Pediatric intramedullary spinal cord tumors: A national cancer database analysis of demographics, patterns of care, and survival.

OBJECTIVE: Query the National Cancer Database (NCDB) to delineate epidemiologic frequency, care patterns, and survival outcomes of pediatric intramedullary spinal cord tumors (IMSCTs). METHODS: IMSCTs included ependymoma, astrocytoma, and hemangioblastoma. We examined data from the NCDB spanning 2004-2018, focusing on IMSCT in children aged 0-21 years. Our analysis included logistic and Poisson regression, Kaplan-Meier survival estimates, and Cox proportional hazards models. RESULTS: This study included 1066 patients aged 0-21 years. 59.4 % of patients were male, while 83.1 % were white. The most common tumor histology was ependymoma (57.5 %), followed by astrocytoma (36.1 %) and hemangioblastoma (6.4 %). 24.9 % of patients received radiotherapy, with radiotherapy utilization being highest among patients aged 6-10 years. Chemotherapy utilization was highest in patients aged 0-5 years. 87.2 % of patients underwent surgical resection, with higher rates in patients aged 16-21 years. Overall survival did not differ significantly between resected and non-resected patients (p = 0.315). Patients in rural areas had worse OS than those in metro areas (HR = 4.42, p = 0.048). Patients with astrocytoma had worse OS compared to other histologies (HR = 2.21, p = 0.003). Astrocytoma patients were over twice as likely to have prolonged LOS compared to ependymoma patients (OR = 2.204, p < 0.001). CONCLUSIONS: In summary, our analysis utilizing the NCDB database provides a comprehensive overview of demographics, care patterns, and outcomes for the largest cohort of pediatric IMSCTs to date. These insights underscore the complexity of managing IMSCTs and emphasize the need for tailored approaches to improve patient outcomes.

Impact of Molecular Subgroups on Prognosis and Survival Outcomes in Posterior Fossa Ependymomas: A Retrospective Study of 412 Cases.

BACKGROUND AND OBJECTIVES: Posterior fossa ependymomas (PFEs) are rare brain tumors classified as PF-EPN-A (PFA) and PF-EPN-B (PFB) subgroups. The study aimed to evaluate the prognosis and survival outcomes in PFEs, with a focus on the impact of molecular subgroups. METHODS: A retrospective study was conducted on 412 patients with PFEs. Kaplan-Meier survival analyses were conducted to evaluate the overall survival (OS) and progression-free survival. Cox regression analyses were conducted to assess the prognostic factors. A nomogram was developed to predict the OS rates of PFEs. RESULTS: The study revealed significant differences between PFA and PFB in patient and tumor characteristics. PFAs were associated with poorer OS (hazard ratios [HR] 3.252, 95% CI 1.777-5.950, P < .001) and progression-free survival (HR 4.144, 95% CI 2.869-5.985, P < .001). World Health Organization grade 3 was associated with poorer OS (HR 2.389, 95% CI 1.236-4.617, P = .010). As for treatment patterns, gross total resection followed by radiotherapy or the combination of radiotherapy and chemotherapy yielded the most favorable OS for PFA ( P = .025 for both), whereas gross total resection followed by radiotherapy rather than observation showed improved OS for PFB ( P = .046). The nomogram demonstrated a high degree of accuracy and discrimination capacity for the prediction of OS rates for up to 10 years. In addition, 6 cases of PFA (3.51%) with H3K27M mutations were identified. CONCLUSION: PFAs demonstrate worse prognosis and survival outcomes compared with PFBs. Both PFAs and PFBs necessitate maximal resection followed by intensive adjuvant therapies in long-term effects.

Recurrence Patterns and Surveillance Imaging in Pediatric Brain Tumor Survivors.

Surveillance magnetic resonance imaging (MRI) is routinely used to detect recurrence in pediatric central nervous system (CNS) tumors. The frequency of neuroimaging surveillance varies without a standardized approach. A single-institutional retrospective cohort study evaluated the frequency of recurrences. This study included 476 patients with the majority diagnosed with low-grade glioma (LGG) (n=138, 29%), high-grade glioma (HGG) (n=77, 16%), ependymoma (n=70, 15%), or medulloblastoma (n=61, 13%). LGG, HGG, and ependymoma patients more commonly had multiply recurrent disease ( P =0.08), with ependymoma patients demonstrating ≥2 relapses in 47% of cases. Recurrent disease was identified by imaging more often than clinical symptoms (65% vs. 32%; P =<0.01). Patients diagnosed with meningioma demonstrated the longest mean time to first relapse (74.7 mo) whereas those with atypical teratoid rhabdoid tumor and choroid plexus carcinoma tended to have the shortest time to relapse (8.9 and 9 mo, respectively). Overall, 22 patients sustained first relapse >10 years from initial diagnosis. With a higher tendency toward detection of tumor recurrence/progression on MRI surveillance in comparison to clinical progression, surveillance imaging is necessary in routine follow up of pediatric CNS tumor survivors. With some relapses >10 years from initial diagnosis, imaging beyond this time point may be useful in particular tumor types. While the study is limited in outcome analysis, earlier detection of recurrence would lead to earlier initiation of treatment and implementation of salvage treatment regimens which can impact survival and quality of life.

