Spontaneous Regression of a Clival Chordoma: An Unusual Case Report.

INTRODUCTION: Chordoma is a malignant and aggressive tumor originating from remnants of the primitive notochord and usually involving the axial skeleton. Spontaneous regression of clival chordomas was described recently. We present the third case report of spontaneous regression of a clival chordoma and discuss similarities of cases and implications for clinical practice. CASE DESCRIPTION: We present the case of a previously healthy 21-year-old Caucasian woman who presented with progressive holocranial headache for 3 months, which encouraged image investigation. Magnetic resonance imaging (MRI) revealed an osteolytic clival lesion hyperintense in T2 and hypointense in T1 images. After 2 months of initial evaluation and surgical proposal, she repeated MRI to allow use for intraoperative neuronavigation. Surprisingly, there was tumor regression. DISCUSSION: The present reported case is somehow different from previous ones and does not share an underlying inflammatory/immunological recognizable fact, being interpreted by us as a spontaneous partial regression of the tumor. We highlight the need for continuous investigation of chordoma regression to uncover the underlying mechanisms.

Endoscope-Assisted Middle Fossa Approach: Optimizing the Surgical Corridor for the Resection of Multicompartmental Chordomas.

Background Skull base chordomas are a major therapeutic challenge. The surgical management involves selecting an approach that will offer the patient the best chance of largest/complete removal while minimizing morbidity and mortality. Methods Medical records and imaging review of two skull base chordomas involving the middle fossa and posterior fossa that were successfully treated with an endoscope-assisted middle fossa approach. Results The use of angled endoscopes provided better identification of anatomical landmarks and improved tumor resection when compared with the microscopic surgical exposure. The approach selection, anatomical landmarks, and technical aspects of the intraoperative setting of the endoscope-assisted approach are discussed. Conclusion Endoscopic assistance in the middle fossa approach is a safe and valuable tool for maximizing the reach of the surgical corridor when treating skull base chordomas.

Endoscopic resection of sellar and suprasellar epidermoid cyst: report of two cases and review of literature.

INTRODUCTION: Epidermoid cysts (EC) are lesions developing from neuroectodermal epithelial cells. They represent 1-2% of all intracranial tumors and are usually found in cerebellopontine angle and parasellar regions. To the best of our knowledge, only 27 cases have been reported of EC in sellar and suprasellar region. In 12 cases out of the 27, surgery was done by craniotomy means. The 7 most recent manuscripts (with 15 patients described) share in common the use of endoscopic endonasal approach (EEA) to perform surgical removal. RESULTS: In this paper, we report the safe removal of epidermoid cysts arising from the pituitary using an EEA in two patients, which should be the sixth such description in literature. In both cases, resection and evolution was favourable. DISCUSSION: Surgical resection is the treatment standard for epidermoid cysts, with total resection including the cyst wall to prevent recurrence when possible. The degree of resection obtained is limited by adherence to nearby neural and vascular structures. The advent of EEA approaches has allowed safe maximal resection especially in midline lesions nearby sellar and suprasellar compartiments.

Social determinants of health and survival on Brazilian patients with glioblastoma: a retrospective analysis of a large populational database.

Background: The majority of patients diagnosed with glioblastoma develop recurrent disease resulting in poor prognoses. The current study aimed to determine the survival rates of patients diagnosed with glioblastoma in Brazil accounting for the influence of age, treatment modalities, public and private practices, and educational level using a population-based national database. Methods: Patients diagnosed with glioblastoma from 1999-2020 were identified from The Fundação Oncocentro de São Paulo database to create a retrospective cohort. Patients were described according to age, education level treatment modalities and medical practice. In a Cox proportional hazards model, controlled for confounding factors for overall survival, the hazard ratio and 95% CI of overall survival in adults was evaluated. Findings: A total of 4,511 patients were included. The median lengths of survival for patients treated in the public and private settings were 8 and 17 months (p<0.001), respectively. Young patients had longer median overall survival (OS: 18 to 40 years, 41 to 60 years, 61 to 65 years, 66 to 70 years and over than 70 years was 22 months, 10 months, 6 months, 5 months, 4 months, respectively (p<0.001). In general, combined treatments were associated with higher median survival compared to monotherapy. The higher educational level, the higher median survival was observed (4 months for illiterate versus 14 months for university degree). In the multivariable analyses, the significant independent predictors for overall survival were practice setting, educational level, age and treatment modalities. Interpretation: Public practice, older patients, less intensive treatment, and lower educational level were associated with worse survival outcomes in Brazilian glioblastoma patients.

