scholarly journals Use of minimal disseminated disease and immunity to NPM-ALK antigen to stratify ALK-positive ALCL patients with different prognosis

Leukemia ◽  
2012 ◽  
Vol 27 (2) ◽  
pp. 416-422 ◽  
Author(s):  
L Mussolin ◽  
C Damm-Welk ◽  
M Pillon ◽  
M Zimmermann ◽  
G Franceschetto ◽  
...  
Keyword(s):  
Minimal Disseminated Disease ◽  
Alk Positive ◽  
Disseminated Disease

Minimal Disseminated Disease Is An Independent Poor Prognosis Marker Among High Risk Burkitt's Lymphoma Patients .

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 2944-2944
Author(s):  
Lara Mussolin ◽  
Marta Pillon ◽  
Gloria Tridello ◽  
Maria Paola Boaro ◽  
Emanuele S d'Amore ◽  
...  
Keyword(s):  
High Risk ◽  
Poor Prognosis ◽  
Conflicts Of Interest ◽  
Burkitt’S Lymphoma ◽  
Chromosome 8 ◽  
Burkitt's Lymphoma ◽  
Long Distance ◽  
Igh Locus ◽  
Minimal Disseminated Disease ◽  
Disseminated Disease

Abstract Abstract 2944 Poster Board II-920 Introduction: The chromosomal translocation t(8;14)(q24;q32) represents a specific tumor marker in Burkitt's lymphoma (BL). This chromosomal aberration involves the MYC oncogene on chromosome 8 and the immunoglobulin heavy-chain (IgH) locus on chromosome 14. We have previously demonstrated that this genetic abnormality can be used as a marker of Minimal Disseminated Disease (MDD) in BL (Mussolin et al, JCO, 2007). The aim of the study was to assess of the prevalence of MDD in BL at diagnosis in children enrolled in the AIEOP LNH-97 clinical protocol and the evaluation of its impact on prognosis. Patients and Methods: We established a simplified long-distance PCR (LD-PCR) assay which can amplify up to 15-20 Kb DNA sequence making it possible to detect the t(8;14) at the genomic level with the sensitivity of 10-4. The assay was based on 4 separate PCR reactions in which one primer complementary to the first exon of the MYC gene is used with one of four primers for the IgH locus (1 for the JH region and 1 for each of the 3 constant regions). Results: LD-PCR was applied to prospectively study 124 BL biopsies and detected a specific PCR product in 88 of them (71%). Of the 88 positive BL patients we studied both the tumor and the bone marrow (BM) at diagnosis in 76: BM was positive by LD-PCR in 25 patients (33%), whereas only 10 (13%) were positive at the standard morphological and/or immunophenotypical analyses. Most of the MDD positive patients (88%) belonged to the R4 Risk Group according to BFM definition (stage III or stage IV according to St. Jude staging classification and LDH≥1000 U/l). The 3-year progression-free survival (PFS) was 68% (SE 10%) in MDD positive R4 patients compared with 96% (SE 4%) in MDD negative R4 patients (p= 0.02), whereas there was no difference in PFS between children with morphological involvement of BM at diagnosis versus those who had negative BM (PFS=62.5% (SE 17%) vs. PFS= 87% (SE 6%), respectively, p= 0.09). By multivariate analysis (including MDD, gender, LDH, CNS involvement) MDD was predictive of higher risk of failure (Hazard Ratio: 8.4 , p= 0.04). Conclusions: We demonstrated that MDD identifies a poor prognosis subgroup among high risk Burkitt's lymphoma patients. We suggest that a more effective risk-adapted therapy, possibly including anti-CD20 monoclonal antibody, should be considered in these patients. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Case Report: Circulating Tumor Cells as a Response Biomarker in ALK-Positive Metastatic Inflammatory Myofibroblastic Tumor

2021 ◽  
Vol 9 ◽  
Author(s):  
Paolo Bonvini ◽  
Elisabetta Rossi ◽  
Angelica Zin ◽  
Mariangela Manicone ◽  
Riccardo Vidotto ◽  
...  
Keyword(s):  
Case Report ◽  
Tumor Cells ◽  
Circulating Tumor Cells ◽  
Predictive Biomarkers ◽  
Response To Treatment ◽  
Lung Lesions ◽  
Response Biomarker ◽  
Alk Positive ◽  
Bilateral Lung ◽  
Disseminated Disease

Inflammatory myofibroblastic tumors (IMTs) are locally aggressive malignancies occurring at various sites. Surgery is the mainstay of treatment and prognosis is generally good. For children with unresectable or metastatic tumors, however, outcome is particularly severe, limited also by the lack of predictive biomarkers of therapy efficacy and disease progression. Blood represents a minimally invasive source of cancer biomarkers for real-time assessment of tumor growth, particularly when it involves the analysis of circulating tumor cells (CTC). As CTCs potentially represent disseminated disease, their detection in the blood correlates with the presence of metastatic lesions and may reflect tumor response to treatment. Herein, we present a case report of a 19-year-old boy with an ALK-positive IMT of the bladder, proximal osteolytic and multiple bilateral lung lesions, who received ALK inhibitor entrectinib postoperatively and underwent longitudinal CTC analysis during treatment. Antitumor activity of entrectinib was demonstrated and was accompanied by regression of lung lesions, elimination of CTCs from the blood and no development of relapses afterwards. Therapy continued without any clinical sign of progression and 24 months since the initiation of treatment the patient remains symptom-free and disease-free.

