Expression of STAT6 and Phosphorylated STAT6 in Primary Central Nervous System Lymphomas

2021 ◽  
Author(s):  
Georgia Karpathiou ◽  
Elise Ferrand ◽  
Florian Camy ◽  
Silvia-Maria Babiuc ◽  
Alexandra Papoudou-Bai ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Clinical Performance ◽  
Prognostic Score ◽  
Performance Status ◽  
Disease Recurrence ◽  
Response To Treatment ◽  
Cytoplasmic Expression ◽  
Phosphorylated Form ◽  
Current Series

Abstract The signal transducer and activator of transcription 6 (STAT6) is implicated in the pathogenesis of some lymphomas including primary central nervous system lymphomas (PCNSLs). The aim of this study was to investigate STAT6 expression and clinicopathologic features in 25 PCNSLs using immunohistochemistry with 2 different anti-STAT6 antibodies. One (YE361) recognizes the C-terminus domain of the STAT6 protein and the other (Y641) recognizes the phosphorylated form of the protein. The phosphorylated STAT6 form was not expressed in any of the cases studied whereas the YE361 STAT6 showed only cytoplasmic expression in 14 (56%) cases. This expression did not correlate with age, prognostic score, multiplicity, invasion of deep structures, response to treatment, disease recurrence, overall survival, or BCL6, BCL2, PD-L1, and CD8 expression. A STAT6 expression score showed a trend for correlating with clinical performance status. It also showed a positive correlation with MYC expression. Thus, the phosphorylated form of STAT6 was not found in the current series, while the YE361 STAT6 showed only cytoplasmic expression and was associated with expression of MYC.

scholarly journals LINC-39. PERFORMANCE STATUS OF PEDIATRIC PATIENTS WITH CENTRAL NERVOUS SYSTEM TUMORS TREATED IN MEXICO, A SINGLE-CENTER EXPERIENCE

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii385-iii386
Author(s):  
Claudia Madrigal-Avila ◽  
Alfonso Perez-Bañuelos ◽  
Rafael Ruvalcaba-Sanchez ◽  
Lourdes Vega-Vega ◽  
Gabriela Escamilla-Asiain
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Multidisciplinary Approach ◽  
Pediatric Patients ◽  
Performance Status ◽  
Retrospective Chart Review ◽  
Cns Tumors ◽  
Surgical Morbidity ◽  
Tertiary Center

Abstract BACKGROUND Central nervous system (CNS) tumors are the most common solid neoplasms in the pediatric age, they comprise about a quarter of all cancers at this age. Little is known about the specific epidemiology of this group in Mexico and there are no reports of results focused on the Performance Status of patients who are treated in a multidisciplinary setting. OBJECTIVE To describe the Performance Status of CNS pediatric patients after being treated with a multidisciplinary approach in a tertiary center. METHODS We report a retrospective chart review of all pediatric patients who presented to the Neuro-Oncology Clinic at Teleton Pediatric Oncology Hospital in Queretaro, Mexico, from December 2014 to January 2020. We analyzed age, gender, the extent of surgical resection and histopathology. Performance Status was assessed using ECOG and Karnofsky/Lansky scores during every patient’s last follow-up visit. RESULTS A total of 56 patients were treated, epidemiology and histopathology variants are similar to those described in the international literature. With a median follow-up of 33 months, 35 patients are alive (62.5%), 28 of them (74.2%) have an excellent Performance Status (ECOG score 0 or Lansky/Karnofsky ≥ 90), 5 (14.2%) scored ECOG 1–2 and only 4 (11.4%) scored ECOG 3–4. CONCLUSIONS A multidisciplinary approach with a focus on Performance Status and the potential for neurological recovery is essential in the management of pediatric patients with CNS tumors. Efforts should be aimed at reducing post-surgical morbidity and early rehabilitation to reintegrate patients into society in the long term.

International Primary Central Nervous System Lymphoma Collaborative Group (IPCG) Study on Low-Grade Primary Central Nervous System Lymphoma in Immunocompetent Patients.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3343-3343
Author(s):  
Agnieszka Korfel ◽  
Kristoph Jahnke ◽  
Brian P. O’Neill ◽  
Jean-Yves Blay ◽  
Lauren Abrey ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Performance Status ◽  
Eastern Cooperative Oncology Group ◽  
Tumor Resection ◽  
Central Nervous System Lymphoma ◽  
Collaborative Group ◽  
Low Grade ◽  
Term Outcome ◽  
Long Term Outcome

