Background:Primitive neuroectodermal tumor (PNET) is a malignant neoplasm that generally arises from bone and soft tissues, with predilection for young adults. This neural crest origin tumors share biologic and histologic features with Ewing's sarcoma (ES).Case Description:We present a case of a 22-year-old woman with history of severe progressive neck pain, without radiation, associated with paresthesia in the right arm, and palpable right posterior cervical mass. Neurological examination showed increased reflexes in all four limbs, bilateral Hoffman's sign, right Babinski's sign, and right hemi-hypoesthesia. Neuroimaging revealed a right posterior cervical lesion with heterogeneous contrast enhancement extending to the neural foramina of the atlas and axis. Patient underwent microsurgical removal of the lesion, and histopathological and immunohistochemical analysis confirmed the diagnosis of peripheral primitive PNET (pPNET). The patient had adjuvant treatment with radiotherapy and chemotherapy. After twelve months, neuroimaging showed no signs of tumor regrowth and the patient had no neurological deficits. However, three months later, the patient developed hydrocephalus and cerebrospinal fluid (CSF) was positive for neoplastic cells. No other treatment was administered and the patient died.Conclusion:pPNET is a rare malignant tumor with poor prognosis, although promising results with multimodal treatment-surgery, radiotherapy, and chemotherapy. Diagnosis requires immunohistochemical analysis, with identification of neuronal differentiation markers.

We report our experience on the treatment of tuberculum sellae meningiomas (TSMs) regarding the involvement of the optic canal and clinical outcomes. We reviewed 23 patients who were operated on between January 1997 and December 2008. The surgical approach was unilateral subfrontal supraorbital osteotomy in one piece. Attempts were made to improve visual function via extra/intradural unroofing of the optic canal, which released the optic nerve. Visual symptoms were present preoperatively in 21 patients, and two patients were asymptomatic. Visual acuity remained intact in 6 patients, improved in 10, was unchanged in 5 patients, and worsened in 2 patients. The postoperative visual field was normal or improved in 17 patients, unchanged in four patients, and worsened in two patients. The optic canal and clinoid were drilled extradurally in eight patients and intradurally in nine patients. Total resection of TSMs was achieved in 19 patients. Incomplete resection occurred in two patients. Decompression of the optic canal seemed to increase the visual outcome. Key words: tuberculum sellae meningioma, optic canal, visual symptoms. Meningiomas do tuberculo selar: aspectos cirúrgicos resuMoApresentamos nossa experiência em 23 pacientes operados com meningiomas do tubérculo da sela, com enfoque na descompressão do nervo óptico e nos sintomas visuais do pós-operatório. Vinte e três pacientes com meningiomas do tubérculo da sela foram operados entre janeiro de 1997 e dezembro de 2008, através do acesso subfrontal via ostetomia supraorbital. Remoção do teto do canal óptico por via extra ou intradural foi realizada em 17 pacientes. Sintomas visuais no pré-operatório ocorreram em 21 pacientes, 2 eram assintomáticos. Melhora visual ocorreu em 10, permaneceu inalterada em 5 e piorou em 2 pacientes. No pós-operatório o campo visual normalizou-se em 17 pacientes, permaneceu inalterado em 4 e diminuiu em 2. Descompressão dos nervos ópticos foi realizada em 17 pacientes. Ressecção total dos meningiomas do tubérculo da sela foi possível em 19 pacientes. Abertura do canal óptico permitiu a manipulação do nervo óptico sem novos déficites. Palavras-chave: meningioma, tubérculo selar, nervo óptico, sintomas visuais.

Background: Intracranial subependymomas are rare slow-growing benign tumors typically located in the ventricular system, accounting for 0.07–0.7% of all intracranial neoplasms. Intraparenchymal subependymoma is extremely rare lesions, imposing a challenging diagnosis and management. Case Description: We describe a case of a supratentorial intraparenchymal mass on left occipital lobe in a 26-year-old woman with progressive headache and visual impairment. Differential diagnosis mainly included gliomas, neuronal-glial tumors, ependymoma, and subependymoma. Complete surgical resection was performed and histopathology analysis confirmed diagnosis of subependymoma. Despite its benign behavior the Ki67/MIB-1 labeling index assessed by immunohistochemistry was 5%. After 1 year of follow-up she was free of tumor recurrence. Conclusion: Intraparenchymal subependymoma is extremely rare tumors and literature review showed only 11 cases reported. In general, they are misdiagnosed as other tumors, so careful attention on clinical and radiological features must be taken when looking at a tumor close to the ventricular system, even though it does not have any obvious direct connection to it. Despite its benign nature, total removal must be attempted given that there are reports of recurrence, especially in partially removed tumors with high proliferation index. The role of adjuvant therapy is still limited and new treatment options are being developed as our knowledge on biological and molecular characteristics advances.

Background:Pneumocephalus is defined as the presence of air within the intracranial vault. A common complication of head trauma and surgery, pneumocephalus is usually related to ventricular shunts, craniotomies, and surgery in the sitting position. Tension (symptomatic) pneumoventricle is a rare entity associated with significant clinical morbidity.Case Description:We report an unusual case of a 15-year-old girl with tension pneumoventricle developed shortly after removal of a choroid plexus papilloma of the fourth ventricle by a midline suboccipital approach while in the sitting position.Conclusion:The presence of a cerebrospinal fluid (CSF) diversion system that causes a decrease in intracranial pressure and the existence of a craniodural defect with or without an obvious CSF leak may be the cause of tension pneumoventricule. According to our present understanding, this is the first report of this peculiar complication of fourth ventricular surgery. We discuss clinical manifestations, surgical management, contributing factors, and mechanisms involved in the pathogenesis of tension pneumoventricle.

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