Brentuximab Vedotin in Combination with Chemotherapy for Pediatric Patients with ALK+ALCL: Results of COG Trial ANHL12P1

Blood ◽  
2021 ◽  
Author(s):  
Eric J. Lowe ◽  
Anne F Reilly ◽  
Megan S Lim ◽  
Thomas G Gross ◽  
Lauren Saguilig ◽  
...  
Keyword(s):  
Pediatric Patients ◽  
Brentuximab Vedotin ◽  
Newly Diagnosed ◽  
Free Survival ◽  
Significant Toxicity ◽  
Excellent Activity ◽  
Minimal Disseminated Disease ◽  
Grade 3 ◽  
Disseminated Disease ◽  
Group Trial

Approximately 30% of pediatric patients with ALCL relapse. While brentuximab vendotin has demonstrated excellent activity in ALCL, it has not been used for newly diagnosed patients. Children's Oncology Group trial ANHL12P1 determined the toxicity and efficacy of brentuximab vedotin with chemotherapy in children with newly diagnosed, non-localized, ALK+/CD30+ ALCL. From 2013 to 2017, 68 children with ALK+ ALCL were enrolled and received brentuximab vedotin (Arm BV). All patients received five-day prophase followed by six cycles of chemotherapy at 21-day intervals. Brentuximab vedotin was given on day 1 of each of the six cycles. Of the 67 eligible patients for toxicity evaluation, 66 completed all six cycles of chemotherapy resulting in 399 cycles evaluable. There were no toxic deaths, no cases of progressive multifocal leukoencephalopathy syndrome, and no cases of grade 3 or 4 neuropathy. The two-year EFS is 79.1% (95% CI, 67.2% to 87.1%). The two-year OS is 97.0% (95% CI, 88.1% to 99.2%). Fourteen patients relapsed and were the only events contributing to EFS. 11 of 14 (79%) relapses occurred within ten months of initial diagnosis, with only one patient (1.5%) having relapsed during therapy. Quantitative RT-PCR for NPM-ALK at baseline (minimal disseminated disease) demonstrated prognostic value and impacted 2-year EFS (P=0.0004). Overall, the addition of brentuximab vedotin to standard chemotherapy does not add significant toxicity, nor does it alter the desired interval between cycles. The addition of brentuximab vedotin prevented relapses during therapy and the overall and event-free survival estimates compare favorably with results obtained using conventional chemotherapy.

scholarly journals Minimal Disseminated Disease in Nonmetastatic Retinoblastoma With High-Risk Pathologic Features and Association With Disease-Free Survival

2016 ◽  
Vol 134 (12) ◽  
pp. 1374 ◽  
Author(s):  
Viviana E. Laurent ◽  
Ana Vanesa Torbidoni ◽  
Claudia Sampor ◽  
Daniela Ottaviani ◽  
Valeria Vazquez ◽  
...  
Keyword(s):  
High Risk ◽  
Disease Free Survival ◽  
Free Survival ◽  
Pathologic Features ◽  
Minimal Disseminated Disease ◽  
Disseminated Disease ◽  
Disease Free

Minimal disseminated disease evaluation and outcome in trilateral retinoblastoma

2018 ◽  
Vol 102 (11) ◽  
pp. 1597-1601 ◽  
Author(s):  
Ana V Torbidoni ◽  
Claudia Sampor ◽  
Viviana E Laurent ◽  
Rosario Aschero ◽  
Saipriya Iyer ◽  
...  
Keyword(s):  
Csf Dissemination ◽  
Stem Cell Rescue ◽  
Systemic Dissemination ◽  
Trilateral Retinoblastoma ◽  
Minimal Disseminated Disease ◽  
Potential Impact ◽  
Disseminated Disease ◽  
Management Challenge ◽  
Disease Free

Trilateral retinoblastoma (TRb) presents a management challenge, since intracranial tumours are seldom times resectable and quickly disseminate. However, there are no risk factors to predict the final outcome in each patient.ObjectiveTo evaluate minimal disseminated disease (MDD) in the bone marrow (BM) and the cerebrospinal fluid (CSF) at diagnosis and during follow-up and reviewing its potential impact in the outcome of patients with TRb.Methods and analysisWe evaluated MDD in five patients with TRb, detecting the mRNA of CRX and/or GD2, in samples from BM and CSF, obtained at diagnosis, follow-up and relapse.ResultsTreatment involved intensive systemic chemotherapy in four patients, one did not receive this treatment and died of progression of the disease. Two patients underwent stem cell rescue. Three patients had leptomeningeal relapse and died. One patient remains disease-free for 84 months. RB1 mutations were identified in the five patients, all of them were null mutations. At diagnosis, one patient had tumour cells in the CSF, and none had the BM involved. Only one case of four presented MDD during follow-up in the CSF, without concomitant detection in the BM. On leptomeningeal relapse, no case had MDD in the BM. In all these cases, cells in the CSF were positive for GD2 and/or CRX.ConclusionCSF dissemination always concluded in the death of the patient, without concomitant systemic dissemination denoting the importance of increasing treatment directed to the CSF compartment. The MDD presence could indicate a forthcoming relapse.

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