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Teratomas account for 18-20% of all intracranial germ cell tumors and mostly occur in the pineal region with only a few cases of pediatric sellar and suprasellar teratomas described in the literature. Here, we present a case of a child with an intracranial mature teratoma with pancreatic features causing vasospasm and subsequent stroke, found to be positive for CDKN2A-an independent variant associated with malignancy and small vessel disease leading to stroke.
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Neoplasias Encefálicas , Acidente Vascular Cerebral , Teratoma , Vasoespasmo Intracraniano , Humanos , Teratoma/complicações , Teratoma/cirurgia , Teratoma/diagnóstico por imagem , Neoplasias Encefálicas/complicações , Neoplasias Encefálicas/diagnóstico por imagem , Vasoespasmo Intracraniano/etiologia , Vasoespasmo Intracraniano/diagnóstico por imagem , Acidente Vascular Cerebral/etiologia , Acidente Vascular Cerebral/diagnóstico por imagem , Acidente Vascular Cerebral/complicações , Síndromes Paraneoplásicas , Masculino , Criança , FemininoRESUMO
Regulatory T-cells (Tregs) are immunosuppressive T-cells, which arrest immune responses to 'Self' tissues. Some immunosuppressive Tregs that recognize seminal epitopes suppress immune responses to the proteins in semen, in both men and women. We postulated that GBMs express reproductive-associated proteins to manipulate reproductive Tregs and to gain immune privilege. We analyzed four GBM transcriptome databases representing ≈900 tumors for hypoxia-responsive Tregs, steroidogenic pathways, and sperm/testicular and placenta-specific genes, stratifying tumors by expression. In silico analysis suggested that the presence of reproductive-associated Tregs in GBM tumors was associated with worse patient outcomes. These tumors have an androgenic signature, express male-specific antigens, and attract reproductive-associated Related Orphan Receptor C (RORC)-Treg immunosuppressive cells. GBM patient sera were interrogated for the presence of anti-sperm/testicular antibodies, along with age-matched controls, utilizing monkey testicle sections. GBM patient serum contained anti-sperm/testicular antibodies at levels > six-fold that of controls. Myeloid-derived suppressor cells (MDSCs) and tumor-associated macrophages (TAMs) are associated with estrogenic tumors which appear to mimic placental tissue. We demonstrate that RORC-Tregs drive poor patient outcome, and Treg infiltration correlates strongly with androgen levels. Androgens support GBM expression of sperm/testicular proteins allowing Tregs from the patient's reproductive system to infiltrate the tumor. In contrast, estrogen appears responsible for MDSC/TAM immunosuppression.
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Neoplasias Encefálicas/patologia , Glioblastoma/patologia , Linfócitos do Interstício Tumoral/metabolismo , Macrófagos Associados a Tumor/metabolismo , Androgênios/metabolismo , Neoplasias Encefálicas/imunologia , Neoplasias Encefálicas/mortalidade , Proteínas de Transporte/genética , Proteínas de Transporte/metabolismo , Bases de Dados Factuais , Estrogênios/metabolismo , Feminino , Glioblastoma/imunologia , Glioblastoma/mortalidade , Humanos , Subunidade alfa do Fator 1 Induzível por Hipóxia/genética , Subunidade alfa do Fator 1 Induzível por Hipóxia/metabolismo , Estimativa de Kaplan-Meier , Linfócitos do Interstício Tumoral/imunologia , Masculino , Microglia/imunologia , Microglia/metabolismo , Membro 3 do Grupo F da Subfamília 1 de Receptores Nucleares/genética , Membro 3 do Grupo F da Subfamília 1 de Receptores Nucleares/metabolismo , Linfócitos T Reguladores/imunologia , Linfócitos T Reguladores/metabolismo , Microambiente Tumoral , Macrófagos Associados a Tumor/imunologiaRESUMO
PURPOSE: Subependymal giant cell astrocytomas (SEGAs) are rare tumors typically found in tuberous sclerosis patients. They typically grow in the region of the foramen of Monro and can occlude it, leading to hydrocephalus. Currently, gross total resection is the standard of care, with low rates of recurrence but high rates of complication, especially with larger lesions. Laser interstitial thermal therapy (LITT) is a newly emerging treatment modality for a variety of pathologies. Here, we present a case series of SEGAs managed via LITT and endoscopic, stereotactic septostomy. METHODS: A retrospective chart review was performed to identify three cases in which SEGAs were treated via LITT and septostomy. Stereotactic ablation was performed via magnetic resonance (MR) thermometry with laser output set to 69% for 2.5 min, with post-ablation scans for visualization of treatment area. RESULTS: Average age at surgery was 8.2 years. Pre-operative tumor volumes were 0.43, 1.51, and 3.88 cm3. Post-operative tumor volumes were 0.25, 0.21, and 0.68 cm3. Mean tumor volume reduction was 70%. No complications occurred. CONCLUSION: LITT with septostomy should be considered a viable primary or adjunct treatment modality for SEGAs.
