scholarly journals LGG-15. COMPREHENSIVE ANALYSIS OF MYB/MYB1-ALTERED GLIOMAS: A MULTI-INSTITUTIONAL EXPERIENCE OF 33 GLIOMAS

2021 ◽  
Vol 23 (Supplement_1) ◽  
pp. i34-i35
Author(s):  
Daniel Moreira ◽  
Susan Spiller ◽  
Thomas Bouldin ◽  
Alan Davidson ◽  
Nasjla Saba-Silva ◽  
...  
Keyword(s):  
Genetic Alterations ◽  
Myelogenous Leukemia ◽  
Molecular Characteristics ◽  
Low Grade ◽  
Cerebrospinal Fluid Diversion ◽  
Institutional Experience ◽  
Diffuse Gliomas ◽  
Acute Myelogenous ◽  
Single Disease Entity

Abstract Background Pediatric diffuse gliomas harbor recurrent genetic alterations, including those in MYB and MYBL1. Regardless of histopathologic classification, low-grade diffuse gliomas with MYB/MYBL1 alterations represent a single disease entity. Additional insight is needed to define optimal therapeutic strategies for these tumors. Methods We retrospectively reviewed gliomas with MYB or MYB1L alterations treated or referred for pathologic review at St. Jude Children’s Research Hospital (St. Jude). Tumor specimens were centrally reviewed. Molecular characterization and clinical data were collated from St. Jude and referring institutions. Results Thirty-three patients were identified. Two tumors had MYBL1 alterations, while 31 had MYB alterations. MYB-QKI fusion was the most common alteration. Eighteen (55%) were male. The median age at diagnosis was 5 years (range, 0–40 years). Most tumors were in the cerebral cortex (22/33), and the most common presentation was seizures (16/33). Three patients (9%) presented with hydrocephalus and required cerebrospinal fluid diversion. Two patients (6%) presented with metastatic disease. Gross-total resection was achieved in 15 patients (45%). Of the 7 patients receiving cytotoxic chemotherapy, no substantial response was observed. Of the 6 patients who received RT, one had disease progression. The median follow-up was 5.9 years. The 5-year event-free survival was 88.1%, while the 5-year overall survival was 96.3%. Two patients died, one of unclear cause and one of treatment-related acute myelogenous leukemia. Using log-rank tests, no difference in outcomes was observed based on molecular characteristics, degree of resection, metastatic status, or treatment modality. Conclusions Although tumors with MYB and MYBL1 alterations present with varying molecular and clinical features, they represent a group of tumors with favorable outcomes. Further characterization is required to identify the subgroup of tumors with a higher propensity for progression.

MOPP/ABV hybrid chemotherapy for advanced Hodgkin's disease significantly improves failure-free and overall survival: the 8-year results of the intergroup trial.

1998 ◽  
Vol 16 (1) ◽  
pp. 19-26 ◽  
Author(s):  
J H Glick ◽  
M L Young ◽  
D Harrington ◽  
R L Schilsky ◽  
T Beck ◽  
...  
Keyword(s):  
Overall Survival ◽  
Relative Risk ◽  
Hodgkin's Disease ◽  
Hodgkin’S Disease ◽  
Myelogenous Leukemia ◽  
Life Threatening ◽  
Acute Myelogenous ◽  
First Relapse ◽  
Sequential Regimen