Improvement of Hemichorea Following Surgical Resection of a Putaminal Cavernous Angioma: Case Report and Review of Literature.

BACKGROUND: Hemichorea may point to a structural lesion in the contralateral basal ganglia with a large list of possible causes. Cavernous angioma may be rarely a possible cause for acute appearance of this movement disorder. CASE DESCRIPTION: We present a rare case of a 32-year-old female patient with hemichorea caused by a cavernoma (or cavernous angioma) in the contralateral insula and putamen with complete improvement of symptoms with surgical resection of the lesion. CONCLUSIONS: We believe that surgical resection of basal ganglia cavernomas may be feasible with minor risks and resolution of clinical symptoms in the immediate postoperative period.

Radiologic Features of Spontaneous Regression of Vestibular Schwannomas.

BACKGROUND: Vestibular schwannoma (VS) is the most common benign tumor originating in the cerebellopontine angle. In most cases, tumors tend to grow and deserve proper treatment. Sometimes they stabilize, and rarely they decrease in size spontaneously. METHODS: We evaluated retrospectively the images of patients with spontaneous tumor regression. We describe the common neuroimage findings of patients with spontaneous tumoral regression. RESULTS: Four patients with diagnosis of VS were followed with magnetic resonance imaging (MRI). There were some relevant features in MRI: a heterogeneous contrast enhancement in the outer layer of the tumor and presence of a cerebrospinal fluid column between the tumor and the entrance of the internal auditory canal. The percentage of tumor diameter reduction ranged from 20% to 40%. CONCLUSIONS: Some MRI features may demonstrate a spontaneous involution of VS and may be closely followed in asymptomatic or oligosymptomatic patients.

Diffuse spinal spreading following previous intracranial intradural chordoma resection: A rare case report.

INTRODUCTION: Chordoma is a malignant tumor that usually involves the axial skeleton. Intradural chordomas are even rarer and 37 cases have been reported to the best of our knowledge. We present a case of a patient with an atypical metastatic diffuse intradural spinal involvement. CASE DESCRIPTION: We present a 33-year-old woman previously submitted to five brain surgeries to treat a posterior fossa intradural chordoma since December 2012. Currently, she presented almost with right and left hemiplegia (grade 2 bilaterally) and also left oculomotor, adbucent and facial nerve paresis. We performed neuroaxis magnetic resonance MR which disclosed stability of posterior fossa tumor which was previously irradiated. However, there were new intradural lesions at the level of C3, T11/T12 and L4/L5/S1 vertebrae. DISCUSSION: With the advent of contemponaeous surgery, radiotherapy options and even available chemotherapy to treat Chordomas (Imatinib), patients may experience enlarged survival and thus face complications such as drop metastases along neuroaxis. Our case illustrates a late (6 years) follow-up presentation of an initial posterior fossa intradural chordoma. It suggests that whole neuraxis involvement may be the final presentation of all patients harbouring chordomas and surviving after adequate initial treatment.

Role of Transnasal Endoscopic Surgery in the Treatment of Superficial Siderosis of Central Nervous System Secondary to Clivus Arachnoidocele: Report of Successful Case and Literature Review.

