Brain Tumor Heterogeneity

Tumor heterogeneity is the concept that different tumor cells provide distinct biomorphological lesions, gene expressions, proliferation, microenvironment and graduated capacity of metastatic lesions. Brain tumor heterogeneity has been recently discussed about the interesting interaction of chronic inflammation, microenvironment, epigenetics and glioma steam cells. Brain tumors remain a challenge with regards to medication and disease, due to the lack of treatment options and unsatisfactory results. These results might be the result of the brain tumor heterogeneity and its multiple resistance mechanisms to chemo and radiotherapy.

Nonsaccular aneurysms: A wide comparison between the four main types.

BACKGROUND: The present study aims to present the most important considerations when it comes to patients features, clinical presentation, localization, morphology, pathogenesis, and the best treatment for each type of the nonsaccular aneurysms. METHODS: We performed a literature review using PubMed. The search was limited to the studies published in English, from 2010 to 2017. RESULTS: Data about the clinical presentation; the aneurysm pathogenesis, morphology, and localization; the patient features; and about the surgical or endovascular approach were analyzed for the four types of nonsaccular aneurysms presented. CONCLUSION: All types of nonsaccular aneurysms have a higher prevalence in young adults. Men are more affected by fusiform and mycotic aneurysms while women suffer more with blister-like aneurysms (BLAs). The mycotic and the BLAs affect more the anterior circulation while the fusiform affects more the posterior circulation. Mycotic and blister-like has as its major complication and clinical presentation the hemorrhage; however, the fusiform aneurysms usually present ischemia and/or mass effect. The reconstructive endovascular techniques for all types of nonsaccular aneurysms presented as the treatment with the best outcomes. Among this technique, the flow diversion presented good results in all types of aneurysms and it seems to promote the best outcomes.

Fusiform aneurysms: A review from its pathogenesis to treatment options.

BACKGROUND: This study aims to present the most important considerations when it comes to patients features, clinical presentation, localization, and morphology of the aneurysm and the treatments outcomes of the fusiform aneurysms. METHODS: We performed a literature review using PubMed. The search was limited to the studies published in English, from 2003 to 2017. RESULTS: The studies analyzed that showed data about the patient features, clinical presentation, the aneurysm localization, morphology, and pathogenesis didn't present much divergence. The surgical and the endovascular approach showed similar treatments outcomes. The reconstructive techniques seem to be safer than the deconstructive. The flow diversion is a technique that showed great results. CONCLUSION: Most of the patients are men, younger than 50 years old, pediatric patients are the most affected. Surgical procedures still have an important place in this field. Reconstructive and deconstructive techniques are both effective; the reconstructive techniques are possibly safer than deconstructive techniques. The most important feature of an aneurysm to predict a bad prognose is to determine if the aneurysm is ruptured. The reconstructive EVT accompanied by dual antiplatelet after and before the procedure showed the best results to treat the basilar fusiform aneurysms. Deconstructive treatment including posterior inferior cerebellar artery occlusion should be considered.

Giant mucopyocele associated with intracranial hypertension: Case report and literature review.

BACKGROUND: Mucoceles are epithelial lined sacs that contain mucous. Eventually, they can be infected and so called mucopyoceles, which are usually slow growing lesions with common bone destruction located in the facial sinus. Mucoceles show multivariate etiology and occur between the fourth and seventh decade of life. CASE DESCRIPTION: Patient, 55-year-old, female, was referred unconscious with Cushing's triad to our department; she had fever since four days. The findings of skull computer tomography highlighted a large bifrontal lesion with an invasion of the rear wall of the frontal sinus, compression of the frontal lobes, and midline deviation. She was taken for an emergency surgery, which showed invasion of the dura and mucous infection. Postoperatively, there was a fast recovery of neurologic level and extubation on the second postoperative day. She took antibiotics for 14 days and was discharged from the hospital without neurologic deficits. CONCLUSIONS: Mucopyoceles are usually slow growing lesions that rarely increases rapidly. Our patient presented signs of intracranial hypertension; therefore, it was necessary to have quick surgical intervention.

McCune-Albright syndrome with craniofacial dysplasia: Clinical review and surgical management.