PURPOSE To compare the efficacy of sequential mechlorethamine, vincristine, procarbazine, and prednisone (MOPP) followed by doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) versus the MOPP/ABV hybrid regimen in advanced-stage Hodgkin's disease. PATIENTS AND METHODS A total of 737 patients with previously untreated stages III2A, IIIB, IVA, or IVB Hodgkin's disease and patients in first relapse after radiotherapy were prospectively randomized to sequential MOPP-ABV or MOPP/ABV hybrid. Of 691 eligible patients, 344 received the sequential regimen and 347 received the hybrid. RESULTS The overall response rate was 95%, with complete responses (CRs) in 79%: 83% on the MOPP/ABV hybrid and 75% on the sequential MOPP-ABVD arm (P = .02). With a median follow-up time of 7.3 years, the 8-year failure-free survival (FFS) rates were 64% for MOPP/ABV hybrid and 54% far sequential MOPP-ABVD (P = .01; 0.69 relative risk of failure, comparing MOPP/ABV hybrid v MOPP-ABVD). The 8-year overall survival rate was significantly better for the MOPP/ABV hybrid (79%) as compared with sequential MOPP-ABVD (71%) (P = .02; relative risk, 0.65). MOPP/ABV hybrid had significantly more life-threatening or fatal neutropenia and pulmonary toxicity than the sequential MOPP-ABVD arm, which was associated with significantly greater thrombocytopenia. Nine cases of acute myelogenous leukemia or myelodysplasia were reported on the sequential regimen as compared with only one on the hybrid (P = .01). CONCLUSION MOPP/ABV hybrid chemotherapy was significantly more effective than sequential MOPP-ABVD. FFS and overall survival were significantly improved on the hybrid arm, which was also associated with a lower incidence of acute leukemia or myelodysplasia.

scholarly journals Chemotherapy for acute myelogenous leukemia in children and adults: VAPA update

Blood ◽  
1983 ◽  
Vol 62 (2) ◽  
pp. 315-319 ◽  
Author(s):  
HJ Weinstein ◽  
RJ Mayer ◽  
DS Rosenthal ◽  
FS Coral ◽  
BM Camitta ◽  
...  
Keyword(s):  
Complete Remission ◽  
Maintenance Therapy ◽  
Induction Chemotherapy ◽  
Acute Myelogenous Leukemia ◽  
Myelogenous Leukemia ◽  
Kaplan Meier ◽  
High Incidence ◽  
Acute Myelogenous ◽  
Leukemia In Children

Abstract We designed a protocol (VAPA) that featured 14 mo of intensive postremission induction chemotherapy in an effort to improve remission durations for patients with acute myelogenous leukemia (AML). One hundred and seven patients under 50 yr of age were entered into this study. The rate of complete remission is 70%. A Kaplan-Meier analysis of patients entering remission predicts that 56% +/- 7% (+/-SE) of patients less than 18 yr and 45% +/- 9% of patients aged 18–50 yr will remain in remission at 3 yr (median follow-up is 43 mo). Patients with the monocytic subtype had a statistically significant shorter duration of remission (2-sided p less than 0.05). There was a high incidence of primary CNS relapse in children. Thirty-one of 41 patients who completed the regimen remain in remission without maintenance therapy. We conclude that the VAPA protocol continues to offer a promising approach to treatment of AML.

Therapy-Related Myelodysplasia and Secondary Acute Myelogenous Leukemia After High-Dose Therapy With Autologous Hematopoietic Progenitor-Cell Support for Lymphoid Malignancies

2000 ◽  
Vol 18 (5) ◽  
pp. 947-947 ◽  
Author(s):  
Ivana N. M. Micallef ◽  
Debra M. Lillington ◽  
John Apostolidis ◽  
John A. L. Amess ◽  
Michael Neat ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Progenitor Cell ◽  
Myelogenous Leukemia ◽  
Hematopoietic Progenitor Cell ◽  
High Dose ◽  
High Dose Therapy ◽  
Hematopoietic Progenitor ◽  
Acute Myelogenous ◽  
Cell Support