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Astrocitoma , Neoplasias Encefálicas , Terapia a Laser , Astrocitoma/diagnóstico por imagem , Astrocitoma/cirurgia , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/cirurgia , Humanos , Lasers , Recidiva Local de Neoplasia , Estudos RetrospectivosRESUMO
OBJECTIVE: The authors assessed the safety and accuracy of stereoelectroencephalography (SEEG) electrode implantation in pediatric patients who had previously undergone craniotomy compared to those without prior cranial surgery. METHODS: The authors performed a retrospective analysis of patients under 25 years of age with medically refractory epilepsy at a single institution who underwent SEEG electrode placement between March 2016 and July 2023. Surgical history and demographic characteristics were collected from the electronic medical records. The coordinates of the anchor bolts and their respective SEEG electrode contacts were manually annotated using postoperative head CT scans. Bolt coordinates were used to calculate the initiated electrode trajectory set by the bolt by using the least-squares method to define a line along the bolt, projected along the length of the electrode. The shortest distance from each electrode contact to this line was calculated to obtain the error measurement. Statistical analysis was conducted using the Kolmogorov-Smirnov test to compare the distribution of errors between groups, the Student t-test was used for continuous variables, and the chi-square/Fisher's exact test was used for categorical variables. RESULTS: Fifty-eight patients underwent a total of 60 SEEG placements and met the inclusion criteria. Eighteen had a history of prior craniotomy and 40 without prior surgery, indicating entirely native cranial bone. Mean age, sex, and mean number of electrodes implanted per surgery were similar between groups. For the electrode contact furthest from the bolt, a mean (IQR) deviation of 1.32 (0.73-2.53) mm was noted for the prior craniotomy group and 1.08 (0.65-1.55) mm for the native bone group (p < 0.0001). A greater number of outliers for the contact furthest from the bolt, defined as > 6 mm from the initiated electrode trajectory, was seen in the prior craniotomy group (p < 0.0001). The complication rate was low and not statistically different between groups. CONCLUSIONS: The authors' analysis draws attention to the effect of the intracranial biomechanical environment along the path of the electrode after traversing past the anchor bolt and found that prior craniotomy was associated with a higher number of contacts with a significant deviation from the initiated trajectory. Despite these deviations, we did not find a difference in the overall low complication rate in both groups. Therefore, the authors conclude that SEEG electrode placement is a safe option in pediatric patients even after prior craniotomy.
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Background: Metastasis of systemic neoplasms to the spine is common; however, the metastasis of primary spinal cord tumors to other regions in the body is an infrequent occurrence. A few case reports have described the metastasis of primary spinal cord tumors, and in most cases, patients were younger than 30 years of age. Case Description: We present an illustrative case of a 47-year-old female with metastatic lesions to the lumbosacral vertebrae years after the initial diagnosis of an intradural, intramedullary spinal cord tumor (IMSCT). Although the surgical biopsy of the IMSCT was nondiagnostic, the patient was not found to have a separate primary neoplastic source, and the specimens of the metastatic lesions from the lumbar vertebral body were of glial origin. Conclusion: Metastasis from primary IMSCTs is extremely rare. Distant vertebral body and intracranial metastasis are even rarer yet possible. The clinical course is highly aggressive and responds poorly to current standard treatment.