Abstract Low-grade primary central nervous system lymphoma is a very rare subtype of primary central nervous system lymphoma (PCNSL), for which almost no data is currently available. The purpose of this retrospective study was to characterize the clinical presentation, course and outcome of patients with low-grade PCNSL. Forty patients (18 male, 22 female) from 18 cancer centers in five countries were identified with a median age of 58 (range, 19–78) years and a median Eastern Cooperative Oncology Group (ECOG) performance status of 1 (range, 0–4). The mean time to diagnosis was 14.8 months (range, 0.25–84). Thirty-two patients (80%) had a B-cell and eight a T-cell lymphoma. Thirty-seven patients (92.5%) showed involvement of a cerebral hemisphere or deeper brain structures, while two evidenced only leptomeningeal involvement, and one patient had spinal cord disease. Treatment was performed in 39 patients: chemotherapy and radiotherapy in 15 (38%), radiotherapy alone in 12 (30%), chemotherapy alone in 10 (25%), and tumor resection alone in two. The median progression-free survival (PFS), disease-specific survival (DSS) and overall survival (OAS) were 61.5 (range, 0–204), 130 (range, 1–204), and 79 (range, 1–204) months, respectively. An age ≥60 years was associated with a shorter PFS (P = .009), DSS (P = .015) and OAS (P = .001) in multivariate analysis. Low-grade PCNSL differ from the high-grade subtype in pathological, clinical and radiological features. In this study, the long-term outcome was better as compared to the results obtained in PCNSL in general with age ≥60 years adversely affecting survival.

Treatment of Burkitt's Like Lymphoma: A Single Institution Case Series

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4962-4962
Author(s):  
Vishal Ranpura ◽  
Shilpan S. Shah
Keyword(s):  
Central Nervous System ◽  
Bone Marrow ◽  
Nervous System ◽  
Peripheral Blood ◽  
Chemotherapy Regimen ◽  
Conflicts Of Interest ◽  
Case Series ◽  
B Cell Lymphoma ◽  
Response To Treatment ◽  
Single Institution

Abstract Abstract 4962 Background: Burkits's like (BL) lymphoma is a new pathologic entity with features partially resembling to Burkitt's lymphoma and diffuse large B cell lymphoma. The incidence of BL lymphoma is infrequent and may present with lymph node enlargement, involvement of bone marrow and peripheral blood. The natural history of BL lymphoma and its treatment remains unclear. Here we present a single institution case series of five patients diagnosed with BL lymphoma and their treatment. Methods: We searched the pathology reports of all patients diagnosed with lymphoma over the last one year. No inclusion or exclusion criteria were used. Results: We identified a total of five patients with diagnosis of BL lymphoma. All patients presented with lymphadenopathy and with no involvement of bone marrow, central nervous system or peripheral blood. Table 1 summarizes characteristics of all patients, their chemotherapy regimen and subsequent response to treatment. Conclusion: BL lymphoma is a new pathologic entity with low incidence. Treatment with DA-EPOCH and R-HyperCVAD has very good response rate. The data is limited by single institution case series and limited follow up time. Further studies are recommended to evaluate optimal chemotherapy regimen. Table 1: Characteristics, presentation, treatment and response in patients with BL lymphoma No Age Sex LDH, Presentation Treatment Response Duration of response 1 68 F 386 Parotid and abdominal lymphadenopathy DA-R-EPOCH CR 16months 2^ 44 M 536 Neck, Axillary, media, abdomen pelvis and sacral mets R-HyperCVAD PR 3months 3* 66 F 310 Inguinal and pelvic lymphadenopathy R-HyperCVAD f/b Autologus BMT, CR 14months 4** 71 M 237 Parotid mass 3 cycles of DA-EPOCH f/b 3 cycles of R-CHOP CR 9months 5^ 52 M 186 Neck Lymphadenopathy R-HyperCVAD CR 3months DOX, DOXOL, and anthracenediones in soluble fractions of human myocardial strips after sequential DOX loading/clearance and anthracenedione treatment Notes: DA-REPOCH: Dose Adjusted Rituximab-Etoposide, Vincristine, Cyclophosphamide, Doxorubicine, Prednisone, R-HyperCVAD: Rituximab, hyperfractionated Cyclophosphamide, Vincristine, Doxorubicin, Dexamethasone, CR: complete remission, BMT: bone marrow transplantation, R-CHOP: rituximab, doxorubicin, cyclophosphamide, vincristine and prednisone. CNS: central nervous system * patient underwent bone marrow transplant because of relapse at current presentation. ** patient was changed from DA EPOCH to R-CHOP as patient was not able to tolerate EPOCH ^ Epatients are halfway through their treatment cycles and are actively getting treatment Disclosures: No relevant conflicts of interest to declare.

