scholarly journals RARE-43. PRIMARY CENTRAL NERVOUS SYSTEM EWING SARCOMA: A SINGLE INSTITUTION EXPERIENCE

2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi230-vi231
Author(s):  
Florencia Yorio ◽  
Lucas Alessandro ◽  
Naomi Arakaki ◽  
Nicolas Palomar ◽  
Alejandro Muggeri ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Ewing Sarcoma ◽  
Brain Mri ◽  
Case Series ◽  
Progression Free Survival ◽  
Whole Body ◽  
Focal Neurological Deficit ◽  
Primary Tumors ◽  
Clinical Records

Abstract INTRODUCTION Ewing Sarcoma (ES) is defined by molecular markers, being t(11,22) the most frequent. Intracranial ES/pPNET usually represent metastases from extracranial sites. Primary Central Nervous System (CNS) lesions are extremely rare. MATERIAL AND METHODS Retrospective review of clinical records from patients with primary CNS ES/pPNET assessed at a neurological center in Argentina between 2007–2019. All confirmed with molecular marker. Clinical characteristics, imaging, histopathology, and treatment response were evaluated. Extensive workup included whole-body CT scan, skeletal-scintigraphy, and positron-emission tomography, excluding extracranial primary lesions in all cases. RESULTS Total 24p. Median age 22yo (2–65); M:F 2:1. Clinical presentation: intracranial hypertension, focal neurological deficit or seizures. In brain MRI 11 supratentorial lesions, 7 infratentorial and 6 diffuse leptomeningeal. Histopathology: diffuse pattern with small round blue cells 13/24p, other patterns were also described. CD99 marked positive in all cases and T(11.22) confirmed as well: 22 PCR and 2 FISH for EWSR1-rearrangement. Misdiagnosis lead to median delay for accuracy of 7,5mo (0–124); including other CNS primary tumors (n= 13), infectious diseases (n= 5) and other diagnoses (n= 4). Most patients (n= 10) were treated with Euro99-protocol (6 cycles plus local radiotherapy (RT)), 4 with Temozolomide, Irinotecan plus RT, 7 with other regimens and 2 received no treatment. Mean overall survival (OS): 58mo (0–132) and progression-free survival 22mo (0–85). Five year OS:41%. CONCLUSION Clinical behavior in primary CNS ES deferred from extraosseous ES, being necessary to apply a specific regimen for this pathology as there is no standard regime for intracranial ES/pPNET. Results showed two different groups of patients with long and very short survival. Presentation, histology, and outcomes are so uneven that we propose if a more specific molecular classification should be necessary. This is, to our knowledge, the largest case series reported describing a heterogeneous group despite the presence of accurate molecular diagnosis.

scholarly journals LINC-05. PRIMARY CENTRAL NERVOUS SYSTEM EWING SARCOMA IN PEDIATRIC AND AYA PATIENTS: 2 INSTITUTIONS EXPERIENCE IN BUENOS AIRES ARGENTINA

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii378-iii379
Author(s):  
Roberto Nicolas Palomar ◽  
Florencia Yorio ◽  
Lucas Alessandro ◽  
Naomi Arakaki ◽  
Alejandro Muggeri ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Treatment Response ◽  
Ewing Sarcoma ◽  
Brain Mri ◽  
Correct Diagnosis ◽  
Accurate Diagnosis ◽  
Molecular Testing ◽  
Clinical Records ◽  
Leptomeningeal Infiltration

Abstract INTRODUCTION Ewing Sarcoma (ES) is defined by molecular markers, being t(11;22)(q24;q12) the most frequent. Intracranial ES usually shows as metastases from extracranial sites. Primary central nervous system (CNS) lesions are extremely rare. MATERIAL AND METHODS Retrospective review of clinical records from patients with primary CNS ES, assessed at 2 institutions in Argentina between 2007–2019. Translocation was evidenced in all cases through molecular testing. Clinical characteristics, imaging, histopathology, and treatment response were evaluated. Extracranial and osseous lesions were excluded. RESULTS 15 patients. Median age at beginning of symptoms: 8 yo (2–20). Most patients had intracranial hypertension syndrome (14/15). In brain MRI, 5/15 supratentorial lesions, 4/15 posterior fosa, 1/15 medullary, 2/15 supra and infratentorial, and 3/15 lesions diffuse leptomeningeal infiltration. Histopathologic findings showed diffuse pattern with small round blue cells in most cases, other patterns were also described. CD99 marked positive in all cases. Misdiagnosis with glial tumors (4/15), medulloblastoma (6/15) and infectious diseases (3/15); led to median delay to accurate diagnosis of 3 months (range 0–67). After correct diagnosis patients were treated with standard ES treatment (6 VIDE cycles plus radiotherapy) in 14/15 patients. Vincristine, irinotecan and temozolamide was used as second line treatment in all relapse cases whenever possible. EFS was 22 months (2- 65). OS at 5 years of follow-up was 46,67% (mean OS 31 mo). CONCLUSION Even though molecular assessment led to accurate diagnosis in all cases, treatment response and outcome showed two different groups of patients with long and very short survival. Adaptative therapy should be considered.

