Outcomes and surgical approaches for pineal region tumors in children: 30 years' experience.

OBJECTIVE: Pineal region tumors account for 2.7%-11% of all CNS tumors in children. In this series, the authors present their surgical results and long-term outcomes from a pediatric pineal region tumor cohort. METHODS: A total of 151 children aged 0-18 years were treated from 1991 to 2020. Tumor markers were collected in all patients; if positive, chemotherapy was performed, and if negative, biopsy was performed, preferably endoscopically. Resection was performed when there was a residual germ cell tumor (GCT) lesion after chemotherapy. RESULTS: The distribution based on histological type, as verified by markers, biopsy, or surgery, was germinoma (33.1%), nongerminomatous GCT (NGGCT) (27.2%), pineoblastoma (22.5%), glioma (12.6%), and embryonal tumor (atypical teratoid rhabdoid tumor) (3.3%). A total of 97 patients underwent resection, and gross-total resection (GTR) was achieved in 64%; the highest GTR rate (76.6%) was found in patients with GCTs, and the lowest (30.8%) was found in those with gliomas. The supracerebellar infratentorial approach (SCITA) was the most common, performed in 53.6% of patients, followed by the occipital transtentorial approach (OTA), performed in 24.7% of patients. Lesions were biopsied in 70 patients, and the diagnostic accuracy was 91.4. The overall survival (OS) rates at 12, 24, and 60 months as stratified by histological type were 93.7%, 93.7%, and 88% for patients with germinomas; 84.5%, 63.5%, and 40.7% for patients with pineoblastomas; 89.4%, 80.8%, and 67.2% for patients with NGGCTs; 89.4%, 78.2%, and 72.6% for patients with gliomas; and 40%, 20%, and 0% for patients with embryonal tumors, respectively (p < 0001). The OS at 60 months was significantly higher in the group with GTR (69.7%) than in the group with subtotal resection (40.8%) (p = 0.04). The 5-year progression-free survival was 77% for patients with germinomas, 72.6% for patients with gliomas, 50.8% for patients with NGGCTs, and 38.9% for patients with pineoblastomas. CONCLUSIONS: The efficacy of resection varies by histological type, and complete resection is associated with higher OS rates. Endoscopic biopsy is the method of choice for patients presenting with negative tumor markers and hydrocephalus. For tumors restricted to the midline and with extension to the third ventricle, a SCITA is preferred, whereas for lesions with extension toward the fourth ventricle, an OTA is preferred.

Prognostic factors of pediatric pineal region tumors at a single institution.

PURPOSE: This study aimed to identify factors of a worse prognosis among different histological types of pineal region tumors in pediatric patients treat at a single institution in a 30-year period. MATERIAL AND METHODS: Pediatric patients (151; < 18 years of age) treated between 1991 and 2020 were analyzed. Kaplan-Meyer survival curves were created, and the log-rank test was used to compare the main prognostic factors in the different histological types. RESULTS: Germinoma was found in 33.1%, with an overall 60-month survival rate of 88%; the female sex was the only factor of a worse prognosis. Non-germinomatous germ cell tumors were found in 27.1%, with an overall 60-month survival rate of 67.2%; metastasis upon diagnosis, residual tumor, and the absence of radiotherapy were associated with a worse diagnosis. Pineoblastoma was found in 22.5%, with an overall 60-month survival rate of 40.7%; the male sex was the only factor of a worse prognosis; a tendency toward a worse outcome was found in patients < 3 years of age and those with metastasis upon diagnosis. Glioma was identified in 12.5%, with an overall 60-month survival rate of 72.6%; high-grade gliomas were associated with a worse prognosis. Atypical teratoid rhabdoid tumors was found in 3.3%, and all patients died within a 19-month period. CONCLUSION: Pineal region tumors are characterized by the heterogeneity of histological types, which exert an influence on the outcome. Knowledge of the prognostic factors for each histological types is of extreme importance to the determination of guided multidisciplinary treatment.

Endoscopic Laser Fenestration to Treat a Bobble-Head Doll Syndrome Caused by Suprasellar Cyst.

