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.

ICAR: endoscopic skull-base surgery.

BACKGROUND: Endoscopic skull-base surgery (ESBS) is employed in the management of diverse skull-base pathologies. Paralleling the increased utilization of ESBS, the literature in this field has expanded rapidly. However, the rarity of these diseases, the inherent challenges of surgical studies, and the continued learning curve in ESBS have resulted in significant variability in the quality of the literature. To consolidate and critically appraise the available literature, experts in skull-base surgery have produced the International Consensus Statement on Endoscopic Skull-Base Surgery (ICAR:ESBS). METHODS: Using previously described methodology, topics spanning the breadth of ESBS were identified and assigned a literature review, evidence-based review or evidence-based review with recommendations format. Subsequently, each topic was written and then reviewed by skull-base surgeons in both neurosurgery and otolaryngology. Following this iterative review process, the ICAR:ESBS document was synthesized and reviewed by all authors for consensus. RESULTS: The ICAR:ESBS document addresses the role of ESBS in primary cerebrospinal fluid (CSF) rhinorrhea, intradural tumors, benign skull-base and orbital pathology, sinonasal malignancies, and clival lesions. Additionally, specific challenges in ESBS including endoscopic reconstruction and complication management were evaluated. CONCLUSION: A critical review of the literature in ESBS demonstrates at least the equivalency of ESBS with alternative approaches in pathologies such as CSF rhinorrhea and pituitary adenoma as well as improved reconstructive techniques in reducing CSF leaks. Evidence-based recommendations are limited in other pathologies and these significant knowledge gaps call upon the skull-base community to embrace these opportunities and collaboratively address these shortcomings.

Approach Selection and Surgical Planning in Posterior Cranial Fossa Meningiomas: How I Do It.

Posterior cranial fossa meningiomas represent approximately 9% of all the intracranial meningiomas. Despite the recent reports of radiation therapy in the management of these tumors, surgical resection continues to be the first line of treatment method aiming the permanent meningioma eradication. The evolution of imaging studies improved the preoperative evaluation of meningiomas providing greater anatomical detail of small structures not previously visualized. Nonetheless, the preoperative radiological evaluation should go beyond the differential diagnosis of a posterior fossa tumor. Anatomo-radiological assessment of meningiomas is discussed in detail. Based on our clinical experience, literature review, and case illustration, we highlight important preoperative anatomo-radiological aspects of posterior fossa meningiomas and their implications in the surgical management of these tumors.

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.

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.

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 Transplanum Transtuberculum Approach for the Resection of a Large Suprasellar Craniopharyngioma.

Objectives To demonstrate an endoscopic endonasal transplanum transtuberculum approach for the resection of a large suprasellar craniopharyngioma. Design Single-case-based operative video. Setting Tertiary center with dedicated skull base team. Participants A 72-year-old male patient diagnosed with a suprasellar craniopharyngioma. Main Outcomes Measured Surgical resection of the tumor and preservation of the normal surrounding neurovascular structures. Results A 72-year-old male patient presented with a 1-year history of progressive bitemporal visual loss. He also referred symptoms suggestive of hypogonadism. Neurological examination was unremarkable and endocrine workup demonstrated mildly elevated prolactin levels. Magnetic resonance images demonstrated a large solid-cystic suprasellar lesion, consistent with the diagnosis of craniopharyngioma. The lesion was retrochiasmatic, compressed the optic chiasm, and extended into the interpeduncular cistern ( Fig. 1 ). Because of that, the patient underwent an endoscopic endonasal transplanum transtuberculum approach. 1 2 3 The nasal stage consisted of a transnasal transseptal approach, with complete preservation of the patient's left nasal cavity. 4 The cystic component of the tumor was decompressed and its solid part was resected. It was possible to preserve the surrounding normal neurovascular structures ( Fig. 2 ). Skull base reconstruction was performed with a dural substitute, a fascia lata graft, and a right nasoseptal flap ( Video 1 ). The patient did well after surgery and referred complete visual improvement. However, he also presented pan-hypopituitarism on long-term follow-up. Conclusions The endoscopic endonasal route is a good alternative for the resection of suprasellar lesions. It permits tumor resection and preservation of the surrounding neurovascular structures while avoiding external incisions and brain retraction. The link to the video can be found at: https://youtu.be/zmgxQe8w-JQ .

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.