BACKGROUND: Fibrous dysplasia (FD) is a benign fibro-osseous lesion related to an abnormal bone development and replacement by fibrous tissue. FD has three clinical patterns namely monostotic, polyostotic, and the McCune-Albright syndrome (MAS). MAS is a rare genetic disorder (about 3% of all FD's) that comprises a triad of polyostotic FD, café-au-lait skin macules, and precocious puberty. MAS can involve the orbit region and cause stenosis in the optic canal, leading the patient to a progressive visual loss. METHODS: We reported a case of craniofacial FD in MAS in a 9-year-old male with progressive visual loss, submitted to optic nerve decompression by fronto-orbito-zygomatic approach, with total recovery. A research was made at Bireme, PubMed, Cochrane, LILACS, and MEDLINE with the keywords: FD/craniofacial/McCune-Albright/Optic compression for the clinical review. RESULTS: A clinical review of the disease was made, the multiple, clinical, and surgical management options were presented, and the case report was reported. CONCLUSION: MAS is a rare disease with a progressive polyostotic FD. Whenever it affects the orbit region, the optic canal, and it is associated with a progressive visual loss, the urgent optic nerve decompression is mandatory, either manually or with a rapid drill. It is known that aggressive approach is associated with less recurrence; it is also associated with worsening of the visual loss in optic nerve decompression. In MAS cases, multiple and less aggressive surgeries seem to be more suitable.

Schwannomas of Peripheral Nerves: Unusual Cause of Sciatic and Literature Review / Schwanomas dos nervos periféricos: causa rara de ciatalgia e revisão da literatura

Schwannomas of peripheral nerves are the most common benign tumors in this location; it has specific radiologic and histologic features that enable its fundamental distinction of neurofibromas. Given its importance in neurosurgical practice, known radiologic and histologic criteria must be updated regarding this disease entity, as well as its therapeutic strategy and results obtained with it. This article reviews this subject, followed by a case report considered out of the ordinary, in which the tumor was located in the sciatic nerve in a patient with neurofibromatosis type 1, treated by microsurgical resection, which evolved favorably.

Os schwannomas dos nervos periféricos são os tumores benignos mais comuns nesta localização. Apresentam características radiológicas e histológicas específicas que possibilitam a sua fundamental distinção dos neurofibromas. Dada a sua importância na prática neurocirúrgica, devem ser conhecidos os critérios radiológicos e histológicos atualizados à respeito desta entidade nosológica, bem como sua estratégia terapêutica e o resultado com ela obtido. Neste artigo, é feita uma revisão do tema, seguido do relato de um caso considerado fora do habitual, no qual o tumor localizava-se no nervo ciático, em uma paciente portadora de neurofibromatose tipo 1, tratada por meio de ressecção microcirúrgica, que evoluiu de forma favorável.

Meningocele sacral anterior associada a fístula retotecal e meningite polimicrobiana: relato de caso / Anterior sacral meningocele associated with polymicrobial meningitis and rectothecal fistula: case report

Meningoceles sacrais anteriores são exemplos raros de disrafismo espinhal, originados da herniação do saco dural, através de um defeito ósseo na parede anterior sacrococcígea. Essa condição foi descrita pela primeira vez em 1837 por Bryant, e existem, aproximadamente, 250 casos relatados até os dias atuais. A tríade defeito ósseo, malformação anorretal e massa pré-sacral configura a síndrome de Currarino, e tal massa pode consistir de um tumor, de uma meningocele sacral anterior ou da associação de ambos. Tal síndrome apresenta incidência desconhecida, e acredita-se que seja uma desordem de transmissão autossômica dominante. O diagnóstico ainda é desafiador, apesar da evolução dos exames de imagem. Tomografia computadorizada (TC) e ressonância magnética (RNM) são primordiais, sendo a última o melhor exame para caracterização. A radiografia simples pode ajudar demonstrando o defeito ósseo sacral e o sacro em ?cimitarra?, sendo indicada para triagem de transmissão familiar. A maioria dos pacientes apresenta constipação e sintomas vesicais compressivos. A meningite é uma complicação rara e séria da meningocele sacral anterior, e a associação entre fístula retotecal e pneumoencéfalo foi relatada em apenas dois artigos de língua inglesa. O tratamento deve ser cirúrgico, uma vez que não há possibilidade de fechamento espontâneo. Em casos não tratados ou com atraso diagnóstico, a mortalidade é superior a 30% quando associados à infecção. Descrevemos um caso de meningocele sacral anterior acometendo uma jovem de 17 anos, assintomática até então, que apresentou meningite polimicrobiana como quadro inicial devido a uma fístula retotecal...