scholarly journals OTHR-04. Gynecological Malignancies With Metastasis To The Central Nervous System: A Case Series and Systematic Review of the Literature

2021 ◽  
Vol 3 (Supplement_3) ◽  
pp. iii15-iii15
Author(s):  
Azeem Sajjad ◽  
Adeleso Adesina ◽  
Penelope Halkiadakis ◽  
Kelsey Murphy ◽  
Kathleen Mulligan ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Overall Survival ◽  
Nervous System ◽  
Literature Review ◽  
Metastatic Disease ◽  
Case Series ◽  
Progression Free Survival ◽  
Gynecologic Malignancies ◽  
Free Survival ◽  
Cns Metastasis

Abstract Introduction Gynecologic malignancies are an increasingly common proportion of central nervous system metastatic disease. As genetic sequencing technology improves and becomes more accessible, mutations associated with CNS metastasis are easier to elucidate. The aims of this case series and systematic literature review are to describe the patient population with CNS metastatic disease from a gynecologic primary, and to investigate why the proportion of CNS metastasis from gynecologic malignancies is increasing. Ultimately, we hope to improve understanding of this subset of metastatic CNS malignancies and improve management strategies. Methods A literature review of articles describing patients from 1990–2020 who were diagnosed with CNS metastasis from a known gynecologic primary malignancy was performed. Demographics, cancer type, mutation characteristics, management for metastatic disease, progression free survival, number of CNS metastases, and location of metastatic disease were assessed. Inclusion criteria were age>18 years, diagnosis of primary ovarian, uterine, or cervical cancer with confirmed metastatic disease to the CNS, including brain parenchyma, leptomeninges, or intradural spinal cord or dural metastases. Exclusion criteria included pediatric population and bony metastases (e.g., bony spine metastases without evidence of meningeal/parenchymal invasion). Results Our review showed that patients with gynecological metastasis to the CNS generally have worse outcomes regarding overall survival, progression free survival, and quality of life than patients without CNS metastasis. Discussion Our results infer that the reported increase in incidence of CNS metastasis from gynecologic malignancies is a reflection of improvement of detection given advances in technology, improved patient follow up, and increased overall survival of patients with gynecologic malignancies. Further characterization of mutations from gynecologic malignancies associated with brain metastasis could result in development of more treatment options for patients in the future and help determine factors that contribute to developing metastasis to the CNS of various degrees, thus, potentially inform treatment strategies.

scholarly journals RARE-45. SARCOMAS INVOLVING THE CENTRAL NERVOUS SYSTEM AT INITIAL PRESENTATION IN CHILDREN AND YOUNG ADULTS: A CASE SERIES

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii452-iii452
Author(s):  
Yen-Lin Liu ◽  
Shu-Mei Chen ◽  
Hsin-Lun Lee ◽  
Jia-Hui Huang ◽  
Shu-Huey Chen ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Ewing Sarcoma ◽  
Case Series ◽  
Stage Iv ◽  
Multimodality Treatment ◽  
Myeloid Sarcoma ◽  
Initial Presentation ◽  
Nerve Sheath Tumor ◽  
The Central Nervous System

