Giant Supratentorial Brain Tumors in Children: Functional Outcome and Progression-Free Survival Analysis.

INTRODUCTION: This study aimed to identify factors affecting progression-free survival (PFS) in pediatric patients with giant supratentorial brain tumors (GSBTs) treated with surgical excision. The secondary aim was to analyze how these same factors affected the functional outcome in the long term. METHODS: We performed a retrospective, analytical, single-center cohort study. We included all pediatric patients with GSBT between January 2014 and June 2018. Patients were followed for a minimum of 24 months for the PFS and overall survival (OS) analysis. Functional status score (FSS) was used to assess the functional outcome. RESULTS: We included 27 patients with GSBT, the median age was six (range 2-12), and eleven patients had a grade IV tumor. The 24-month PFS and OS were 51.85% and 74.04%, respectively. A PFS-ending event or treatment failure occurred in 13 patients. We found that patients with postoperative FFS >16 have a worse PFS than patients with a postoperative FSS <15 (HR 4.51; p = 0.03). Patients with more than three surgeries had worse PFS than patients with one or two procedures (HR 11.39; p = 0.004). High-grade tumors were associated with worse PFS than low-grade tumors (HR 1.55; p = 0.04). Finally, patients with CNS infections had worse PFS than patients without that complication (HR 2.70; p = 0.04). CONCLUSIONS: GSBTs in pediatric patients are complex lesions that require multidisciplinary management. Surgical management and quality of life should be considered when choosing the best treatment. Factors influencing long-term PFS were high-grade histopathology, the need for three or more surgeries, postoperative FSS >16, and CNS infections.

The prognostic significance of different degrees of resection of skull base chordoma.

BACKGROUND: Complete resection combined with postoperative radiotherapy is ideal for skull base chordomas. The recent literature suggests that the degree of surgical resection is the most important prognostic factor. METHODS: We retrospectively analyzed the clinical data of 16 patients with initial chordoma treated at our center between August 2015 and December 2021 and conducted a retrospective study on the prognosis of surgical treatment of skull base chordoma between 2013 and 2022. RESULTS: According to the Kaplan-Meier method, there was a significant difference in PFS between patients aged > 50 years and < 50 years, and no significant difference was observed in PFS for tumor involvement of the internal carotid artery, dura, or superior or inferior clivus. However, there was still a correlation with prognosis. As observed in the included literature, the 5-year overall survival rate was significantly higher in patients undergoing total skull base chordoma resection than in those undergoing subtotal resection (STR), which in turn was significantly higher than in those undergoing partial resection (PR). Patients undergoing subtotal resection had significantly better 5-year PFS rates than those undergoing PR. CONCLUSION: Our study shows that gross total resection and STR have better survival in patients with skull base chordomas compared to PR.

Spheno-orbital meningiomas: Is orbit reconstruction mandatory? Long-term outcomes and exophthalmos improvement.

Background: Meningiomas correspond to one-third of all primary central nervous system tumors. Approximately 9% of them are spheno-orbital meningiomas (SOMs), presenting significant clinical symptoms as visual impairment and orbital esthetics. This article aims to evaluate exophthalmos' improvement in a surgical series without orbital reconstruction. Methods: We consecutively included all patients diagnosed with SOM, admitted to a single institution for 10 years. Surgical resection was the standard of care, associated or not with adjuvant radiation therapy. The radiological investigation included preoperative and postoperative head CT or MRI. We quantified proptosis through imaging. Results: Forty patients composed this series, 87.5% were female. Proptosis was the most common presentation (90%), followed by decreased visual acuity (65%), motility deficit (20%), and headache (20%). Gross total resection was achieved in 65% of the procedures. In late outcomes, 78% of the patients maintained or improved visual acuity and 85% maintained or improved headache. Proptosis significantly improved after surgery and along with the follow-up (P < 0.001). Ten patients were submitted to adjuvant RT, six of them after a subtotal resection. All patients of this subgroup had proptosis. It was observed a higher frequency of worse in visual acuity in patients submitted to RT (71% vs. 28%, P = 0.038). Conclusion: Resection of SOM was sufficient to stop the evolution of visual deficit and allowed the improvement of proptosis. Orbital reconstruction does not seem to be an essential step in reducing enophthalmos.

