scholarly journals Treatment strategy for multisegmental cervicomedullary ependymoma: illustrative case

2021 ◽  
Vol 2 (25) ◽  

BACKGROUND Сervicomedullary ependymoma (CME) is a rare tumor of the central nervous system. The CME treatment strategy is insufficiently represented in the literature and is a complex task for neurosurgeons. OBSERVATIONS The authors describe an infrequent case of a large multisegmental CME that extended from the medulla oblongata to the cervical spinal cord at the level of the sixth cervical vertebra in a 21-year-old female. Neurological disorders presented with headache, dysphagia, hypophonia, and weakness in the limbs. Subtotal removal of the tumor was performed according to intraoperative neurophysiological monitoring (IONM) results. A wait-and-see approach with patient follow-up was chosen. LESSONS Total tumor removal of the CME is the most important favorable prognostic factor. Subtotal resection can be considered if the borders of the tumor are unclear and the result of IONM is unfavorable. The role of postoperative radiation therapy in the case of subtotal removal of the tumor remains controversial.


2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii189-ii189
Author(s):  
Philip Haddad ◽  
Furqan Akhtar ◽  
Kevin Gallagher

Abstract BACKGROUND Although meningiomas are among the most prevalent types of brain tumors, AMs account for around 4% of all meningiomas. AMs tend to be more aggressive with relatively higher rates of recurrence and mortality. Gross total resection (GTR) has been the standard of care when possible. However, GTR itself is not always enough to prevent the recurrence of AMs. The role of PORT remains controversial in AM as the comparative studies to support its use have provided conflicting RESULTS: The purpose of this meta-analysis is to evaluate the impact of PORT on clinical outcomes according to the extent of resection in AMs. METHODS A review of the medical literature was conducted using online databases. Inclusion criteria consisted of AM diagnosis, English language, Simpson graded resections, and comparative studies reporting recurrence rates (RcR), Progression-Free Survival (PFS), and Overall Survival (OS) with hazard ratios (HR) or Kaplan-Meier curves. A meta-analysis was conducted using an inverse variance method with a random-effects model. RESULTS Twenty-two comparative studies with a total of 5,129 patients were included and analyzed. When GTR was attained, PORT was associated with improved RcR (HR =0.72, 95%CI:0.59-0.86) and PFS (HR=0.77, 95%CI:0.65-0.90), but not OS (HR=0.93, 95%CI:0.83-1.04). When subtotal resection (STR) was attained, PORT was associated with improved PFS (HR=0.35, 95%CI:0.26-0.48) as well as OS (HR=0.70, 95%CI:0.54-0.89). The extent of surgery also impacted AM outcomes as GTR demonstrated superior PFS (HR=0.45, 95%CI:0.31-0.65) and OS (HR=0.30, 95%CI:0.13-0.72). CONCLUSIONS This is the first meta-analysis to show that PORT is associated with PFS benefit in AMs with GTR and STR. Moreover, PORT significantly improved OS of AMs that underwent STR but had no impact on OS when GTR was achieved. In the absence of randomized clinical trials, this meta-analysis represents the most compelling data supporting the use of PORT in this patient population.



2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii276-iii276
Author(s):  
Olga Zheludkova ◽  
Lyudmila Olkhova ◽  
Yuri Kushel’ ◽  
Armen Melikyan ◽  
Marina Ryzhova ◽  
...  

Abstract We analyzed 105 patients under 18 years. The median age was 21 months. There were 54 boys and 51 girls. The supratentorial tumors were in 53 patients, infratentorial in 48, and in spinal cord in 4. 60 had stage M0,29-М+and 16-Mx. All the patients got surgical treatment:total tumor removal in 34,subtotal in 37,partial in 30,and biopsy in 4;75 patients got chemoradiotherapy to ATRT-2006;6-CWS;13-EU-RHAB;5-HIT-SKK;individual schemes in 6. RESULTS: 47 are alive,1 was LFU, and 57 died. PFS was 32%±0.05; the five-year OS 40%±0.05. The median survival-30 months, the median progression-free survival-12 months, and the median of follow-up-23 months. PFS was significantly better in patients more than 12 months compared to patients younger than 12 months:40 and 12%;p=0.00161.After total resection PFS was higher compared to subtotal resection, partial resection, and tumor biopsy:48,38,0,and 0%(p=0.025). After chemoradiotherapy, PFS was higher compared to patients without radiotherapy: 49and 0%(р=0.0000000).PFS for stage M0 was higher compared to stage M+and stage Mx:41,15,and 27%,respectively(р=0.00032).PFS was better for the tumors in the spinal cord and infratentorial location compared to the supratentorial location:67,37,and 25%(p=0.0876).The survival rate was higher among the patients who got treatment according to the ATRT-2006 protocol compared to EU-RHAB, individual regimens, CWS, and HIT-SKK:39,19,17,17,and 0% respectively;p=0.00159.The survival was higher among the patients who got intraventricular/intrathecal Methotrexate,Cytarabine, Prednisolone than among the patients who got only Methotrexate or none at all:40,0,and 5%, respectively; p=0.00015. CONCLUSIONS: Survival was significantly better in patients more than 12month, without metastases, with total removal tumor, chemotheradiotherapy by ATRT-2006 protocol with i/t, i/v Methotrexate/Cytarabine/Prednisolone.