Abstract Sarcomas of bone, soft tissue, or neural origin may occasionally invade the central nervous system (CNS), causing diagnostic and therapeutic challenges. We aim to investigate the clinical features of sarcomas involving the CNS at initial presentation. During 2015/01–2019/12, nine consecutive patients (4 Males and 5 Females) younger than 30 years of age treated at a University Healthcare System in Northern Taiwan were included. The median age was 8.7 years (range, 2–24 years); diagnoses were Ewing Sarcoma with EWSR1 rearrangements (n=4), CIC-NUTM1 Sarcoma (n=1), Osteosarcoma (n=2), Malignant Peripheral Nerve Sheath Tumor (MPNST; n=1), and extramedullary myeloid sarcoma (n=1). The tumors originated from the skull (n=1), dura (n=1), vertebra (n=4), spinal canal (n=1), or extra-CNS sites (n=2). Four patients had metastases (1 Ewing sarcoma, 2 osteosarcoma, and 1 extramedullary myeloid sarcoma). The main symptom at diagnosis was facial/eye pain (n=2), back pain (n=3), arm weakness (n=1), or gait disturbance (n=3). Upfront neurosurgical decompression (n=7) or urgent radiotherapy (n=1) was performed in most patients. At a median follow-up duration of 20.1 months, the overall survival rate was 70%. All patients with Ewing sarcoma (n=4) and CIC-NUTM1 sarcoma (n=1) achieved Complete Response after surgery, interval-compressed chemotherapy, radiotherapy, and adjuvant chemotherapy. Patients with stage IV osteosarcoma (n=2) had Partial Response; the patients with MPNST and extraskeletal myeloid sarcoma died of Progressive Disease at 18 and 3 months after diagnosis, respectively. We conclude that timely decompression, early diagnosis, and histology-driven multimodality treatment are effective strategies in managing sarcomas involving the CNS.

scholarly journals COVID-19 Accompanied with Intracerebral Hemorrhage: A Case Series

2020 ◽  
Vol 15 (4) ◽  
Author(s):  
Athena Sharifi-Razavi ◽  
Zahra Sedaghat ◽  
Mana Baziboroun ◽  
Narges Karimi
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Intracerebral Hemorrhage ◽  
Central Nervous System Involvement ◽  
Case Series ◽  
Severe Illness ◽  
Cerebral Vein ◽  
Focal Neurological Deficit ◽  
Vein Thrombosis ◽  
Number Of Patients

Introduction: The new coronavirus, also known as COVID-19, can potentially involve in the central nervous system. The most important neurological manifestations include dizziness, headache, hypogeusia, hyposmia, ataxia, seizure, ischemic stroke, cerebral hemorrhage, encephalopathy, encephalitis, meningitis, seizure, cerebral vein thrombosis, and Guillain-Barre syndrome. Case Presentation: In this case series, we reported five patients with consciousness alteration and focal neurological deficit and neuroimaging that is consistent with intracerebral hemorrhage. In all patients, there was an association with COVID-19 infection. Conclusions: While the neurological manifestation of COVID-19 has not been appropriately defined, it is possible that a number of patients, particularly those who suffer from a severe illness, had central nervous system involvement. Thus, the neurologists should be aware of the likelihood of any neurological symptoms of COVID-19 infection.

scholarly journals Giant central nervous system tuberculoma in pediatric patients: surgical case series

2021 ◽  
Author(s):  
Xiao Xiao ◽  
Qiang Li ◽  
Yan Ju
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Basal Ganglia ◽  
Third Ventricle ◽  
Case Series ◽  
Cerebellar Lesion ◽  
Thalamic Lesion ◽  
Tb Treatment ◽  
Frontal Horn ◽  
Clinical Records

AbstractCentral nervous system tuberculoma is rare and challenging situation. Clinical records of patients with pathologically proven tuberculoma were retrospectively reviewed. Clinical presentation, lesion location, radiological characteristics, perioperative and surgical management, and outcome is summarized and analyzed. Eight patients were included and there was one girl. Age ranged from 3 to 14 years with mean age 9.8 years. Clinical duration ranged from 20 days to 2 years, and 3 patients had previous lung tuberculosis with anti-TB treatment. The lesion was in cerebellum in 6 cases, including 1 involving basal ganglia and 1 involving thalamus. The lesion was in basal ganglia, thalamus, and third ventricle in 1 case, and in T12-L1 spinal cord in another. Cerebellar lesion was resected via paramedian suboccipital approach in 5 patients, basal ganglia lesion via trans-cortical frontal horn approach in 2 patients, and intra-spinal lesion via trans-laminar approach in 1 patient. Follow-up ranged from 10 to 24 months. Of the 8 patients, 6 returned to normal life. One patient had cerebellar lesion resected and the thalamic lesion reduced in size after anti-TB treatment. One patient died from TB spreading. Our data showed that most patients can be successfully treated by resection of the lesion. Low T2 signal, ring shaped enhancement and peripheral edema strongly suggest tuberculoma. Empirical anti-TB treatment should be initiated perioperatively.