The bobble-head doll syndrome (BHDS) is a rare acquired head movement disorder characterized by up and down or side-to-side movement, most commonly seen in the first decade of life. The syndrome occurs more often in lesions causing third ventricle dilatation such as suprasellar or third ventricle cyst, but it is also found in other pathologies associated with hydrocephalus like shunt dysfunctions, trapped fourth ventricle, congenital aqueductal stenosis, Dandy-Walker syndrome, and cerebellar malformations. The pathophysiology of this head movement has different origins theories; one states that this stereotyped movements empties the cyst and move the dome away from the foramina of Monro, which relieves the symptoms of hydrocephalus; the other suggests that the extrapyramidal tracts (rubrotegmentospinal and reticulospinal) are stimulated by the compression of dorsomedial nucleus of the thalamus by the cyst, whose tracts innervate the neck muscles resulting in the bobbling head movements. This video (Video 1) presents a clinical case of BHDS caused by suprasellar cyst in a 10- year-old boy treated by endoscopic procedure. A ventricular-cyst-cisternostomy was performed resulting in complete improvement of the head movements and uneventful recovery. Postoperative images demonstrate decreasing of the cyst lesion and resolution of the hydrocephalus.

Ruptured Bilateral Pericallosal Artery Aneurysm in Mirror Position: Case Report

Introduction Pericallosal artery (PA) aneurysms represent 2 to 9% of all intracranial aneurysms, and their management remains difficult. Objective The aim of the present study is to describe the case of an adult woman with subarachnoid hemorrhage and bilateral PA aneurysm in mirror position. Case Report A 46-year-old woman was referred to our institution 20 days after a sudden severe headache. She informed that she was treating her arterial hypertension irregularly, and consumed 20 cigarettes/day. The patient was neurologically intact at admission. A non-contrast computed tomography (CT) on the first day of the onset of the symptoms revealed hydrocephaly and subarachnoid hemorrhage (Fisher III). An angio-CT/digital subtraction arteriography showed bilateral PA aneurysms in mirror position. The patient was successfully treated with surgery via the right interhemispheric approach (because the surgeon is right-handed); the surgeon performed the proximal control with temporary clipping, and introduced an external ventricular drain at the end of the surgery. The patient was discharged on the fourth postoperative day without any additional neurological deficits or ventricular shunts. Conclusion Ruptured PA aneurysm is a surgically challenging aneurysm due to the many anatomical nuances and risk of rebleeding. However, the operativemanagement of ruptured bilateral PA aneurysms is feasible and effective.

Aneurysm of the Fenestrated Basilar Artery: Case Report and Literature Review

Introduction Aneurysms of the vertebrobasilar junction are rare, but when present, they are often associated with fenestration of the basilar artery. Frequently, the endovascular treatment is the first choice due to the complex anatomy of the posterior fossa, which represents a challenge for the open surgical treatment alternative. Case Report A 47-year-old man was admitted to the emergency unit with headache, diplopia, neck pain and mental confusion. The neurological exam showed: score of 15 in the Glasgow coma scale (GCS), no motor or sensitivity deficit, palsy of the left sixth cranial nerve and Hunt-Hess grade III. The computed tomography (CT) scan showed subarachnoid hemorrhage (Fisher III) and hydrocephalus. The patient was submitted to ventricular-peritoneal shunt. A diagnostic arteriography was performed with 3D reconstruction, which showed evidence of fenestration of the basilar artery associated with aneurysm in the right vertebrobasilar portion. An aneurysm coil embolization was performed without complications. The patient was discharged 19 days later maintaining diplopia, with paralysis of the left sixth cranial nerve, but without any other complaints or neurological symptoms. Discussion Fenestration of the basilar artery occurs due to failure of fusion of the longitudinal neural arteries in the embryonic period, and it is associated with the formation of aneurysms. The endovascular treatment is the first choice and several techniques are described, including simple coiling, balloon remodeling, stent-assisted coiling, liquid embolic agents and flow diversion devices. The three-dimensional rotational angiography (3DRA) is an extremely helpful tool when planning the best treatment course. Conclusion Fenestrated basilar artery aneurysms are rare and complex vascular diseases and their treatment improved with the advent of the 3D angiography and the development of the endovascular techniques.