Anterior sacral meningoceles are rare examples of spinal dysraphism, originated from the dural sac herniation through a bone defect in the anterior wall of the sacrococcygeal bone. This condition was first described in 1837, by Bryant, and there are, approximately, 250 cases reported until today. The triad bone defect, anorectal malformation and presacral mass configure the Currarino syndrome, and such mass may consist of a tumor, an anterior sacral meningocele or combination of both. The incidence is unknown and it is believed to be an autosomal dominant disorder. The diagnosis is still challenging, despite the evolution of imaging. CT and MRI are essential, although the MRI is the best test for characterization. Plain radiographs can help demonstrate the sacral bone defect and the ?scimitar? sacrum, and they are suitable for familial transmission screening. Most patients have constipation and compressive bladder symptoms. Meningitis is a rare and severe complication of anterior sacral meningocele and association of rectothecal fistula and pneumocephalus was reported in only two articles in the English language. The surgical treatment must be performed, since there is no possibility of spontaneous closure. In cases not treated or with late diagnose, mortality is higher than 30% when associated with infection. We describe a case of anterior sacral meningocele, involving a 17-year-old girl, asymptomatic until then, with polymicrobial meningitis as initial clinical feature due to a rectothecal fistula...

Evoluçäo maligna de um ganglioglioma: relato de caso / Malignant evolution of a ganglioglioma: case report

Relatamos o caso de um paciente de oito anos de idade, com quadro clínico de cefaléia, náuseas e vômitos. A angiografia cerebral mostrava massa frontal nao vascularizada. O paciente foi submetido a craniotomia, com remoçao da lesao cujo estudo anátomo-patológico comprovou o diagnóstico de ganglioglioma. O paciente recebeu radioterapia no pós-operatório. Durante o seguimento, 16 anos após, houve recidiva da lesao, comprovada pela tomografia computadorizada do crânio, sendo submetido a nova cirurgia, cujo exame anátomo-patológico revelou ser glioblastoma multiforme. Gangliogliomas sao tumores raros do sistema nervoso central, contendo mescla de células neuronais e gliais. A anaplasia ocorre somente no componente glial, sendo este, portanto, responsável pelo prognóstico desta lesao.

Melanomas metastáticos intracranianos: análise de 13 casos / Malignant melanoma brain metastases: analysis of 13 cases

Os autores apresentam análise retrospectiva de 13 pacientes com o diagnóstico de melanoma metástico intracerebral. A lesäo primária foi detectada em 9 pacientes. A idade dos pacientes variou entre 28 e 84 anos. O tempo transcorrido entre o diagnóstico da lesäo primária e a metástase cerebral variou entre 6 e 60 meses. Todos os pacientes foram submetidos a craniotomia, com remoçäo da lesäo. A mortalidade nos primeiros 30 dias foi zero. A sobrevida dos pacientes variou entre 2 e 55 meses. O tempo médio de sobrevida de todo o grupo foi 14 meses. Embora o tratamento cirúrgico näo seja curativo, a sobrevida é significativamente prolongada. Os critérios para a indicaçäo da cirurgia, radioterapia e quimioterapia säo discutidos.

Aneurisma intracraniano gigante em criança de nove anos: relato de caso / Giant intracranial aneurysm in a 9 year-old child: case report

É relatado o caso de menina de 9 anos de idade com quadro de hemorragia intracraniana devido a aneurisma gigante da porçao distal da artéria cerebral média. Aneurismas cerebrais sao considerados raros em crianças, principalmente na primeira década de vida. Em crianças, eles tendem a ocorrer principalmente em ramos arteriais periféricos e sao frequentemente gigantes. Sao discutidos os achados clínicos e a patogênese dessa lesao.

Condroblastoma benigno do osso occipital: relato de caso / Benign chondroblastoma of the occipital bone: a case report

Condroblastoma benigno é tumor ósseo raro, tendo como origem células cartilagíneas. Classicamente este tumor acomete regioes epifisárias de ossos longos, sendo incomum o envolvimento de ossos do crânio, principalmente o osso occipital. Relatamos o caso de uma paciente com 16 anos de idade, com diagnóstico de condroblastoma benigno do osso occipital e discutimos os principais aspectos deste tumor.

Angiolipoma epidural torácico: relato de caso / Thoracic epidural angiolipoma: report of a case

Angiolopomas da coluna vertebral constituem neoplasias raras (40 casos descritos na literatura). Em sua maioria estes tumores säo epidurais, apresentam características benignas, contendo elementos vasculares e elementos adiposos maduros. A sintomatologia é inespecífica, mas a tomografia computadorizada e principalmente a ressonância eletromagnética permitem fazer o diagnóstico com precisäo, como exemplifica o caso relatado