scholarly journals Autologous Stem Cell Transplantation with Thiotepa, Busulfan, and Cyclophosphamide Conditioning in Patients with Primary Central Nervous System Lymphoma: A Remarkable Outcome Form Single-Center Experience

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 3462-3462 ◽  
Author(s):  
Seyoung Seo ◽  
Jung Yong Hong ◽  
Dok Hyun Yoon ◽  
Jeong Hoon Kim ◽  
Young Hyun Cho ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Overall Survival ◽  
Nervous System ◽  
Prognostic Score ◽  
Central Nervous System Lymphoma ◽  
Salvage Chemotherapy ◽  
High Dose ◽  
Event Free Survival ◽  
Free Survival ◽  
Baseline Characteristics

Abstract Introduction High dose chemotherapy (HDC) followed by autologous stem cell transplantation (ASCT) has been adopted as an effective treatment in patients with relapsed or refractory primary central nervous system lymphoma (PCNSL) and also has been proposed as a consolidative treatment option for newly diagnosed PCNSL. HDC-ASCT may overcome chemoresistance mediated by blood-brain barrier by affording higher drug concentrations in the central nervous system. We investigated the feasibility of thiotepa, busulfan, and cyclophosphamide (TBC) conditioning followed by ASCT in patients with PCNSL. Method Between December 2012 and July 2015, a total of 27 patients with PCNSL underwent TBC conditioning followed by ASCT. Those with a complete or partial response after induction chemotherapy or salvage chemotherapy proceeded with TBC conditioning followed by ASCT. TBC conditioning consists of thiotepa 250 mg/m2 on days -9 to day -7, busulfan 3.2 mg/kg on days -6 to day -4 and cyclophosphamide 60 mg/kg on days -3 to day -2. The event free survival (EFS) was defined from the date of transplant to the date of relapse, progression or any cause of death, while overall survival (OS) was calculated from the date of transplant to death. Result Baseline characteristics were summarized in table 1. Twenty patients received TBC conditioning followed by ASCT as a consolidative therapy after high-dose methotrexate-based induction chemotherapy and the other 7 patients received TBC conditioning followed by ASCT after salvage chemotherapy due to relapsed or refractory disease. The median time to neutrophil recovery (absolute neutrophil count >500/uL) and platelet recovery (>20000 x103/uL) were 8 (range, 7-9) and 8 (range, 4-15) days, respectively. All 27 patients experienced febrile neutropenia and 33.3% of patients (9/27) and 7.4% of patients (2/27) had documented bacterial and viral infection, respectively. Commonly observed nonhematologic grade 3 or 4 toxicities were mucositis (63%), diarrhea (59.3%) and nausea (25.9%). The 100-day transplant-related mortality rate was 0%. With median follow-up duration of 27.8 months (range 6.7-42.6), median EFS and OS were not reached. The 2-year EFS and OS estimates were 76.8% (95% CI: 68.4-85.2) and 88.9% (95% CI: 82.9-94.9), respectively (Figure 1). Conclusion ASCT with TBC conditioning appears to be feasible in patients with PCNSL. Although survival outcomes are encouraging, longer follow-up is required. Further studies are warranted to investigate the role of ASCT with TBC conditioning in both clinical settings of consolidative treatment of newly diagnosed PCNSL and salvage treatment of relapsed or refractory PCNSL. Table 1 Baseline characteristics (n=27) *Conventional cytology; flow cytometry not performed $The cutoff for normal CSF protein concentration was 45 mg/dL in patients ¡Â 60 years old and 60 mg/dL in patients more than 60 years old. *MSK RPA, Memorial Sloan-Kettering prognostic score determined by recursive partitioning $Periventricular, basal ganglia, brainstem and cerebellar lesion Table 1. Baseline characteristics (n=27). / *Conventional cytology; flow cytometry not performed. / $The cutoff for normal CSF protein concentration was 45 mg/dL in patients ¡Â 60 years old. / and 60 mg/dL in patients more than 60 years old. / *MSK RPA, Memorial Sloan-Kettering prognostic score determined by recursive partitioning. / $Periventricular, basal ganglia, brainstem and cerebellar lesion Figure 1 Event-free survival and overall survival. Figure 1. Event-free survival and overall survival. Disclosures No relevant conflicts of interest to declare.

Outcomes of urothelial carcinoma patients with central nervous system metastases.