Primary germinoma of the medulla oblongata: illustrative case.

BACKGROUND: Primary central nervous system germinomas of the medulla oblongata are extremely rare and usually have been found in young female Asian patients. The authors present an illustrative case of a patient who presented with severe medullary and posterior cord syndrome, the first South American case published to date, to the authors' knowledge. OBSERVATIONS: Initially, the radiological differential diagnosis did not include this entity. The lesion was located at the obex and exhibited a well-delineated contrast enhancement without hydrocephalus. An emergency decompressive partial resection following functional limits was performed. After histological confirmation, radiotherapy was indicated, with complete remission achieved at a 6-month follow-up. The patient, however, continued to have a severe proprioceptive disorder. The literature review identified 21 other such patients. The mean age for this location was 23 years, with a strong female and Asian origin predilection. All tumors exhibited contrast enhancement, and only one presented with hydrocephalus. LESSONS: In the absence of elevated tumor markers, radiological clues such as a well-delineated, contrast-enhanced lesion arising from the obex, without hydrocephalus, associated with demographic features such as young age, female sex, and Asian heritage, should evoke a high level of suspicion for this diagnosis. Gross total resection must not be attempted, because this tumor is potentially curable with high-dose radiotherapy.

Third ventricle colloid cysts: An endoscopic case series emphasizing technical variations.

BACKGROUND: Colloid cyst treatment with purely endoscopic surgery is considered to be safe and effective. Complete capsule removal for gross total resection is usually recommended to prevent recurrence but may not always be safely feasible. Our objective was to assess the results of endoscopic surgery using mainly aspiration and coagulation without complete capsule resection and discuss the rationale for the procedure. METHODS: A retrospective review was conducted of 45 consecutive symptomatic patients with third ventricle colloid cysts that were surgically treated with purely endoscopic surgery from 1997 to 2018. RESULTS: Mean age was 35.4 years. Male-to-female ratio was 1:1. Clinical presentation included predominantly headache (80%). Transforaminal was the most used route (71.1%) followed by transeptal (24.5%) and interforniceal (4.4%). Capsule was intentionally not removed in 42 patients (93.3%) and cyst remnants were absent on postoperative MRI in 36 (85%). Mild complications occurred in 8 patients (17.8%). Surgery was statistically associated with cyst volume and ventricular size reduction. There were no serious complications, shunts or deaths. Follow-up did not show any recurrence or remnant growth that needed further treatment. CONCLUSION: Gross total resection may not be the main objective for every situation. Subtotal resection without capsule removal seems to be safer while preserving good results, especially in a limited resource environment. Remnants left behind should be followed but tend to remain clinically asymptomatic for the most part. Surgical planning allows the surgeon to choose among the different resection routes and techniques available. Decisions are predominantly based on preoperative imaging and intraoperative findings.

Abordaje interhemisférico contralateral transfalcino para resección de empendimoma supratentorial extraventricular: presentación de caso y análisis de literatura / Contralateral Transfalcine Interhemispheric Approach for Resection of Extraventricular Supratentorial Empendymoma: Case Presentation and Literature Analysis