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BACKGROUND: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis characterized histologically by foamy histiocytes and Touton giant cells in a background of fibrosis. Bone pain with long bone osteosclerosis is highly specific for ECD. Central nervous system involvement is rare, although dural, hypothalamic, cerebellar, brainstem, and sellar region involvement has been described. OBSERVATIONS: A 59-year-old man with a history of ureteral obstruction, medically managed petit mal seizures, and a left temporal lesion followed with serial magnetic resonance imaging (MRI) presented with worsening seizure control. Repeat MRI identified bilateral amygdala region lesions. Gradual growth of the left temporal lesion over 1 year with increasing seizure frequency prompted resection. A non-Langerhans cell histiocytosis with a BRAF V600E mutation was identified on pathology. Imaging findings demonstrated retroperitoneal fibrosis and long bone osteosclerosis with increased fluorodeoxyglucose uptake that, together with the neuropathologic findings, were diagnostic of ECD. LESSONS: This case of biopsy-proven ECD is unique in that the singular symptom was seizures well controlled with medical management in the presence of similarly located bilateral anterior mesial temporal lobe lesions. Although ECD is rare intracranially, its variable imaging presentation, including the potential to mimic seizure-associated medial temporal lobe tumors, emphasizes the need for a wide differential diagnosis.
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Background: Cauda equina syndrome (CES) is typically caused by a compressive etiology from a herniated disk, tumor, or fracture of the spine compressing the thecal sac. Here, we report a CES mimic - acute aortic occlusion (AAO), a rare disease that is associated with high morbidity and mortality. AAO can compromise spinal cord blood supply and leads to spinal cord ischemia. Case Description: Our patient presented with an acute onset of bilateral lower extremity pain and weakness with bowel/bladder incontinence, a constellation of symptoms concerning for CES. However, on initial imaging, there was no compression of his thecal sac to explain his symptomology. Further, investigation revealed an AAO. The patient underwent an emergent aortic thrombectomy with resolution of symptoms. Conclusion: AAO can mimic CES and should be considered in one's differential diagnosis when imaging is negative for any spinal compressive etiologies.
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BACKGROUND: The coronavirus (COVID-19) pandemic has caused unprecedented suspensions of neurosurgical elective surgeries, a large proportion of which involve spine procedures. The goal of this study is to report granular data on the impact of early COVID-19 pandemic operating room restrictions upon neurosurgical case volume in academic institutions, with attention to its secondary impact upon neurosurgery resident training. This is the first multicenter quantitative study examining these early effects upon neurosurgery residents caseloads. METHODS: A retrospective review of neurosurgical caseloads among seven residency programs between March 2019 and April 2020 was conducted. Cases were grouped by ACGME Neurosurgery Case Categories, subspecialty, and urgency (elective vs. emergent). Residents caseloads were stratified into junior (PGY1-3) and senior (PGY4-7) levels. Descriptive statistics are reported for individual programs and pooled across institutions. RESULTS: When pooling across programs, the 2019 monthly mean (SD) case volume was 214 (123) cases compared to 217 (129) in January 2020, 210 (115) in February 2020, 157 (81), in March 2020 and 82 (39) cases April 2020. There was a 60% reduction in caseload between April 2019 (207 [101]) and April 2020 (82 [39]). Adult spine cases were impacted the most in the pooled analysis, with a 66% decrease in the mean number of cases between March 2020 and April 2020. Both junior and senior residents experienced a similar steady decrease in caseloads, with the largest decreases occurring between March and April 2020 (48% downtrend). CONCLUSIONS: Results from our multicenter study reveal considerable decreases in caseloads in the neurosurgical specialty with elective adult spine cases experiencing the most severe decline. Both junior and senior neurosurgical residents experienced dramatic decreases in case volumes during this period. With the steep decline in elective spine cases, it is possible that fellowship directors may see a disproportionate increase in spine fellowships in the coming years. In the face of the emerging Delta and Omicron variants, programs should pay attention toward identifying institution-specific deficiencies and developing plans to mitigate the negative educational effects secondary to such caseloads reduction.