2020 ◽  
Vol 38 (6_suppl) ◽  
pp. 468-468
Author(s):  
Erik Andrews ◽  
Catherine Curran ◽  
Petros Grivas ◽  
Leonidas Nikolaos Diamantopoulos ◽  
Ravindran Kanesvaran ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Radiation Therapy ◽  
Urothelial Carcinoma ◽  
Systemic Therapy ◽  
Tumor Regression ◽  
Performance Status ◽  
Poor Performance ◽  
Poor Performance Status ◽  
Best Response

468 Background: Urothelial carcinoma (UC) metastatic to the central nervous system (CNS) is extremely rare. Given the lack of systematic data regarding outcomes in UC metastatic to the CNS, we conducted a retrospective multicenter study to more broadly characterize outcomes. Methods: Patients (pts) diagnosed with metastatic UC to the CNS were identified from collaborating institutions. Data were collected for demographics, clinical and pathological variables. Descriptive statistics were reported to examine tumor regression, time to treatment discontinuation or failure (TTF), and overall survival (OS). Results: 39 pts with UC metastatic to the CNS were evaluable from 6 institutions. The ECOG-PS ranged from 0-4 (median 2), the median age was 67 (range 39-82), and 8 (20.5%) were female. The histology consisted of pure urothelial, mixed predominant urothelial, and mixed predominant non-urothelial in 26 (66.7%), 8 (20.5%), and 5 (12.8%) pts, respectively. The sites of CNS metastases (mets) were the brain, meninges, and a combination of CNS sites in 37 (95%), 1 (2.5%) and 1 (2.5%) pts, respectively. 33 (84.6%) pts received radiation therapy (RT) to their mets and 6 (15.4%) did not. Of 16 pts who received systemic therapy (ST), cisplatin-based chemotherapy, non-cisplatin based chemotherapy, and PD-1/L1 inhibitors were administered in 6 (37.5%), 5 (31.25%), and 5 (31.25%) pts, respectively. The overall median TTF and OS were 90 and 154 days (d). The median OS for pts who had ST compared to those who did not was 193.5 (n=12) versus 71 (n=17) d. The median OS for pts who had RT compared to those who did not was 92 (n=25) versus 15.5 (n=4) d. The median OS for pts who received neither ST nor RT, compared to pts receiving both, was 12 (range 7-19) versus 232 (n=11) d. Best response to systemic therapy PR, CR, SD and PD were seen in 2 (12.5%), 1 (6.25%), 1 (6.25%) and 12 (75%) pts, respectively. For the 5 pts who received RT only, best response PR and PD were seen in 2 (40%) and 3 (60%) pts, respectively. Conclusions: Pts with UC metastatic to the CNS present with a poor performance status and have outcomes that are dismal and appear worse than those with metastases to other organs. A combination of systemic and radiation therapy might achieve improved outcomes in selected pts.

Abstract TP111: Primary Angiitis of Central Nervous System . Clinical Profile and Outcome of Forty Seven Patients

Stroke ◽  
2017 ◽  
Vol 48 (suppl_1) ◽  
Author(s):  
Pn Sylaja ◽  
Soumya Sundaram ◽  
Deepak Menon ◽  
Sapna Sreedharan ◽  
Jayadevan ER ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Treatment Group ◽  
Clinical Profile ◽  
Small Vessel ◽  
Neurological Deficits ◽  
Response To Treatment ◽  
Progressive Dementia ◽  
Number Of Patients ◽  
Primary Angiitis

Introduction: Primary angiitis of central nervous system (PACNS) is a rare and serious disorder of uncertain cause, with significant morbidity and mortality. Objective: To determine the clinical profile, response to treatment and outcome in patients with PACNS. Methodology: This is retrospective study of 47 patients from January 2000 to Dec 2015 with either angiographically or biopsy proven PACNS. Outcome at 6 months was considered poor, if modified rankin scale was &gt 2. Relapse was defined as occurrence of new neurological deficits after clinical improvement, progression of existing disability or radiological worsening. Results: The median age at diagnosis was 36 years (IQR 17) and 33 (70.2%) were males. Twenty-nine patients (61.7%) had angiography positive, 14 patients (38.3%) had biopsy confirmed and 4 patients (8.5%) had both angiography and biopsy positive PACNS .Stroke was the initial presentation in 33 patients (70.2%) followed by rapidly progressive dementia in 11 patients (23.4%) and myelopathy in 3 patients (6.4%). After a median follow-up of 2.8 years (range 0.5-15) , poor 6 month outcome was observed in 12 (25.5%), relapse in 24 (51.1%) and mortality in 3 (6.4%) patients. The median delay from onset of symptoms to start of immunosuppressive medication was 3.5 months (0.5- 63).More number of patients in the delayed treatment group had relapse compared to the early treatment group (59.1% vs 52.2%, p= 0.76). Among the 34 patients who received steroids as the initial treatment, 61.8% had relapse, whereas in patients on steroid with cyclophosphamide, only 38.5% had relapse (p=0.176). Of the 25 patients with abnormal CSF, 16 (64%) had relapse (p=0.209) and of the 18 patients with abnormal meningocortical biopsy, 15 patients (83.3%) had relapse. The NIHSS at admission &gt 5 (p=0.046), and presence of severe small vessel ischemic changes in MRI (p= 0.04) were predictors of relapse. Conclusion: PACNS is a rare disease with delayed diagnosis. Severity of neurological deficit at presentation and presence of severe small vessel ischemic changes in MRI were predictors of future relapse.