2012 ◽  
Vol 11 (3) ◽  
pp. 242-244
Author(s):  
Marcelo Campos Moraes Amato ◽  
Caio César Marconato Simões Matias ◽  
João Alberto Assirati Junior ◽  
Aline Paixão Becker ◽  
Carlos Gilberto Carlotti Junior ◽  
...  

Hemangioblastomas of the central nervous system (CNS) are low-grade highly vascularized tumors that may be sporadic or associated with Von Hippel-Lindau disease. Extradural hemangioblastomas are uncommon and those located extra and intradurally are even rarer. This study uses an illustrative case and literature review to discuss the difficulties to consider the correct diagnosis and to select the best surgical approach. A 57 years-old white male patient presented with myelopathy and right C5 radiculopathy. The images showed a lobulated, hourglass shaped, highly enhanced extra/intradural lesion that occupied the spinal canal and widened the C4-C5 right intervertebral foramen. Total resection of the intradural lesion was achieved through a posterior approach, but the extradural part could only be partially removed. Complete improvement was observed after four months of follow-up and the residual tumor has been followed up clinically and radiologically. Even though the preoperative impression was of a spinal schwannoma, the histopathological examination revealed grade I hemangioblastoma as per WHO. Despite their rarity, current complementary exams allow considering the diagnosis of hemangioblastoma preoperatively. That is essential to a better surgical planning in view of the particular surgical features of this lesion.



2021 ◽  
Vol 2 (2) ◽  
Author(s):  
Yoshiteru Shimoda ◽  
Shinya Sonobe ◽  
Kuniyasu Niizuma ◽  
Toshiki Endo ◽  
Hidenori Endo ◽  
...  

BACKGROUND An arteriovenous fistula is an abnormal arteriovenous shunt between an artery and a vein, which often leads to venous congestion in the central nervous system. The blood flow near the fistula is different from normal artery flow. A novel method to detect the abnormal shunting flow or pressure near the fistula is needed. OBSERVATIONS A 76-year-old woman presented to the authors’ institute with progressive right upper limb weakness. Right vertebral angiography showed a fistula between the right extracranial vertebral artery (VA) and the right vertebral venous plexus at the C7 level. The patient underwent endovascular treatment for shunt flow reduction. Before the procedure, blood pressures were measured at the proximal VA, distal VA near the fistula, and just at the fistula and drainer using a microcatheter. The blood pressure waveforms were characteristically different in terms of resistance index, half-decay time, and appearance of dicrotic notch. The fistula was embolized with coils and N-butyl cyanoacrylate solution. LESSONS During endovascular treatment, the authors were able to digitally record the vascular pressure waveform from the tip of the microcatheter and succeeded in calculating several parameters that characterize the shunting flow. Furthermore, these parameters could help recognize the abnormal blood flow, allowing a safer endovascular surgery.





PEDIATRICS ◽  
1958 ◽  
Vol 22 (6) ◽  
pp. 1064-1073
Author(s):  
Raymond F. Hain ◽  
Gerald D. LaVeck

Metachromatic leuko-encephalopathy is a familial degenerative disease of the central nervous system included with Schilder's disease as a type of diffuse cerebral sclerosis. The disease usually has its onset early in childhood and is characteried by progressive motor and mental deterioration with ataxia, muscular weakness, spasticity, optic atrophy, convulsions and finally dementia. The concentration of protein of the cerebrospinal fluid is frequently elevated. The pathologic findings consist of demyelination, destruction of axons, gliosis and the accumulation of metachromatic granules in the brain and other organs. The metachromasia can be demonstrated in formalin-fixed frozen sections with a toluidine blue stain. It is not demonstrable in paraffin sections. Histochemical studies indicate this abnormal material is probably a complex of glycolipids and protein. It has been reported elsewhere that early diagnosis can be established by demonstrating the metachromatic materials in urinary sediment or in a real biopsy. An illustrative case has been presented.