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Aneurysms of the posterior inferior cerebellar artery (PICA) are rare, with limited consensus on appropriate management. These aneurysms have been noted to have a faster growth rate and are more prone to rupture. Accessing these aneurysms for microsurgical clipping is challenging, and has traditionally required significant removal of the occipital condyle, putting the patient at risk for future complications. Therefore, some have opted to utilize minimally invasive techniques such as a pipeline stent, though these methods can fail to cause complete occlusion of the aneurysm. The current case describes a patient who was found to have a PICA aneurysm that was initially managed with a pipeline stent. However, upon further follow up, the aneurysm showed continued filling, leading to the decision to clip the aneurysm. In this case, we describe the use of a far lateral approach for accessing and clipping a PICA aneurysm with minimal removal of the occipital condyle. The patient successfully tolerated the surgery and was discharged home.
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Background and Importance: Intracranial artery fenestrations are very rare, however, when found, there is a high association with cerebral aneurysms. Clinical Presentation: This report describes a patient with multiple anterior and posterior circulation intracranial artery fenestrations and an anterior communicating artery aneurysm presenting with a thunderclap headache found to have a subarachnoid hemorrhage (SAH). The patient was treated with open surgery via clipping after a diagnostic angiography and did very well. Conclusion: There is an association between cerebral fenestrations and aneurysms, but it has not been studied in a prospective manner. This case is unusual in that the patient had both anterior and posterior circulation fenestrations, which is uncommon. Clinicians should have a high index of suspicion in patients being evaluated for SAH who have a cerebral artery fenestration with no aneurysm found.
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BACKGROUND: Overdrainage after cerebrospinal fluid diversion remains a significant morbidity. The hydrostatic, gravitational force in the upright position can aggravate this. Siphon control (SC) mechanisms, as well as programmable and flow regulating devices, were developed to counteract this. However, limited studies have evaluated their safety and efficacy. In this study, direct comparisons of the complication rates between siphon control (SC) and non-SC (NSC), fixed versus programmable, and flow- versus pressure regulating valves are undertaken. METHODS: A retrospective chart review was performed over all shunt implantations from January 2011 to December 2016 within the Houston Methodist Hospital system. Complication rates within 6 months of the operative date, including infection, subdural hematoma, malfunction, and any other shunt-related complication, were analyzed via Fisher's exact test, with P < 0.05 regarded as significant. Subgroup analyses based on diagnoses - normal pressure hydrocephalus (HCP), pseudotumor cerebri, or other HCP - were also performed. RESULTS: The overall shunt-related complication rate in this study was 19%. Overall rates of infection, shunt failure, and readmission within 180 days were 3%, 11%, and 34%, respectively. No difference was seen between SC and NSC groups in any complication rate overall or on subgroup analyses. When comparing fixed versus programmable and flow- versus pressure-regulating valves, the latter in each analysis had significantly lower malfunction and total complication rates. CONCLUSIONS: Programmable and pressure regulating devices may lead to lower shunt malfunction and total complication rates. Proper patient selection should guide valve choice. Future prospective studies may further elucidate the difference in complication rates between these various shunt designs.
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[This corrects the article DOI: 10.7759/cureus.671.].
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UNLABELLED: Cerebral cavernous malformations (CCM) are vascular malformations prone to intracerebral hemorrhage and epilepsy. Studies about the natural history and clinical presentation in the Hispanic population are lacking [7]. Retrospectively, we identified demographics and clinical features of Hispanic patients with CCM in our neurology clinic. Comparison with studies in the non-Hispanic White population with CCM was conducted. RESULTS: A total of 15 Hispanic patients diagnosed with CCM were identified in our neurology clinic. The majority of our patients presented with symptomatic hemorrhage (27%, n = 4) or seizure(s) (47%, n = 7). Of 15 patients, there were a total of four patients (27%) that had recurrent hemorrhages with an annual rate of recurrent hemorrhage of 8.04%. Our cohort had a higher rate of intracerebral hemorrhage after any initial mode of presentation (47%, n = 7) compared with non-Hispanic White population studies: 11% (n = 32) and 4% (n = 5). (p < 0.05). CONCLUSIONS: Hispanic patients with CCM have a higher rate of hemorrhage during follow-up after any presentation of CCM (p<0.05) when compared to CCM in the non-Hispanic White population.