BACKGROUND: Superficial siderosis (SS) of the central nervous system is a disease characterized by deposition of hemosiderin in the leptomeninges (arachnoid and pia mater) due to chronic intradural bleeding. One of the etiologic mechanisms proposed is a dural breach secondary to trauma with a consequent arachnoidocele in contact with an exuberant venous plexus. We describe a unique case of clival arachnoidocele treated by an endoscopic endonasal approach and closure of the defect with fat and nasoseptal flap. CASE DESCRIPTION: A 35-year-old man with a history of severe head trauma 20 years ago presented with hearing deficit and a mild motor ataxia impairing gait. Magnetic resonance imaging disclosed hemosiderin deposition throughout the brain cortical layer and in the cerebellum, affecting the dentate nucleus as well. A computed tomography revealed an osteolytic formation in the clivus, involving the inner bone table and bone marrow. The patient was then submitted to an endoscopic endonasal transclival approach to close the defect. CONCLUSIONS: SS may be a result of several etiologies generating repetitive meningeal bleeding. Our patient had the diagnosis of posttraumatic clival arachnoidocele and SS probably related to trauma with some dural injury. An endoscopic endonasal approach with tear reconstruction is feasible and successful to address clival arachnoidoceles and, in this case, to avoid progression of the SS.

Epithelioid pituicytoma: An unusual case report.

BACKGROUND: Pituicytomas are considered World Health Organization Grade I malignancies. Until September 2017, a total of 81 cases of pituicytomas were diagnosed and described in literature. We present such a case in which histopathology shows an epithelioid pattern, a rare variant of pituicytoma. As far as we know, this is only the second such case described in the literature. CASE DESCRIPTION: A 61-year-old male patient presented with complaints of progressive decrease in visual acuity for about 7 months, worse on the left side. Laboratory and endocrinological investigation returned normal values. Magnetic resonance imaging revealed a mixed solid-cystic lesion, measuring about 3.1 × 2.2 × 2.9 cm. The lesion presented with intermediate signal intensity in T1 and T2 sequences and showed avid postcontrast enhancement. The patient underwent resection through a left pterional approach. Pathology revealed a glial neoplasm with an epithelioid pattern and moderate cellularity with rounded-elongated cell nuclei and with a broad eosinophilic cytoplasm. Absence of cellular pleomorphism, any mitotic figures, or areas of necrosis was noted. CONCLUSION: The epithelioid variant of pituicytomas differs from the commonly encountered forms of this tumor which typically present in a fascicular pattern. Microsurgical resection is the treatment of choice. However, in many cases, subtotal resection was performed because of a considerable risk for neurovascular injuries.

Endoscopic endonasal management of cerebrospinal fluid rhinorrhea after anterior clinoidectomy for aneurysm surgery: changing the paradigm of complication management.

Resection of the anterior clinoid process results in the creation of the clinoid space, an important surgical step in the exposure and clipping of clinoidal and supraclinoidal internal carotid artery aneurysms. Cerebrospinal fluid rhinorrhea is an undesired and potentially serious complication. Conservative measures may be unsuccesful, and there is no consensus on the most appropriate surgical treatment. Two patients with persistent transclinoidal CSF rhinorrhea after aneurysm surgery were successfully treated with a combined endoscopic transnasal/transeptal binostril approach using a fat graft and ipsilateral mucosal nasal septal flap. Anatomical considerations and details of the surgical technique employed are discussed, and a management plan is proposed.

Endoscopic endonasal management of cerebrospinal fluid rhinorrhea after anterior clinoidectomy for aneurysm surgery: changing the paradigm of complication management / Endoscopia endonasal no tratamento da fístula liquórica após clinoidectomia anterior na cirurgia de aneurisma: mudando o paradigma no manejo desta complicação

ABSTRACT Resection of the anterior clinoid process results in the creation of the clinoid space, an important surgical step in the exposure and clipping of clinoidal and supraclinoidal internal carotid artery aneurysms. Cerebrospinal fluid rhinorrhea is an undesired and potentially serious complication. Conservative measures may be unsuccesful, and there is no consensus on the most appropriate surgical treatment. Two patients with persistent transclinoidal CSF rhinorrhea after aneurysm surgery were successfully treated with a combined endoscopic transnasal/transeptal binostril approach using a fat graft and ipsilateral mucosal nasal septal flap. Anatomical considerations and details of the surgical technique employed are discussed, and a management plan is proposed.