scholarly journals Cytology of Extraneural Metastases of Nonhematolymphoid Primary Central Nervous System Tumors: Six Cases with Histopathological Correlation and Literature Update

2021 ◽  
Vol 65 (6) ◽  
pp. 529-540
Author(s):  
Joerg Schwock ◽  
Lorna Mirham ◽  
Zeina Ghorab
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Tertiary Care ◽  
Case Series ◽  
Cns Tumors ◽  
Salivary Gland Neoplasm ◽  
Time Interval ◽  
Myxopapillary Ependymoma ◽  
Primary Tumor Site ◽  
Primary Tumors

<b><i>Introduction:</i></b> Extraneural/-cranial metastases (ENM) of primary central nervous system (CNS) tumors are rare and may be diagnostically challenging. We describe the cytomorphological and pertinent clinical features of ENM in a case series assessed by fine-needle aspiration (FNA). A search of the laboratory information systems of 2 tertiary care centers in Toronto (2000–2015) was performed. Cases with direct extracranial/-spinal extension of CNS neoplasms were excluded. Microscopic slides of FNA and surgical specimens were reviewed. Demographic and clinicopathological data were retrieved. <b><i>Case Presentation:</i></b> Six cases were identified with the original diagnoses of glioblastoma, glioblastoma with primitive neuroectodermal tumor-like components, anaplastic ependymoma, myxopapillary ependymoma, atypical meningioma, and hemangiopericytoma. Median patient age at first diagnosis was 44 years (range 22–56). The time interval between initial diagnosis and first metastatic disease manifestation was 3 months to 19 years. All FNA diagnoses were rendered correctly. In 4 cases, immunohistochemistry was used to support the diagnosis. All cases had prior surgical intervention at the primary tumor site. In 4 cases, the ENM location was the ipsilateral parotid or buccal area. Two primary tumors in midline location developed ENM in the scapular area. <b><i>Discussion/Conclusion:</i></b> ENM are a rare manifestation of a range of different primary CNS tumors and may involve the ipsilateral head and neck mimicking clinically a salivary gland neoplasm. FNA can rapidly discriminate ENM from other, potentially more indolent conditions. Awareness of the clinical history is paramount to avoid diagnostic confusion.

Synthesis and Evaluation of 18F-INER-1577-3 as a Central Nervous System (CNS) Histone Deacetylase Imaging Agent

2020 ◽  
Vol 16 (8) ◽  
pp. 978-990
Author(s):  
Ming-Hsin Li ◽  
Han-Chih Chang ◽  
Chun-Fang Feng ◽  
Hung-Wen Yu ◽  
Chyng-Yann Shiue
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Histone Deacetylases ◽  
Authentic Sample ◽  
Cancer Cell Line ◽  
Radiochemical Yield ◽  
Imaging Agent ◽  
Whole Body ◽  
Imaging Agents ◽  
Synthesis Time

Background:: Epigenetic dysfunction is implicated in many neurologic, psychiatric and oncologic diseases. Consequently, histone deacetylases (HDACs) inhibitors have been developed as therapeutic and imaging agents for these diseases. However, only a few radiotracers have been developed as HDACs imaging agents for the central nervous system (CNS). We report herein the synthesis and evaluation of [18F]INER-1577-3 ([18F]5) as an HDACs imaging agent for CNS. Methods:: [18F]INER-1577-3 ([18F]5) was synthesized by two methods: one-step (A) and two-step (B) methods. Briefly, radiofluorination of the corresponding precursors (11, 12) with K[18F]/K2.2.2 followed by purifications with HPLC gave ([18F]5). The quality of [18F]INER- 1577-3 synthesized by these methods was verified by HPLC and TLC as compared to an authentic sample. The inhibitions of [18F]INER-1577-3 and related HDACs inhibitors on tumor cells growth were carried out with breast cancer cell line 4T1 and MCF-7. The whole-body and brain uptake of [18F]INER-1577-3 in rats and AD mice were determined using a micro-PET scanner and the data was analyzed using PMOD. Results: : The radiochemical yield of [18F]INER-1577-3 synthesized by these two methods was 1.4 % (Method A) and 8.8% (Method B) (EOB), respectively. The synthesis time was 115 min and 100 min, respectively, from EOB. The inhibition studies showed that INER-1577-3 has a significant inhibitory effect in HDAC6 and HDAC8 but not HDAC2. PET studies in rats and AD mice showed a maximum at about 15 min postinjection for the whole brain of a rat (0.47 ± 0.03 %ID/g), SAMP8 mice (5.63 ± 1.09 %ID/g) and SAMR1 mice (7.23 ± 1.21 %ID/g). Conclusion:: This study showed that INER-1577-3 can inhibit tumor cell growth and is one of a few HDACs inhibitors that can penetrate the blood-brain barrier (BBB) and monitor HDAC activities in AD mice. Thus, [18F]INER-1577-3 may be a potent HDACs imaging agent, especially for CNS.

