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The authors describe a very rare case of an angiosarcoma originating from the petrous portion of the temporal bone that evolved as an indolent lesion until it became a giant retroauricular mass. A biopsy demonstrated that it was an angiosarcoma. A presurgical embolization from the left occipital and left parietal branches of the left middle meningeal artery was performed, followed by a total resection of the tumor. The patient developed a transient dysphasia during early follow-up, from which, subsequently, she fully recovered. There were no signs of recurrence in the current 3 years of follow-up. Free margins can be achieved even in some giant tumors and remain the most important prognostic factor for soft tissue malignant tumors with intracranial infiltration.

Purpose The objective of this study is to evaluate the meningioma`s hormone receptor expression in patients who presented synchronous or metachronous meningioma and breast cancer and compare its profile to the non-breast cancer associated meningiomas described in the literature. Methods The authors conducted a retrospective descriptive study evaluating all cases of synchronous and metachronous meningiomas and breast cancer from January 1st, 1990 to December 31st, 2020. The study was approved by the Ethics Review Board. Variables related to meningioma (age and year of diagnosis, histological grade and subtype, topography, the presence of progesterone and/or estrogen receptors) and to breast cancer (age and year of diagnosis, the histological grade and subtype, the presence of progesterone and/or estrogen receptors) were collected on the hospital`s electronic health records. Results A total of 12 cases were included in the study, being 2 (17%) synchronous and 10 (83%) metachronous. The mean age at meningioma diagnosis was 60.58 (± 10,99). The most frequent intracranial topography was convexity and sphenoid wing meningiomas (25% each). Ten patients (83%) had grade 1 meningiomas and 2 patients (16.6%) presented with grade 2 meningiomas. Eleven cases of meningiomas expressed progesterone receptors (91.67%) while 3 cases expressed estrogen receptors (25%). One patient did not express hormonal receptor. Conclusion The WHO histological analysis and steroid receptors profile did not seem to be different in the meningiomas included in this sample when compared to the description of no-breast cancer associated meningiomas.

Background Navigated transcranial magnetic stimulation (nTMS) is being used for different purposes in patients with brain tumors. However, the procedure requires a positive electrophysiological response. For patients with negative response in rest conditions, active motor threshold (AMT) may be used. However, sometimes it is difficult to obtain AMT measures owing to inability of the patient to sustain steady muscle contraction. Herein, we describe a simple method by using a hand dynamometer to obtain AMT measures during nTMS session. Case Description A woman aged 68 years underwent total removal of a right frontal lobe oligodendroglioma World Health Organization grade II 15 years ago. Cranial magnetic resonance imaging during follow-up revealed local recurrence. In the postoperative period, she developed left upper limb paresis. A postoperative nTMS session was performed for motor electrophysiological evaluation. However, using the standard technique for AMT measurement, the patient was unable to perform sustained muscle contraction as required. A hand dynamometer was used. It allowed sustained muscle contraction for AMT measurement. A counter force for the index finger flexion, the hand support to stabilize hand joints, and a numerical screen serving for both the examiner and the patient as a feedback parameter may explain the success obtained with this simple device. Conclusions Although more studies are necessary to validate the method, the hand dynamometer should be considered for patients unable to sustain muscle contraction during AMT measurement.

In the treatment of complex paraclinoidal and giant cavernous aneurysms, preservation of the patency of the internal carotid artery (ICA) is not always possible, and therapeutic occlusion of the carotid is still an important option for their management. A complete preoperative evaluation of the carotid reserve circulation, including the use of temporary balloon occlusion test and single photon emission computerized tomography (SPECT) should be included in the current paradigms of paraclinoidal and intracavernous aneurysms management. We present a series of fifteen patients with sixteen giant or complex carotid cavernous or ophthalmic aneurysms that were treated following a protocol for our preoperative decision-making analysis. Extracranial to intracranial saphenous vein bypass was reserved to the cases where carotid occlusion would be associated with high risk of ischemic complications and was performed in three patients. Besides the difficulties in dealing with those complex aneurysms, good clinical outcome was possible in our experience with the designed paradigm. No tratamento de aneurismas paraclinoideos complexos e cavernosos gigantes, a preservação da patência vascular nem sempre é possível, e a oclusão terapêutica da carótida ainda é uma opção importante no seu manejo. Uma avaliação pré-operatória completa da reserva circulatória carotídea, incluindo o uso do teste de oclusão temporária por balão associado à tomografia computadorizada por emissão de fóton único (SPECT) podem ser de grande utilidade para definir a opção terapêutica a ser adotada. Nós apresentamos uma série de quinze pacientes com dezesseis aneurismas complexos ou gigantes do segmento oftálmico e cavernoso da artéria carótida, que foram tratados de acordo com determinado protocolo de investigação pré-operatória. Anastomose com enxerto de veia safena entre a carótida extra e intracraniana foi reservada para os casos em que a oclusão carotídea estaria associada a um alto risco de complicações isquêmicas e foi realizado em três pacientes. Apesar das dificuldades em lidar com aneurismas complexos como os aqui relatados, é possível obter um bom resultado clínico nestes pacientes com o paradigma desenhado.