RESUMO A ressecção da clinóide anterior resulta na criação do espaço clinoideo, um passo cirúrgico importante na exposição e clipagem de aneurismas dos segmentos clinoideo e supraclinoideo da artéria carótida interna. Fístula liquórica é uma das complicaçoes mais indesejadas e é potencialmente grave. O manejo com medidas conservadoras pode ser bem sucedido, e não há consenso sobre o tratamento cirúrgico mais adequado. Dois pacientes com rinorréia persistente secundária a fistula liquórica transclinoidal após cirurgia de aneurisma foram tratados com sucesso por uma abordagem endoscópica combinada transnasal/transseptal binostril usando um enxerto de gordura e retalho de mucosa naso-septal ipsilateral. Considerações anatômicas e detalhes da técnica cirúrgica empregada são discutidos, e um plano de manejo destes tipo de fistula líquorica é proposto.

Functional Reconstruction of Temporomandibular Joint after Resection of Pigmented Villonodular Synovitis with Extension to Infratemporal Fossa and Skull Base: A Case Report.

Introduction Pigmented villonodular synovitis (PVNS) is a benign but aggressive lesion arising from sinovia. The temporomandibular joint (TMJ) is hardly ever involved. Methods We describe a case of PVNS arising in the left TMJ involving infratemporal fossa soft tissue and the skull base; we also present the reconstruction. Results A 37-year-old woman had progressive mandibular swelling for 6 months. Computed tomography of the skull revealed an osteolytic lesion in the left TMJ, involving the upper mandible, condyle, and glenoid fossa and extending to the infratemporal fossa and fossa media through a defect in temporal bone. Surgical management included a left pterional craniotomy to reach the temporal skull base and resect the intracranial tumor and a facial approach with partial left mandibulectomy and resection of left condyle, glenoid fossa, and tumor removal in infratemporal fossa. Mandible function was restored with prosthetic reconstruction of the condyle. She progressively started to eat solid foods after 3 months, becoming increasingly functional and asymptomatic. At 30 months' follow-up, she had no sign of tumoral recurrence and showed asymptomatic and normal TMJ function. Conclusion PVNS should be considered in the differential diagnosis of bone neoplasms affecting young patients. In such cases, radical excision is mandatory and TMJ prosthesis for local reconstruction may be used to preserve functionality.

Vasa Vasorum and the Growing of Thrombosed Giant Aneurysm of the Vertebral Artery: A Case Report.

BACKGROUND: Giant aneurysms arising from the vertebral artery (VA) are often associated with thrombosis and present as mass lesions with slow progression of symptoms and signs. A consensus is still to be established on the underlying growth mechanisms and surgical management of thrombosed giant aneurysms of the VA. CASE DESCRIPTION: Here, we report the case of a 54-year-old man who sought neurosurgical care after 3 months of progressive cervical axial pain. He underwent cervical magnetic resonance imaging and cerebral angiography, which revealed a giant VA aneurysm with thrombosed component. After failure of proximal and distal endovascular treatment, it was decided to adopt a surgical approach, which revealed a markedly developed vasa vasorum in the aneurysmal walls, raising the possibility of intra-aneurysmal nutrition from vasa vasorum. The patient recovered progressively and almost completely after surgery. CONCLUSIONS: Another report described the case of a 58-year-old woman harboring a partially thrombosed giant aneurysm of the VA. At the time of resection, a marked development of vasa vasorum on the occluded VA and the neck of the aneurysm was noted. We highlight the need to comprehend vasa vasorum as potential sources for aneurysmal growth.

Intradural chordoma presenting with intratumoral bleeding.