Author(s):  
Nick Ward

Residual upper limb dysfunction after injury to the central nervous system is a major clinical, socioeconomic and societal problem. Upper limb dysfunction can occur in many disorders of the central nervous system including cervical spinal cord injury and multiple sclerosis, but therapeutic approaches for upper limb dysfunction after stroke are the most thoroughly investigated. General approaches to treatment require:�(i)�avoidance of complications such as spasticity, pain, and loss of range; (ii) early high-dose engaging functional motor training; (iii) consideration of how neuroplastic processes might be engaged to enhance the effects of training. The evidence to deliver optimal personalized treatment strategies for all patients is lacking, but there is evidence that higher doses and intensity of upper limb therapy will be beneficial to most patients. Recent work has focused on how technological innovation might be used to promote recovery of upper limb function.



Author(s):  
Marc Kent ◽  
Eric N. Glass ◽  
Lindsay Boozer ◽  
Rachel B. Song ◽  
Elyshia J. Hankin ◽  
...  

ABSTRACT Two cats were presented with multifocal neurological signs. One cat’s signs progressed over 2 wk; the other cat progressed over 5 days. Examinations were consistent with a process involving the prosencephalon, vestibular system, and general proprioceptive/upper motor neuron systems. MRI of the brain and cervical spinal cord reveal widespread T2 hyperintensity of the white matter. Affected areas included the cerebrum, cerebral peduncles, corticospinal tracts of the pons and medulla, and the cerebellum. T2 hyperintensity was present in all funiculi of the spinal cord. Diffusion-weighted imaging (DWI) and apparent diffusion coefficient (ADC) maps were consistent with cytotoxic or intramyelinic edema. Differential diagnosis included toxic or metabolic/degenerative leukoencephalopathies. Necropsies revealed widespread spongy degeneration of the central nervous system white matter. Toxicologic assays of liver specimens revealed desmethylbromethalin, a metabolite of bromethalin. Bromethalin is a rodenticide that causes uncoupling of oxidative phosphorylation. Antemortem diagnosis is challenging. DWI and ADC maps were instrumental in narrowing the differential diagnosis and raised the index of suspicion for bromethalin. Bromethalin intoxication should be considered in all animals with a progressive course of multifocal neurologic deficits. MRI, specifically, DWI and ADC maps, may serve as a biomarker of cytotoxic or intramyelinic edema associated with spongiform leukoencephalomyelopathy.



Neurosurgery ◽  
2009 ◽  
Vol 64 (2) ◽  
pp. E387-E388 ◽  
Author(s):  
Riccardo Ciarpaglini ◽  
Ernesto Pasquini ◽  
Diego Mazzatenta ◽  
Andrea Ambrosini-Spaltro ◽  
Vittorio Sciarretta ◽  
...  

Abstract OBJECTIVE Purely intradural clival chordomas are rare neoplasms, and only a few cases have been reported. The reported cases present features similar to ecchordosis physaliphora, which is a notochordal remnant. We describe these 2 entities and their differential diagnoses, clinical courses, and management. This is the first reported case to be treated using a neuroendoscopic technique. CLINICAL PRESENTATION A 60-year-old man presenting with memory loss underwent magnetic resonance imaging, which revealed an intradural retroclival mass without bone involvement. INTERVENTION The patient underwent an endoscopic transsphenoidal-transclival procedure with subtotal removal of the tumor. Histological findings confirmed the diagnosis of a chordoma. CONCLUSION Even if some parameters exist for a differential diagnosis, ecchordosis physaliphora and intradural chordoma may represent different aspects of the spectrum of the same pathology. Intradural clival chordomas have a better prognosis with respect to classic chordomas. Therefore, in subtotal removal such as that performed in our case, postoperative radiation therapy should be performed only if a regrowth of the remnant is seen during neuroradiological follow-up.



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