Background Optochiasmatic tuberculomas are very rare lesions. They can occur with concomitant tuberculous meningitis, and pulmonary tuberculosis or as the only manifestation of the disease. The authors present a case of optic pathways tuberculoma with radiologic appearance simulating an optic pathways glioma. Case description We report a case of a 20-year-old man with mental retardation due to anoxic encephalopathy who developed a sudden bilateral amaurosis. He also presented with diabetes insipidus, panhypopituitarism, right proptosis, and chemosis. Computed tomography (CT) and magnetic resonance imaging (MRI) showed an enhancing lesion in the optochiasmatic region extending to both optic nerves, with a mass in the right orbit, mimicking an optic pathways glioma. There was no other evidence of systemic involvement of the tuberculosis. The lesion was explored through a right pterional transylvian approach with opening of the optic canal and orbital roof, and a biopsy and an internal decompression were performed. Histopathological studies demonstrated a granulomatous lesion with central caseous necrosis with acid-fast bacilli. The patient improved after treatment with tuberculostatic drugs, but vision recovery could not be achieved. Conclusion Visual compromise in tuberculosis is associated with hydrocephalus, optical neuritis or tuberculomas involving the optic pathways. Reviewing the literature on tuberculomas of the optochiasmatic area, we could not find any other case with such extensive involvement of the optic pathways that was radiologically suggestive of an infiltrating glioma. Histopathological studies remain crucial in the diagnosis of intrinsic expansive processes of the optochiasmatic region.

Diversos fatores têm sido associados ao desenvolvimento de edema peritumoral nos meningiomas. Foram estudados os aspectos radiológicos e anátomo-patológicos de 51 meningiomas intracranianos operados no Hospital Universitário Clementino Fraga Filho (HUCFF). Dois terços dos meningiomas apresentavam edema perilesional. O tamanho dos meningiomas correlacionou-se com a presença de edema, sendo mais frequente nos meningiomas grandes (>4cm). A localização parece, também, influenciar no desenvolvimento do edema peritumoral, sendo mais acentuado nos meningiomas da asa do esfenóide e incomum nos meningiomas do tubérculo selar. Os subtipos histológicos de meningioma não se correlacionaram com a intensidade do edema peritumoral. Dos diversos mediadores químicos descritos na literatura recente relacionados ao desenvolvimento de edema peritumoral em tumores intracranianos, destaca-se o fator de crescimento do endotélio vascular (VEGF). A expressão nos meningiomas do VEGF e de seu receptor flk-1 foi estudada com técnica imuno-histoquímica, demonstrando a sua expressão nas células tumorais.

The article describes a woman with 53 year-old that has presented diplopia when looking down and adopted a tilted head position in order to read for the last six months. The neuro-ophthalmic examination shows isolated right superior oblique muscle paresis. The magnetic resonance and the angioresonance show the dolicoectatic basilar artery compressing the right ventral lateral brainstem.

The purpose of the present clinical case is to remind clinicians that craniopharyngiomas, which are benign neoplasms with a high incidence of local recurrences, may also present ectopic recurrences which may at first go unsuspected. These tumors most commonly arise in the suprasellar region and despite their benign histology, they may infiltrate the surrounding neurovascular structures making total removal challenging. Ectopic recurrences of craniopharyngiomas are very rare. We describe an adult patient with ectopic recurrence of craniopharyngioma, emphasizing unique features of the case presentation and its physiopathological aspects. A 49-year-old male presented with headache and visual field defect and was diagnosed with a suprasellar tumor. He was submitted to neurosurgery and histological examination revealed an adamantinomatous craniopharyngioma. Postoperative magnetic resonance imaging (MRI) showed complete tumor resection. The patient remained asymptomatic with no imaging signs of local recurrence during follow up. Five years after surgery, the patient noticed a rapidly growing lump at the surgical incision site. He reported a mild to moderate local trauma 4 months before. A MRI showed a subgaleal cystic tumor arising in the pathway of the craniotomy. Surgical resection of that cystic lesion was performed and histological examination revealed an adamantinomatous craniopharyngioma. One year later no recurrences have been detected. The case reported has two particular features: the local trauma as a potential trigger for tumor progression and the rapidly growing pattern of the ectopic recurrent tumor. We emphasize that although ectopic recurrences of craniopharyngiomas are rare, they may occur away from the primary tumor and quite late in the follow up of the patient.