Intradural clival chordomas are very rare, and only 29 cases have been reported to our knowledge. They arise purely intradurally without bone or dural involvement and may differ from classic clival chordomas in physiopathology and management. We present a 28-year-old woman who presented with intradural clival chordoma and tumoral bleeding. After initial gross macroscopic surgical resection, she presented with tumor recurrence after 2 years, again with intratumoral bleeding. Although usually considered to have a more favorable prognosis in comparison to typical chordomas, intradural chordomas appear to behave as typical chordomas. Intratumoral bleeding may be a sign of an aggressive lesion and risk of recurrence. We highlight the differential diagnosis of intrinsic posterior fossa bleeding, especially in young patients. Intradural chordomas may be underdiagnosed and incorrectly treated as other types of parenchymal hemorrhage.

The endoscopic endonasal approach for extradural and intradural clivus lesions.

OBJECTIVE: To report the use of the endoscopic transnasal transclival approach to treat tumors involving the clivus region. METHODS: The clinical records of 38 patients with clivus lesions were retrospectively reviewed to determine the surgical technique used. All patients were surgically treated using any of the options of the endoscopic transnasal transclival approach at the São Paulo Skull Base Center from 2000-2011. A transsphenoidal, transpterygoidal, retropharyngeal, or a combination of approaches was chosen based on the tumor topography. RESULTS: Chordomas were the most frequent tumor (26 of 38), followed by chondrosarcoma (2 of 38). Biopsy only was performed in 6 patients with metastasis to the clivus, and 1 patient with fibrous dysplasia underwent a planned partial resection. Gross total resection (GTR) was achieved in 15 of 31 (48%) patients with indications for GTR. For centrally located tumors, GTR was achieved in 75% (15 of 20 patients). Fistula was the most frequent complication (6 of 31; 19%) but was much lower in the most recent series using the nasoseptal flap (1 of 16; 6%). Tumors with lateral extensions or with previous treatment had the worst results. The presence of intradural extension was not a limiting factor for GTR. CONCLUSION: Endoscopic transnasal surgery is an alternative approach to treatment of clivus lesions, and, in expert hands, this technique can obtain good results. Lateral extension and previous treatment were factors that could make the surgery more difficult. Intradural extension did not limit the radicality of the removal.

Laceration of the posterior inferior cerebellar artery by suboccipital puncture of the cisterna magna: case report.

We report the case of a 27 year old man who presented to the emergency room of a hospital with headache, vomiting and an episode of loss of conciousness. A cranial CT scan was normal and the patient discharged. Ten hours later he came to the emergency room of our hospital with the same complaints. A technically difficult cisternal puncture in an anxious patient who moved during the needle introduction was done. The CSF sample showed 1600 intact red blood cells without other alterations. His headache worsened and after 6 hours he became drowsy, numb and exhibited decerebration signs. A new CT scan showed diffuse subarachnoid and intraventricular blood. An emergency angiogram demonstrated laceration of a left posterior-inferior cerebellar artery in its retrobulbar loop with a pseudoaneurysm. He was successfully treated by surgical clipping without injury. Sixteen days later he was discharged with a normal neurological exam.

Laceration of the posterior inferior cerebellar artery by suboccipital puncture of the cisterna magna: case report

Relatamos o caso de um homem de 27 anos que procurou o pronto atendimento de um hospital com cefaléia intensa, vômitos e um episódio de perda de consciência. Uma tomografia de crânio foi normal e o paciente foi dispensado. Dez horas após, o paciente procurou o setor de emergência do nosso hospital com as mesmas queixas. Uma punção suboccipital tecnicamente dificultada pela ansiedade do paciente que se movimentou durante a coleta foi realizada evidenciando amostra de LCR levemente hemorrágico. A análise do LCR mostrou presença de 1600 hemácias íntegras sem aumento de leucócitos nem alterações bioquímicas. Houve piora acentuada da cefaléia e após 6 horas apresentou sonolência, torpor e sinais de descerebração. Nova tomografia mostrou sangue no espaço subaracnóideo e nos ventrículos. Uma angiografia realizada de emergência demonstrou laceração da artéria cerebelar póstero-inferior esquerda com a formação de um pseudoaneurisma. O paciente foi de imediato operado tendo sido realizada a clipagem do pseudoaneurisma com sucesso. Dezesseis dias após o paciente teve alta com exame neurológico normal.