Paulo Moacir Mesquita Filho

Background: Rhabdomyosarcoma (RMS) is a malignant tumor originating from mesenchymal cells destined for skeletal muscle differentiation and is commonly recognized as a pediatric disease, accounting for <1% of malignant solid tumors in adults. As its clinical presentation depends on anatomical location, orbital tumors commonly present with proptosis and eyelid changes, while contiguous invasion of the central nervous system (CNS) is exceedingly uncommon. Regarding prognosis, the alveolar subtype generally results in poorer outcomes, especially in the adult population. Case Description: A 34-year-old man presented with a 3-month history of progressive left supraorbital protuberance, associated with proptosis, and no other neurological impairment. Imaging studies identified an infiltrative orbital lesion with soft tissue attenuation, eroding the cranial vault and extending into the frontal lobe, accompanied by vasogenic edema. Surgical resection was performed through a left frontal craniotomy. Anatomopathological and immunohistochemical studies confirmed alveolar RMS, presumably derived from extraocular muscles. Positron emission tomography-computed tomography revealed metastatic spread to mediastinal lymph nodes and bone marrow, whose biopsy demonstrated positivity for the PAX3–FOXO1 rearrangement. The patient received adjuvant radiotherapy and chemotherapy with irinotecan, vincristine, actinomycin D, ifosfamide, cyclophosphamide, and vinorelbine. Fifteen months after completing the maintenance phase, the patient shows no evidence of disease. Conclusion: Adult orbital alveolar RMS with CNS invasion is a rare and aggressive presentation that requires targeted and multimodal management. The lack of guidelines for the adult population implies the need to adapt therapeutic strategies from pediatric protocols. Comprehensive diagnosis integrating imaging, histopathology, and molecular testing is essential for accurate classification and treatment planning.

Background: Neurological manifestations of paracoccidioidomycosis are uncommon, and the clinical presentation can include a variety of symptoms that depends specially on the lesion’s site. Case Description: This article reports the case of a 63-year-old rural worker who presented with progressive imbalance and diplopia. Magnetic resonance imaging suggested a likely inflammatory/infectious etiology. The diagnosis was confirmed by the intraoperative frozen section and direct microscopic examination. Treatment started with amphotericin B and, later on, with itraconazole due to decreased renal function. The patient responded well and had improvement in symptoms. Conclusion: Being aware of the patient environment is crucial for diagnosing neuroparacoccidioidomycosis. A detailed anamnesis, with description of professional and residence location, as well as other risky activities, should be done. The treatment must be individualized depending on clinical conditions and comorbidities, but amphotericin B and itraconazole are the most indicated and standardized options.

Diffuse large B-cell lymphoma (DLBCL) is the most frequent subtype of non-Hodgkin lymphoma, accounting for 30-40% of cases. Symptoms are nonspecific and can range from nasal obstruction to visual disturbances, making diagnosis difficult and easily confused with benign inflammatory conditions and upper respiratory tract infections. In this article, we report the case of a 79-year-old female patient who sought care due to frontal headache and left hemifacial pain. After imaging studies, which revealed an infiltrative lesion in the maxillary sinus extending to the nasal cavity, orbit, and lateral wall, and biopsy, the patient was diagnosed with diffuse large B-cell lymphoma and referred for oncological treatment. The signs and clinical manifestations of diffuse large B-cell lymphoma (DLBCL) in the maxillary sinus are nonspecific. Diagnosis requires biopsy for evaluation, showing high proliferation of large B cells, with fibrosis and necrosis. Initial treatment for DLBCL involves chemotherapy using monoclonal antibodies.

Air embolism (AE) is a subtype of embolism, caused by the entry of air into the vascular system. It is a predominantly iatrogenic complication, and its symptomatic form is severe, although uncommon. In some cases, a venous thrombi may pass into the arterial system through a venous-arterial shunt, characterizing a paradoxical embolism. Here, we describe the case of a previously healthy 44-year-old female who underwent cauda equina decompression and lumbar epidural abscess drainage. The patient suffered a paradoxical AE intraoperatively and died after 4 days. The occurrence of AE in lumbar spine surgeries in the prone position is rare, but the surgical team must be attentive to its clinical signs and quickly institute initial management when necessary.

Traumatic carotid artery dissection (TCAD) usually occurs after a direct cervical trauma or blunt trauma that causes hyperextension and excessive rotation of the neck. The most frequent presentation of TCAD is stroke, with 80% of patients developing a stroke in the first week after the trauma. Recent data indicate that symptoms start later in non-survivors (19.5 hours after trauma) compared with survivors (12.5 hours). In this case report, a young boy, 14 years-old, developed TCAD after he fell from his bike. He developed ischemic stroke symptomatology in the eighth day of hospitalization, with full recovery of symtoms after acetylsalicylic acid (AAS) administration. The use of antithrombotic medication is recommended, aiming for better neurological outcomes and prevention of stroke in TCAD patients, as highlighted by our case. However, the treatment recommendations are based on observational studies and expert opinion, owing to the lack of concrete data on the treatment of carotid artery blunt trauma. New studies and data are required to improve diagnosis and treatment of TCAD.