Introducción: los ependimomas supratentoriales extraventriculares son una entidad sumamente infrecuente, solo 45 casos han sido reportados en la literatura. La mayoría de los ependimomas se localizan a nivel infratentorial e intraventricular, solo en un grupo pequeño de casos no presentan continuidad con el sistema ventricular. El objetivo de este trabajo es describir y presentar el caso de un tumor inusual, cuya importancia radica en la baja prevalencia de casos reportados en la literatura y en la particularidad del abordaje quirúrgico seleccionado. Descripción del caso: paciente de sexo masculino, de 16 años de edad, con diagnóstico de lesión ocupante de espacio sólida-quística, a nivel frontal izquierdo, entre el giro frontal superior y giro del cíngulo. Se optó por realizar un abordaje interhemisférico contralateral transfalcino, logrando la resección total de la lesión tumoral. Se obtuvo el diagnóstico histopatológico de ependimoma Grado II según clasificación de la OMS. Discusión: en base a la información analizada en los diferentes artículos, los hallazgos imagenológicos y anatomopatológicos del caso presentado coinciden con lo relatado en la literatura acerca de los ependimomas supratentoriales extraventricualres. Es imprescindible la utilización de técnicas de inmunohistoquímica para la correcta tipificación del tumor ya que las características del mismo son fácilmente confundibles con otras entidades y su correcta graduación tiene implicancias pronósticas y terapéuticas. Conclusión: los ependimomas supratentoriales extraventricualares son neoplasias sumamente inusuales. La resección quirúrgica es considerada el tratamiento de primera línea para mejorar el pronóstico y la sobrevida. El abordaje interhemisférico contralateral transfalcino nos permitió lograr la exéresis total de la lesión tumoral, favoreciéndonos un adecuado ángulo de trabajo y reduciendo así la transgresión del parénquima cerebral

ntroduction: extraventricular supratentorial ependymomas are an extremely rare entity, only 45 cases have been reported in the literature. Most ependymomas are located at the infratentorial and intraventricular level, only in a small group of cases don ́t present continuity with the ventricular system. The aim of this paper is to describe and to present the case of an unusual tumor, the importance lies in the low prevalence of cases reported in the literature and in the particularity of the selected surgical approach. Case description: a 16-year-old male patient with a diagnosis of a solid-cystic space-occupying lesion, at the left frontal level, between the superior frontal gyrus and the cingulate gyrus, measuring 40mm x 50mm x 60mm. A contralateral transfalcine interhemispheric approach was chosen, achieving total resection of the lesion. The histopathological diagnosis of Grade II ependymoma was obtained according to WHO. Discussion: based on the information analyzed in the different articles, the imaging and pathological findings of the case presented coincide with what is reported in the literature about supratentorial extraventricular ependymomas. The use of immunohistochemical techniques is essential for the correct typing of the tumor since its characteristics are easily confused with other entities and its correct graduation has prognostic and therapeutic implications Conclusion: extraventricular supratentorial ependymomas are extremely rare neoplasms. Surgical resection is considered the first-line treatment to improve prognosis and survival. The contralateral transfalcine interhemispheric approach allowed us to achieve a total resection of the lesion, favoring an adequate working angle and thus reducing the transgression of the brain parenchyma

Survival and prognostic factors in childhood medulloblastoma: A Brazilian single center experience from 1995 to 2016.

BACKGROUND: Medulloblastoma is the most common malignant brain tumor in the pediatric population. Despite prognosis improvement in the past two decades, one-third of the patients still remain incurable. New evidence suggests that medulloblastoma comprises four distinct entities; therefore, treatment de-escalation is required. The aim of this article is to evaluate epidemiological data from patients treated at our institution. The primary objective is to analyze overall survival (OS) and event-free survival (EFS) and the secondary objective is to identify prognostic factor from this cohort. METHODS: We retrospectively analyzed 69 patients who underwent surgical resection for medulloblastoma among 423 children from the tumor registry data bank of Santo Antônio Children's Hospital from 1995 to 2016. Kaplan-Meier method and Cox regression analysis were used to identify OS, EFS, and prognostic factors. RESULTS: The 5-year OS and EFS rates found were 44.5% and 36.4%, respectively. The extent of resection and radiotherapy as adjuvant treatments was positively correlated to outcome while metastatic disease at diagnosis was negatively related to OS. Age younger than 3 years old did not have a worse outcome in our cohort. CONCLUSION: Similar results to population-based studies were found, but we still face difficulties due to living in a developing country. In the near future, we look forward to new diagnostic techniques that will enable us to classify medulloblastomas according to molecular subgroups.