Changes in the neutrophil to lymphocyte ratio as predictors of outcome in pediatric patients with central nervous system tumors undergoing surgical resection

2021 ◽  
pp. 1-8
Author(s):  
Al Flores-Bustamante ◽  
Laura Hernández-Regino ◽  
Manuel-De-Jesús Castillejos-López ◽  
Daniel Martínez-Rodríguez ◽  
Arnoldo Aquino-Gálvez ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Overall Survival ◽  
Nervous System ◽  
Surgical Resection ◽  
Pediatric Patients ◽  
Poor Response ◽  
Response To Treatment ◽  
Central Nervous System Tumors ◽  
Nervous System Tumors ◽  
Lymphocyte Ratio

BACKGROUND: Changes in neutrophil to lymphocyte ratio (ΔNLR) have been used as a clinical tool for stratification and prognosis of patients with solid tumors, there is scarce evidence of their clinical relevance in patients with tumors of the central nervous system who have also undergone surgical resection. OBJECTIVE: Determine if (ΔNLR) are associated with poor response to treatment and worse prognosis in pediatric patients with central nervous system tumors (CNST) who underwent surgical resection. METHODS: We performed a retrospective cohort study; demographic, clinical, and hematological variables were evaluated, Kaplan-Meier survival curves and Cox proportional hazards regression model were performed to evaluate prognosis. RESULTS: The ΔNLR cutoff value obtained through the third interquartile range was 4.30; The probability of survival and complete response to treatment was different between patients with high ΔNLR when compared to patients with low ΔNLR (p= 0.013, p=≪ 0.001, respectively). A high ΔNLR behaved as an independent predictor of worse Overall Survival (HR 2,297; 95% CI: 1,075–4.908, p= 0.032). CONCLUSION: An elevated ΔNLR was a predictor of poor response to treatment and a prognostic factor for worse Overall Survival in pediatric patients with CNST undergoing surgical resection.

Successful Intravenous Immunoglobulin Treatment of Atypical Lichen Myxedematosus Associated with Hypothyroidism and Central Nervous System Involvement: Case Report and Discussion of the Literature

2013 ◽  
Vol 17 (1) ◽  
pp. 69-73 ◽  
Author(s):  
Magnus Macnab ◽  
Patrick Kenny
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Intravenous Immunoglobulin ◽  
Thyroid Dysfunction ◽  
Central Nervous System Involvement ◽  
Response To Treatment ◽  
Ivig Treatment ◽  
Systemic Complications ◽  
Disease Case ◽  
Lichen Myxedematosus

Background: Lichen myxedematosus (LM) is a rare idiopathic disorder characterized by papules, plaques, and/or nodules in the skin secondary to mucin deposition and variable dermal fibrosis in the absence of thyroid disease. Case reports are the predominant literature on LM due to its rarity, and the data are limited regarding the disease. No standard treatment regimen exists, and the response to treatment varies. Objective: This report adds to the limited literature on atypical LM associated with thyroid dysfunction and contributes to the growing body of evidence supporting intravenous immunoglobulin (IVIg) as effective therapy for extensive cases. Methods and Results: We report an unusual case of atypical LM associated with hypothyroidism, central nervous system (CNS) disturbances, and atrial fibrillation in a 64-year-old male. The patient experienced remarkable improvement within 3 months of beginning IVIg treatment; however, he required repeat therapy due to the recurrence of CNS symptoms 3 months after treatment. Conclusion: We agree with previous authors that thyroid dysfunction alone should not preclude a diagnosis of LM. Additionally, we contribute to the increasing evidence of IVIg being an effective treatment, particularly in the setting of systemic complications or acute worsening of LM.

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