 46.3) and 46% (28.8, 63.4) by investigator assessment and independent review, respectively. The 24-month PFS rates were 30% and 40%, respectively. The most common treatment-related adverse events were dry skin (31%), fatigue (29%), and pyrexia (26%). No treatment-related deaths were reported. In pediatric patients with relapsed orrefractory BRAF V600-mutated HGG, dabrafenib exhibited sustained objective tumor responses and a manageable safety profile.

Bilateral, biventricular lesions present a challenging scenario in neurosurgery, often requiring complex surgical techniques for management. Gangliogliomas (GG), while typically indolent, can manifest as anaplastic variants (AGG), necessitating comprehensive treatment strategies. This case study explores a unique surgical approach for a patient with bilateral, intra-extraventricular lesions infiltrating the corpus callosum, highlighting the complexities of managing such cases. A 63-year-old female presented with a progressive intraventricular lesion infiltrating the left frontal lobe, diagnosed initially as a ganglioglioma. Following resection and histological examination, the lesion was confirmed as a WHO Grade 1 ganglioglioma. Subsequently, a contralateral lesion emerged, necessitating a novel surgical approach to achieve maximal safe resection while minimising neurological deficits. The technique involved extending the surgical corridor contralaterally along the tumour route, guided by neuronavigation and fluorescence imaging. The surgical approach enabled maximal safe resection of the lesion, with postoperative imaging confirming complete resection in most sites except for a known infiltration in the right posterior lateral ventricle. Histological examination revealed AGG, prompting subsequent adjuvant radiotherapy due to its aggressive nature. The management of bilateral, biventricular lesions such as AGG requires innovative surgical approaches tailored to individual patient characteristics. The case highlights the efficacy of a transtumoral approach in achieving maximal safe resection while minimising neurological sequelae. Moreover, it underscores the importance of comprehensive treatment strategies, including adjuvant therapies, in addressing aggressive histological variants of gangliogliomas.

Ganglioglioma (GG) with anaplasia (anaplastic ganglioglioma) is a rare and controversial diagnosis. When present, anaplasia involves the glial component of the tumor, either at presentation or at recurrence. To date, most published cases lack molecular characterization. We describe the histologic and molecular features of 3 patients presenting with BRAF p. V600E-mutant GG (CNS WHO grade 1) with high-grade glial transformation at recurrence. The tumors occurred in pediatric patients (age 9-16 years) with time to recurrence from 20 months to 7 years. At presentation, each tumor was low-grade, with a BRAFV600E-positive ganglion cell component and a glial component resembling pleomorphic xanthoastrocytoma (PXA) or fibrillary astrocytoma. At recurrence, tumors resembled anaplastic PXA or high-grade astrocytomas without neuronal differentiation. CDKN2A homozygous deletion (HD) was absent in all primary tumors. At recurrence, 2 cases acquired CDKN2A HD; the third case showed loss of p16 and MTAP immunoexpression, but no CDKN2A/B HD or mutation was identified. By DNA methylation profiling, all primary and recurrent tumors either grouped or definitely matched to different methylation classes. Our findings indicate that malignant progression of the glial component can occur in GG and suggest that CDKN2A/B inactivation plays a significant role in this process.

Ganglioglioma is a rare neoplasm most common in children and adolescents. It is typically located in the supratentorial compartment, with the temporal lobe being the most common tumor location. Anaplastic ganglioglioma is a WHO grade III ganglioglioma, a rare subtype accounting for a small minority of ganglioglioma cases. Posterior fossa anaplastic ganglioglioma in an adult is incredibly rare; only 3 prior cases have been reported. Only 1 adult anaplastic ganglioglioma in the cerebellum has been reported. We present the second reported adult cerebellar anaplastic ganglioglioma.