Aneurysms arising at the proximal intracranial carotid artery (between cavernous sinus and posterior communicating artery) and at the anterior communicating complex may present with technical difficulties regarding appropriate access without excessive brain retraction. In a series of 15 patients the authors show the use of the cranio-orbital approach, formerly designed in the access of skull base tumors, for these subgroups of aneurysms. They discuss the advantages of a basal avenue in minimizing brain retraction, better anatomical orientation, obviation of tissue ressection and easier extra-dural bone removal. It was not identified any complication due to the described approach. Aneurismas situados junto à artéria carótida intracraniana proximal (entre seio cavernoso e artéria comunicante posterior) e ao nível do complexo comunicante anterior podem apresentar dificuldades técnicas relacionadas ao acesso apropriado sem retração cerebral excessiva. Em uma série de 15 pacientes os autores mostram o uso do acesso crânio-orbital, utilizado inicialmente para abordagem a tumores da base do crânio, para esses subgrupos de aneurismas. Discutem suas vantagens em minimizar retração, melhor orientação anatômica, evitar ressecção de tecido neural e facilitar remoção óssea. Não foi identificada qualquer complicação devida ao acesso descrito.

Treatment of intracranial tumoral lesions is related to its correct histological diagnostic. We present a retrospective analysis of 32 patients submitted to 36 cerebral biopsies using neuronavigation and 44 patients using frame-based stereotaxy. Mean age was 46.6 and 49.3 years old respectively. Sex distribution in both groups was 50% for each. Most of lesions were lobar in both groups. Diagnostic yielding was 91.7% and 83.4%, respectively (p=0.26). We found in the postoperative CT scans intracranial hemorrhages in 13.8% cases of the first group and 9.8% cases in the second. Most of them were mild post-operative hemorrages in the biopsy site. There was one death related to the procedure in each group. Astrocytomas and metastatic adenocarcinomas were the most frequent diagnosis. Diagnostic yielding and the number of postoperative hemorrhage and death were similar on both groups and the same found in the literature.

Introduction: Epidermoid tumors are benign slow-growing tumors that can be found in several locations in the brain or spinal cord, mainly within the cerebellopontine and suprasellar cisterns, rarely giving neurological complications. The occurrence of multiple intracranial epidermoid cysts or ruptured epidermoid cysts with intrathecal seeding is very rare. Furthermore, multiple fat-intensity lesions can be a challenge in the neuroradiological diagnosis. Methods: We reviewed the cases of nonmeningiomatous tumors of the cavernous sinus and the epidermoid tumors operated in our hospital in the last 8 years and their neurological symptoms. Results: We describe a case of a 51-year-old female with longstanding recurrent headaches and mild numbness over her left cheek. Her imaging studies showed an epidermoid tumor in the left cavernous sinus and multiple fat-intensity lesions in the subarachnoid space. Radical removal of the epidermoid tumor was obtained through a cranio-orbitozygomatic approach with an extradural exposure of the cavernous sinus without any postop additional morbidity. Conclusions: We have documented the CT and MR imaging appearance and the operative findings of a very rare case of epidermoid tumor of the cavernous sinus with intrathecal dissemination of epidermoid cyst.

Introduction: Vestibular schwannomas are slow-growing tumors with progressive hearing and vestibular symptoms as the usual presentation. Cystic degeneration in schwannomas are found not so rarely, and it is probably secondary to microhemorrhages inside the tumor. We report a very unusual case of vestibular schwannoma with a massive hemorrhage inside the tumor and abrupt clinical presentation. Methods: We reviewed the cases of vestibular schwannomas treated by the authors with particular attention to their presentation symptoms and the occurrence of associated cysts. Results: We report a case of a 52-year-old female with acute presentation symptoms of intracranial hypertension and compression of the brain stem due to a large cystic vestibular schwannoma with massive bleeding inside the cyst. Total removal of the tumor was obtained through a suboccipital retrosigmoidal approach after the drainage of the hemorrhagic collection inside the tumor. Pathological studies confirmed hemosiderin deposition in the walls of the cyst. Conclusions: We report the CT and MR imaging appearance and the operative findings of a very rare case of massive intratumoral bleeding in a vestibular schwanoma. This unusual and unpredictable occurrence would be considered as an additional risk in the cases treated conservatively or with gamma-knife.

Background: Chordomas are rare slowly growing tumors that originate from remnants of the notochord. They have a malignant local behavior, causing symptoms due to bone infiltration and compression of neurovascular structures. Only a few cases of brain tumors associated with pathological laughter have been reported in the literature. Case Description: We report a case of a 42-year-old male patient with this atypical clinical presentation treated at our institution, and discuss the concerning literature. Conclusion: Although being a very rare presentation of chordomas, pathological laughter is usually expected to improve after brain stem decompression.