Introdução Aneurismas da junção vertebrobasilar são raros, mas quando presentes, geralmente estão associados à fenestração da artéria basilar. Frequentemente, o tratamento endovascular é a primeira opção devido à complexidade da anatomia da fossa posterior, o que representa um obstáculo para a alternativa de tratamento com cirurgia aberta. Relato de Caso Um homem de 47 anos de idade deu entrada na unidade de emergência com cefaleia, diplopia, dor no pescoço e desorientação. O exame neurológico mostrou: 15 pontos na escala de coma Glasgow (ECG), ausência de déficit motor ou de sensibilidade, paralisia do sexto nervo craniano I esquerdo, Hunt-Hess grau III. A tomografia computadorizada apresentou hemorragia subaracnoidea (Fisher grau III) e hidrocefalia. O paciente foi submetido a shunt ventricular-peritoneal. A arteriografia diagnóstica foi feita com reconstrução 3D, que comprovou fenestração da artéria basilar associada a aneurisma na porção vertebrobasilar direita. Realizamos embolização do aneurisma com molas, sem complicações. O paciente recebeu alta 19 dias depois, mantendo diplopia, paralisia do sexto nervo craniano esquerdo, sem outras complicações ou sintomas neurológicos. Discussão A fenestração da artéria basilar ocorre devido à falência da fusão das artérias neurais longitudinais no período embrionário e está associada à formação de aneurismas. O tratamento endovascular é a primeira opção e várias técnicas são descritas, incluindo simples embolização, remodelagem por balão, embolização assistida com stent, agentes embólicos líquidos e dispositivos de desvio de fluxo. Para planejar o melhor tratamento, angiografias rotacionais 3D são extremamente úteis. Conclusão Aneurismas de artéria basilar fenestrada são doenças vasculares raras e complexas, e seu tratamento foi aprimorado com o advento de angiografias 3D e desenvolvimento de técnicas endovasculares.

Intradural Schwannoma Exacerbating the Symptoms of Degenerative Lumbar Stenosis: Case Report

Introduction Schwannoma is a common intradural slow-growing, benign and encapsulated tumor that originates from the myelin sheaths of the nerve fibers. However, a lumbar schwannoma complicating the symptoms of spinal stenosis is an extremely rare association. Aim To describe the case of a woman presenting a lumbar schwannoma in association with spinal stenosis. Case Report A 53 year-old female was referred to neurosurgical evaluation due to the worsening of a lumbar pain that was irradiating to the left inferior leg along the anterolateral surface. A neurological examination revealed motor deficits for extension of the left leg and attenuation of the left patellar reflex. Magnetic resonance imaging (MRI) showed lumbar spinal stenosis due to flavum ligament hypertrophy and disc herniation in the L3L4 and L4L5 segments, and an expansive lesion with homogeneous contrast enhancement occupying the left neuroforamen of the L3L4 segment. The patient underwent surgical resection of the tumor and decompression of the stenotic segments with posterior screw instrumentation from L3 to L5. She presented an uneventful recovery and significant improvement of the lumbar pain, and was still free of symptoms 6 months after surgery. An anatomopathological examination defined the tumor as a schwannoma (Grade I ­ World Health Organization [WHO]). Conclusion The present study highlights that lumbar schwannoma is a possible etiology complicating the symptoms of patients with previous lumbar spinal stenosis. It is important to treat both pathologies to improve the patients' symptoms.

Introdução O schwannoma é um tumor intradural comum, benigno, de crescimento lento e encapsulado que se origina da bainha de myelina das fibras nervosas. No entanto, a presença de um schwannoma lombar complicando os sintomas de estenose do canal medular é uma associação extremamente rara. Objetivos Descrever o caso de uma paciente portadora de schwannoma lombar exacerbando os sintomas de estenose do canal lombar. Relato de Caso Uma mulher de 53 anos de idade foi encaminhada para avaliação neurocirúrgica devido a relato de piora dos sintomas de dor lombar que irradiavam preferencialmente para o membro inferior esquerdo na sua face antero-lateral. O exame físico neurológico revelou a presença de déficit motor para a extensão do membro inferior esquerdo e redução do reflexo patelar esquerdo. A imagem de ressonância magnética mostrou a presença de estenose do canal lombar devido à hipertrofia do ligamento amarelo e herniações discais nos segmentos L3L4 e L4L5. Além disso, o estudo radiológico também evidenciou a presença de uma lesão expansiva ocupando o neuroforamen de L3L4 com captação homogênea do meio de contraste. A paciente foi submetida à ressecção cirúrgica do processo neoplásico e descompressão do canal medular com artrodese através de instrumentação dos segmentos L3L4L5 via posterior em um único ato operatório. A paciente apresentou uma recuperação pós-operatória adequada e mantém-se assintomática em segmento clínico seis meses após o procedimento cirúrgico. O estudo anatomo-patológico e imuno-histoquímico definiu o processo expansivo como schwannoma (Grau I da OMS). Conclusão O presente estudo destaca que o schwannoma lombar é uma possibilidade etiológica para os pacientes portadores de estenose do canal lombar que apresentam piora progressiva dos sintomas ou novos déficits neurológicos. É importante tratar as duas patologias para que o paciente obtenha melhor resultado clínico no controle dos sintomas.