PURPOSE: To evaluate the incidence of and risk factors for therapy-related myelodysplasia (tMDS) and secondary acute myelogenous leukemia (sAML), after high-dose therapy (HDT) with autologous bone marrow or peripheral-blood progenitor-cell support, in patients with non-Hodgkin’s lymphoma (NHL). PATIENTS AND METHODS: Between January 1985 and November 1996, 230 patients underwent HDT comprising cyclophosphamide therapy and total-body irradiation, with autologous hematopoietic progenitor-cell support, as consolidation of remission. With a median follow-up of 6 years, 27 (12%) developed tMDS or sAML. RESULTS: Median time to development of tMDS or sAML was 4.4 years (range, 11 months to 8.8 years) after HDT. Karyotyping (performed in 24 cases) at diagnosis of tMDS or sAML revealed complex karyotypes in 18 patients. Seventeen patients had monosomy 5/5q−, 15 had −7/7q−, seven had −18/18q−, seven had −13/13q−, and four had −20/20q−. Twenty-one patients died from complications of tMDS or sAML or treatment for tMDS or sAML, at a median of 10 months (range, 0 to 26 months). Sixteen died without evidence of recurrent lymphoma. Six patients were alive at a median follow-up of 6 months (range, 2 to 22 months) after diagnosis of tMDS or sAML. On multivariate analysis, prior fludarabine therapy (P = .009) and older age (P = .02) were associated with the development of tMDS or sAML. Increased interval from diagnosis to HDT and bone marrow involvement at diagnosis were of borderline significance (P = .05 and .07, respectively). CONCLUSION: tMDS and sAML are serious complications of HDT for NHL and are associated with very poor prognosis. Alternative strategies for reducing their incidence and for treatment are needed.

scholarly journals Implications of Potential Cure in Acute Myelogenous Leukemia: Development of Subsequent Cancer and Return to Work

Blood ◽  
1997 ◽  
Vol 90 (12) ◽  
pp. 4719-4724 ◽  
Author(s):  
Marcos de Lima ◽  
Sara S. Strom ◽  
Michael Keating ◽  
Hagop Kantarjian ◽  
Sherry Pierce ◽  
...  
Keyword(s):  
Return To Work ◽  
Marrow Transplant ◽  
Invasive Cancer ◽  
Myelogenous Leukemia ◽  
Full Time ◽  
Number Of Patients ◽  
Increased Risk ◽  
Acute Myelogenous ◽  
Subsequent Cancer

Abstract Chemotherapy produces extended remissions, and potential cures, in a small minority of patients with acute myeloid leukemia (AML). We explored whether potentially cured patients were at increased risk of subsequent invasive cancer and were able to return to work. Potentially cured patients were defined as those in first or second complete remission (CR) for at least 3 years based on hazard rates for recurrence or death in CR, which declined sharply after this time. Patients who received allogeneic marrow transplant were excluded. We used questionnaires, phone contact, and chart review to obtain information about subsequent cancer and work status. The number of patients who developed invasive cancer was compared with the number expected based on age, gender, and years of follow-up using the Connecticut Tumor Registry. A total of 215 patients met our criteria for potential cure: 203 in first CR and 12 in second CR (of 1,663 treated between 1965 and December, 1992). At a median of 9.2 years from first or second CR, 163 (76%) remain alive in CR. Fifteen patients developed 18 invasive cancers (expected number of patients, 8.8; observed/expected, 1.70; 95% CI, 0.96 to 2.84; P = .06). Patients initially treated between 1973 to 1979, patients above the potentially cured cohort's median age of 40 years, and those who presented with abnormalities of chromosomes 5 and/or 7 were more likely to develop subsequent cancer, whereas the observed/expected ratio for younger patients was 1.05 (95% CI, 0.13 to 3.80; P = .56). Seventy-four percent of the patients who were working full-time and who were under age 50 at time of treatment for AML have been working full-time in the last 6 months. Only 17 of 56 patients who are currently not working cited physical limitation as the reason. Patients with potentially cured AML are likely to be able to return to work, and at least if younger do not, on average, have an increased risk of invasive cancer.

Allogeneic stem cell transplantation for patients over age 65 with acute myelogenous leukemia and myelodysplastic syndrome.