Development of Chromosome 1q+ Specific Treatment for Highest Risk Pediatric Posterior Fossa Ependymoma.

PURPOSE: There are no effective treatment strategies for children with highest-risk posterior fossa group A ependymoma (PFA). Chromosome 1q gains (1q+) are present in approximately 25% of newly diagnosed PFA tumors, and this number doubles at recurrence. Seventy percent of children with chromosome 1q+ PFA will die because of the tumor, highlighting the urgent need to develop new therapeutic strategies for this population. EXPERIMENTAL DESIGN: In this study, we utilize 1q+ PFA in vitro and in vivo models to test the efficacy of combination radiation and chemotherapy in a preclinical setting. RESULTS: 5-fluorouracil (5FU) enhances radiotherapy in 1q+ PFA cell lines. Specifically, 5FU increases p53 activity mediated by the extra copy of UCK2 located on chromosome 1q in 1q+ PFA. Experimental downregulation of UCK2 resulted in decreased 5FU sensitivity in 1q+ PFA cells. In in vitro studies, a combination of 5FU, retinoid tretinoin (ATRA), and radiation provided the greatest reduction in cellular proliferation and greatest increase in markers of apoptosis in 1q+ PFA cell lines compared with other treatment arms. Similarly, in vivo experiments demonstrated significant enhancement of survival in mice treated with combination radiation and 5FU and ATRA. CONCLUSIONS: These results are the first to identify a chromosome 1q+ specific therapy approach in 1q+ PFA. Existing phase I studies have already established single-agent pediatric safety and dosages of 5FU and ATRA, allowing for expedited clinical application as phase II trials for children with high-risk PFA.

Ependimoma anaplásico supratentorial cortical. Reporte de caso / Cortical supratentorial anaplastic ependymoma. Case report

Introducción: Los ependimomas son tumores neuroepiteliales que representan del 2 al 9% de todos los tumores del SNC. Existe una variante ectópica que se ubica alejada de las células ependimarias que darían su origen. Esta última variante se presenta más frecuentemente en el compartimiento supratentorial. Objetivo: Reporte de un caso de ependimoma anaplásico occipital cortical y revisión de la literatura de esta entidad con una baja casuística mundial.Reporte de caso: Masculino de 35 años, ex tabaquista, cefalea de 2 meses de evolución y disminución de la agudeza visual. Al examen se constató cefalea holocraneana, mareos y hemianopsia homónima derecha por confrontación. Fondo de ojo (FO): edema de papila bilateral, con hemorragias peripapilares en ojo izquierdo. Estudios neuroradiológicos: lesión cortical occipital izquierda con refuerzo homogéneo post contraste, limites netos y abundante edema perilesional. Screening oncológico negativo. Resección quirúrgica, la pieza quirúrgica presentaba buen plano de clivaje, coloración blanquecina y consistencia duroelástica. Hallazgos vinculables con Ependimoma Anaplásico. Se le realizó tratamiento adyuvante STUPP. Screening de siembra metastásica craneoespinal negativo. Al año post tratamiento no se evidenció recidiva de la lesión, con mejoría del CVC y sin edema de papila en el FO control. Conclusiones: Los ependimomas anaplásicos supratentoriales corticales son una entidad rara. En pacientes jóvenes con imágenes que evidencian lesión cortical única con realce homogéneo post contraste debe considerarse a esta entidad dentro de los diagnósticos diferenciales. La resección quirúrgica es el tratamiento de elección, seguido de RT, en variantes malignas o con resección incompleta.

Introduction: Ependymomas are neuroepithelial tumors, representing 2 to 9% of all CNS tumors. There is an ectopicvariant located away from the ependymal cells that wouldoriginate. This last variable occurs more frequently in thesupratentorial compartment.Objectives: Case report of an occipital cortical anaplasticependimoma. Literature review of this entity with a low globalcasuistry. Case report: Male 35 years, former smoker, headache 2 monthsof evolution and decreased visual acuity. Physical examination found holocraneal headache, dizziness and right homonymoushemianopia by confrontation. Ocular fundus (OF): bilateralpapilledema, peripapilar bleeding in left eye. Neuroradiological studies: left occipital cortical lesion with homogeneousreinforcement after contrast, net limits and abundant perilesionaledema. Negative oncological screening. Surgical resection, Thesurgical specimen showed good cleavage plane, whitish and firm,elastic consistency. Linkable findings with anaplasticependimoma. STUPP Adyuvant therapy was performed. Negativescreening of craniospinal metastatic seeding. During posttreatment year showed no recurrence of injury, with CVCimprovement, without papilledema in the ocular fundus control.Conclusions: Cortical supratentorial anaplastic ependimoma arerare. In young patients with images that show unique corticallesion with homogeneous reinforcement after contrast this entitymust be considered between differential diagnoses. Surgicalresection is the treatment of choice, followed by radiotherapy, inmalignant variants of incomplete resection.