Primary central nervous system lymphoma (PCNSL) is a rare, aggressive non-Hodgkin lymphoproliferative neoplasm. Surgery is traditionally limited to biopsy due to past studies, but recent strong evidence continues to challenge this status quo in selected patients. Here, the authors characterize a case to illustrate the potential role of surgery and foster research on integrative medical management approaches for this disease. A 73-year-old woman was admitted to the hospital with aphasia and confusion. Neuroimaging suggested a lymphoproliferative process. The patient underwent cytoreductive surgery to resect the lesion. Microscopically, large infiltrating lymphoid cells that induced brain tissue damage were observed, and a diagnosis of diffuse large B-cell lymphoma was made based on immunohistochemistry. The patient evolved clinically post surgery. A complete response to further chemotherapy maintained the patient's clinical recovery. This rare case highlights the potential of surgical intervention in the management of selected patients with PCNSL. The authors also underscore the recent, meta-analytic evidence on surgery followed by combined chemotherapy for the management of specific cases. The reported recovery in an elderly patient is noteworthy and adds to the literature on this rare subtype of brain tumors. Future research should consider investigating a potential profile of candidates for resection and combined chemotherapy in PCNSL.

Melanomas originating from the dura-mater are extremely rare tumors. The diagnosis is complex, and usually only made after excluding other entities. The prognosis is poor, with average free-disease survival of 20 months, after treatment with complete surgical excision and adjuvant therapy. We report the case of a 41-year-old asymptomatic patient, presenting with a subcutaneous mass in the left parieto-occipital region, later diagnosed as a primary dura mater melanoma. Treatment included complete microsurgical excision, radiotherapy and adjuvant immunotherapy. Therefore, due to the rare nature of the disease and its high lethality, correct diagnosis and treatment are medical challenges.

BACKGROUND AND PURPOSE: The development of flow diverters has changed the endovascular approach to intracranial aneurysms. On the basis of good results, the indications for flow diverters have expanded to include aneurysms of different shapes, locations, and sizes. The objective of the study was to report on the performance of the Flow Re-Direction Endoluminal Device (FRED) in intracranial aneurysm treatment at early and medium-term follow-up. MATERIALS AND METHODS: This single-arm, multicentric, prospective, observational study assessed aneurysm treatment with the FRED. The primary outcome was complete aneurysm occlusion at 6 and 12 months, and the secondary outcome was to evaluate the safety of the FRED with respect to stroke and death rates. RESULTS: Between June 2016 and August 2018, a total of 100 consecutive patients with 131 aneurysms were treated in 107 procedures. Total occlusion rates were 91% and 95% at 6 and 12 months. There was 1 death, and the total final morbidity rate was 1.8%. The complication rate was 4.6%. CONCLUSIONS: As reported previously, the FRED has proved to be a safe and effective tool, with high occlusion rates. The design of the stent makes it more difficult to perform balloon angioplasty compared with similar devices. A branch arising from the aneurysm sac was found to be a predictor of nonocclusion at 12 months, though larger series are needed to estimate the magnitude of the association.

Schwannomas are the fourth most common primary neoplasms affecting the brain and cranial nerves. Central lesions commonly arise from sensory nerve roots, and a common intracranial site is the vestibular branch of the 8^th nerve (>85%). We present the case report of a patient who has a schwannoma extending from the pterygopalatine fossa to the orbit, complaining about facial pain in the trajectory of the trigeminal ophthalmic branch. Schwannomas represent 1 to 2% of all neoplasms of the orbit, and trigeminal schwannomas are extremely rare, accounting for less than 0.5% of all intracranial tumors. Schwannomas são a quarta neoplasia primária mais comum que afeta o cérebro e os nervos cranianos. As lesões centrais comumente surgem de raízes nervosas sensitivas, e um sítio intracraniano comum é o ramo vestibular do oitavo nervo (mais de 85% dos casos). Apresentamos o relato de caso de um paciente portador de schwannoma que se estende da fossa pterigopalatina até a órbita, com queixa de dor facial no trajeto do ramo oftálmico do nervo trigêmeo. Schwannomas representam 1–2% de todas as neoplasias da órbita e schwannomas trigeminais são extremamente raros, respondendo por menos de 0,5% de todos os tumores intracranianos. Received: 08 May 2020 Accepted: 04 August 2020 Publication Date: 16 October 2020 (online) © 2020. Sociedade Brasileira de Neurocirurgia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/) Thieme Revinter Publicações Ltda. Rua do Matoso 170, Rio de Janeiro, RJ, CEP 20270-135, Brazil

Lhermitte-Duclos disease (LDD), or cerebellar dysplastic gangliocytoma, is a rare type of cerebellar tumor, from unknown origin. Patients can be asymptomatic for several years, but there are usually imprecise neurological signs for long periods.