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. 6529-6529
Author(s):  
Henry Jacob Conter ◽  
Gabriela Rondon ◽  
Nhu-Nhu Nguyen ◽  
Julianne Chen ◽  
Elizabeth J. Shpall ◽  
...  
Keyword(s):  
Chronic Gvhd ◽  
Age Groups ◽  
Myelogenous Leukemia ◽  
Cancer Center ◽  
Acute Myelogenous ◽  
Grade Ii ◽  
Md Anderson ◽  
Active Disease

6529 Background: ASCT represents a potentially curative approach for AML and MDS, diseases that primarily affects patients in 7th and 8th decade of life. Here, we report outcomes of patients older than 64 treated at the MD Anderson Cancer Center from 1996 until December 2011. Methods: 182 patients older than 64 received an ASCT for AML (n=143) or MDS (n=39). Outcomes of interest were transplant-related mortality (TRM), incidence of acute and chronic graft-versus-host disease (GVHD), incidence of relapse, and overall survival (OS) - which were estimated from the date of transplant. Results: The median age of patients was 67 (range 65-79). Most patients were transplanted with active disease (table). Median follow-up for alive patients was 12.6 months (n=63; range 0-118). The cumulative incidence of 100-day, 1 year, and 3 year TRM was 14%, 18%, and 21%, respectively. 26% of patients developed grade II-IV acute GVHD and 35% suffered from chronic GVHD. The actuarial incidence of relapse was 46% at 1 year and 53% at 3 and 5 years. Actuarial OS was estimated to be 45% at 1 year, 28% at 3 years, and 21% at 5 years. 3 year OS for patients transplanted in CR and with active disease was 40% versus 23% (p=0.02). OS of patients age 65-69 or >69 was 30% vs. 20% (p=0.06) at 3 years for all patients; compared to 38% versus 27% (p=0.23) for patients in those age groups transplanted in CR. Conclusions: Although a significant minority of patients older than 64 years may achieve long-term disease control, new approaches are needed to reduce TRM and relapse in this cohort of patients. [Table: see text]

scholarly journals T-Cell–Depleted Allogeneic Bone Marrow Transplantation as Postremission Therapy for Acute Myelogenous Leukemia: Freedom From Relapse in the Absence of Graft-Versus-Host Disease

Blood ◽  
1998 ◽  
Vol 91 (3) ◽  
pp. 1083-1090 ◽  
Author(s):  
Esperanza B. Papadopoulos ◽  
Matthew H. Carabasi ◽  
Hugo Castro-Malaspina ◽  
Barrett H. Childs ◽  
Stephen Mackinnon ◽  
...  
Keyword(s):  
Bone Marrow ◽  
T Cell ◽  
Complete Remission ◽  
Myelogenous Leukemia ◽  
Allogeneic Bone ◽  
Graft Versus Host ◽  
Immune Mediated ◽  
Acute Myelogenous ◽  
Postremission Therapy

Thirty-one consecutive patients with acute myelogenous leukemia (AML) in first complete remission and 8 with AML in second complete remission received T cell–depleted allogeneic bone marrow transplants from HLA-identical sibling donors. Patients received myeloablative cytoreduction consisting of hyperfractionated total body irradiation, thiotepa, and cyclophosphamide. Those patients at risk for immune-mediated graft rejection received additional immune suppression with antithymocyte globulin and methylprednisolone in the early peritransplant period. Patients with AML who underwent allogeneic T-cell–depleted bone marrow transplantations (BMT) in first or second remission have achieved respective disease-free survival (DFS) probabilities of 77% (median follow-up at approximately 56 months) and 50% (median follow-up at approximately 48 months). Ten of 31 patients transplanted in first remission were ≥ 40 years old and have attained a DFS at 4 years of 70%. For patients with AML transplanted in first or second remission, the respective cause-specific probabilities of relapse were 3.2% or 12.5%, and those of nonleukemic mortality were 19.4% or 37.5%. There were no cases of immune-mediated graft rejection and no cases of grade II to IV acute graft-versus-host disease (GVHD). All survivors enjoy Karnofsky performance scores (KPS) of 100%, except 2 patients with KPS of 80% to 90%. T-cell–depleted allogeneic BMT can provide durable DFS together with an excellent performance status in the majority of patients with de novo AML. In addition, GVHD is not an obligatory correlate of the graft-versus-leukemia benefit or freedom from relapse afforded by allogeneic BMT administered as postremission therapy for AML. This study provides a basis for prospective comparison with other postremission therapies considered standard in the management of patients with this disease.

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