scholarly journals Treatment of secondary central nervous system involvement in systemic aggressive B cell lymphoma using R-MIADD chemotherapy: a single-center study

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Yuchen Wu ◽  
Xuefei Sun ◽  
Xueyan Bai ◽  
Jun Qian ◽  
Hong Zhu ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Central Nervous System Involvement ◽  
Central Nervous System Lymphoma ◽  
Progression Free Survival ◽  
B Cell Lymphoma ◽  
High Dose ◽  
Cns Involvement ◽  
The Central Nervous System ◽  
Secondary Central Nervous System

Abstract Background Secondary central nervous system lymphoma (SCNSL) is defined as lymphoma involvement within the central nervous system (CNS) that originated elsewhere, or a CNS relapse of systemic lymphoma. Prognosis of SCNSL is poor and the most appropriate treatment is still undetermined. Methods We conducted a retrospective study to assess the feasibility of an R-MIADD (rituximab, high-dose methotrexate, ifosfamide, cytarabine, liposomal formulation of doxorubicin, and dexamethasone) regimen for SCNSL patients. Results Nineteen patients with newly diagnosed CNS lesions were selected, with a median age of 58 (range 20 to 72) years. Out of 19 patients, 11 (57.9%) achieved complete remission (CR) and 2 (10.5%) achieved partial remission (PR); the overall response rate was 68.4%. The median progression-free survival after CNS involvement was 28.0 months (95% confidence interval 11.0–44.9), and the median overall survival after CNS involvement was 34.5 months. Treatment-related death occurred in one patient (5.3%). Conclusions These single-centered data underscore the feasibility of an R-MIADD regimen as the induction therapy of SCNSL, further investigation is warranted.

scholarly journals ML-23 The outcome of malignant lymphoma of the central nervous system in a single institution

2020 ◽  
Vol 2 (Supplement_3) ◽  
pp. ii18-ii18
Author(s):  
Kiyonori Kuwahara ◽  
Shigeo Ohba ◽  
Kazuyasu Matsumura ◽  
Saeko Higashiguchi ◽  
Daijiro Kojima ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Adverse Events ◽  
Malignant Lymphoma ◽  
Survival Time ◽  
Progression Free Survival ◽  
Treatment Method ◽  
The Other ◽  
High Dose ◽  
Other Hand

Abstract Background: Although high dose-methotrexate therapy has been performed for primary central nervous system malignant lymphoma (PCNSL), R-MPV (rituximab, methotrexate (MTX), procarbazine and vincristine) therapy is currently the first line therapy for (PCNSL) in our hospital. This study examines the results of R-MPV therapy comparing with past treatment. Method/Subjects: Thirty-seven patients treated at our hospital from 2009 to 2020 were included. Overall survival time, progression free survival time, and toxicities were evaluated. Results: The average age of patients was 65.7 years. Patients included 21 males and 16 females. Thirty-six patients were diagnosed DLBCL by resected brain tumor tissues, and one was diagnosed DLBCL by vitreous biopsy. As initial treatment, rituximab±HD-MTX therapy (R±MTX group) was performed in 20 cases, HD-MTX therapy plus radiation (R±MTX+RT group) was performed in 12 cases, and RMPV therapy was performed in 5 cases (R-MPV group). Median OS of all cases was 69 months and median PFS was 38 months. Median OS was 69 months in R±MTX group and could not be calculated in R±MTX+RT, and R-MPV groups. Median PFS was 16 months and 56 months in R±MTX group and R±MTX+RT, respectively, and could not be calculated in the R-MPV group. Although the R-MPV group had a short follow-up period, the results were considered to be comparable to those of the R±MTX+RT group. On the other hand, grade 3/4 adverse events occurred in 50%, 25%, and 100%, respectively. Conclusion: R-MPV therapy may delay the timing of radiation and reduce the amount of radiation. On the other hand, the frequency of adverse events is high, and more strict management of treatment is required.

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