Ependymoma in children: molecular considerations and therapeutics insights

A multi-modality approach that encompasses maximal surgical resection in combination with adjuvant therapy is critical for achieving optimal disease control in children with ependymoma. In view of its complex biology and variable response to therapy, ependymoma remains a challenge for clinicians involved in the care of these patients. Meanwhile, translation of molecular findings can characterize unique features of childhood ependymoma and their natural history. Furthermore, understanding the biology of pediatric ependymoma serves as a platform for development of future targeted therapies. In line with these goals, we review the molecular basis of pediatric ependymoma and its prognostic implications, as well as novel therapeutic advances in the management of ependymoma in children (AU)

Late dissemination of ependymoma: case report

Spinal cord dissemination over 10 years after surgical removal of the fourth ventricle ependymoma without local recurrence is extremely rare. A 49-year-old maleunderwent a macroscopically gross total removal of the fourth ventricle ependymoma and postoperative radiotherapy to the posterior fossa. Twelve years after the initial operation, the patient complained from uncontrolled fever attacks, low back pain and numbness of the legs. Spinal Magnetic Resonance Imaging revealed intradural extramedullary mass lesions located at the thoracic 2-3 and lumbar 5 vertebrae levels. Cerebrospinal fluid examination showed no tumour cells. He underwent total excision of these spinal lesions. Although the majority of there currences take place within a few years after surgery, we experienced a case with multiple spinal disseminations12 years after the resection of the fourth ventricle ependymoma and administration of the radiation therapy to the posterior fossa. Up to our knowledge, this case represents the second unusual late recurrence reported in the literature. We conclude that low grade ependymomas should be followed neurologically and radiologically for more than10 years after the initial treatment

La diseminación raquídea, después de la extirpaciónquirúrgica de un ependimoma del cuarto ventrículo, sin recurrencia local, es muy rara. Un varón de 49 años fue intervenido de un ependimoma del IV ventrículo, con resección total y radioterapia postoperatoria de la fosa posterior. Doce años después de esta intervención, el paciente comenzó a quejarse de episodios febriles incontrolables, dolor lumbar y adormecimiento en las piernas. La resonancia magnética mostraba lesiones localizadas a la altura de la 2-3ª vértebra dorsal y de la L5. El líquido cefalorraquídeo no mostraba células tumorales. Fue operado de ambos tumores raquídeos, con resección total. Aunque la mayoría de las recurrencias tienen lugar en los primeros años después de la operación, hemos observado un caso con diseminación raquídea múltiple, después de la resección de un ependimoma del IV ventrículo y radioterapia de la fosa posterior. Que sepamos, este caso es el segundo de recurrencia tardía, publicado en la literatura. En conclusión, los ependimomas de bajo grado deben ser vigilados neurológica y radiológicamente durante más de diez años después del tratamiento inicial

Thallium-201SPECT assessment in the detection of recurrences of treated gliomas and ependymomas

INTRODUCTION: The aim of this study was to establish the value of thalium-(201) single-photon emission computed tomography ((201)Tl-SPECT) in the detection of recurrences in the follow-up of patients with treated primary neuroepithelial tumours. MATERIAL AND METHODS: Sixty-three (201)Tl-SPECT were performed in 36 patients with glioma (12 males, mean age of 46 +/- 13 years). All patients underwent surgery and adjuvant radiotherapy (and some of them received chemotherapy). All patients were submitted to morphological neuroimaging techniques as well (and (201) Tl-SPECT). Mean follow-up was 18.3 +/- 14.6 months. Gold standard was based on clinical follow-up, therapeutical decisions (at least 4 months after (201)Tl-SPECT) and imaging features. RESULTS: Sensitivity and specificity of (201)Tl-SPECT to detect glioma recurrences were 90% and 100% respectively and 93% accuracy. Sensitivity and specificity for high grade tumours, were 100% respectively. Due to 4 false negatives, sensitivity and specificity for low grade gliomas were 78% and 100%. In the positive (201)Tl-SPECT group of patients overall survival was 13.64% at the end of the study. The negative (201)Tl-SPECT group had 84.62% overall survival at the end of the study (p = 0.0003). CONCLUSIONS. (201)Tl-SPECT is a valuable and noninvasive diagnostic procedure to detect recurrence or progression disease for treated gliomas and ependymomas. (201)Tl-SPECT has a good correlation with short term prognosis with excellent diagnostic accuracy (AU)

Estudio epidemiológico de quistes epidermoides cerebrales en Aragón y La Rioja / An epidemiological study about ependymomas in Aragon and La Rioja

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