Chordoid Glioma of the Third Ventricle, a Rare Tumor with an Unexpected Outcome

Chordoid glioma is a rare tumor of the third ventricle whose imaging features are difficult to distinguish from other more common lesions in this location. There are only 83 cases described so far in the literature. Although gross total resection (GTR) is the treatment of choice, immediate postoperative mortality with this approach can be as high as 29%, and morbidity among survivors can reach 67%. We report a case of a male patient of advanced age, with a third ventricle mass lesion, who presented with a progressive right temporal hemianopia. Imaging was compatible with craniopharyngioma, meningioma or even metastasis. Chordoid glioma was not considered in the differential diagnosis. The patient underwent surgery and GTR was achieved. There were no postoperative complications, and the patient was discharged from the hospital three weeks later. Unexpectedly, two days afterwards, he suffered a major brainstem hemorrhagic stroke and, unfortunately, died.

O glioma cordoide é um tumor raro do terceiro ventrículo, e as suas características imagiológicas são difíceis de distinguir de outras lesões mais comuns nesta localização. Até a data presente, existem apenas 83 casos de gliomas cordoides descritos na literatura. A remoção macroscópica total destes tumores deve ser o tratamento de escolha; no entanto, a mortalidade pós-operatória imediata pode chegar aos 29%, e a morbilidade pode atingir os 67% entre os sobreviventes. Nós descrevemos o caso de um homem idoso com uma lesão tumoral no terceiro ventrículo, que se manifestou com uma hemianopsia temporal direita progressiva. Os exames de imagem eram compatíveis com craniofaringioma, meningioma ou até metástase. O glioma cordoide não foi considerado como uma das hipóteses no diagnóstico diferencial inicial. O paciente foi submetido a cirurgia, tendo-se obtido a remoção macroscópica total. Não houve qualquer complicação no período pós-operatório, e o paciente teve alta hospitalar após três semanas. Inesperadamente, dois dias após a alta clínica, o paciente sofreu um AVC hemorrágico do tronco cerebral, e acabou por falecer.

Presentación de un paciente con fascitis nodular pseudosarcomatosa del cuero cabelludo / Presentation of a Patient with Pseudosarcomatous Nodular Fasciitis of the Scalp

La fascitis nodular es una enfermedad infrecuente caracterizada por una tumoración de crecimiento rápido, curso benigno y características pseudosarcomatosas, descrita por Konwaler como fibromatosis pseudosarcomatosa subcutánea, frecuente en las partes blandas en extremidades superiores y tronco; en el cráneo es rara. El pronóstico es bueno y si la exéresis es completa se logra la curación total. Se presentó un paciente de 35 años, con lesiones multinodulares de seis años de evolución, única al inicio, con aumento de tamaño, número de lesiones y cambios de coloración luego, que ocuparon ambas regiones frontales y parietales, firmes, rosáceas, poco movibles. La tomografía axial computarizada de cráneo reveló lesiones multinodulares frontales en partes blandas con erosión de tabla externa. Se realizó tratamiento quirúrgico con exéresis total de la lesión e injerto en un segundo tiempo. La biopsia informó fascitis nodular pseudosarcomatosa del cuero cabelludo. Luego de dos años la lesión no ha recidivado.

The nodular fasciitis is an uncommon fast growth disease, benign and pseudosarcomatous, described by Konwaler as subcutaneous pseudosarcomatous fibromatosis, frequent in the upper extremities and the trunk, and rare in the scalp. The outcome is good if the resection is completely performed, and the cure is total. A 35- year- old patient with multinodular lesions six years ago of progress in the time was presented, which was in the frontal and parietal zone of the skull. The CT scan showed a little erosion of the bone. A total resection of the lesion was done. The biopsy showed pseudosarcomatous nodular fasciitis totally. No relapse in two years.