This Article Full Text Full Text (PDF) Purchase Article View Shopping Cart Alert me when this article is cited Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Save to my personal folders Download to citation manager Rights & Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Seidemann, K. Articles by Reiter, A. Search for Related Content PubMed PubMed Citation Articles by Seidemann, K. Articles by Reiter, A. Social Bookmarking                            What's this?

Primary Mediastinal Large B-Cell Lymphoma With Sclerosis in Pediatric and Adolescent Patients: Treatment and Results From Three Therapeutic Studies of the Berlin-Frankfurt-Münster Group

K. Seidemann, M. Tiemann, I. Lauterbach, G. Mann, I. Simonitsch, K. Stankewitz, M. Schrappe, M. Zimmermann, C. Niemeyer, R. Parwaresch, H. Riehm, A. Reiter

From the Department of Pediatric Hematology and Oncology, Medizinische Hochschule Hannover; Lymphnode Registry founded by the Society of German Pathologists, Institute of Hematopathology, Christian-Albrechts-Universität, Kiel; Department of Pediatric Hematology and Oncology, Technische Universität, Dresden; Department of Pediatric Hematology and Oncology, Justus-Liebig-University, Giessen; Department of Pediatric Hematology and Oncology, Universitätskinderklinik Freiburg, Germany; St. Anna-Kinderspital; and Institute of Pathology, University of Vienna, Vienna, Austria.

Address reprint requests to Alfred Reiter, MD, NHL-BFM Study Center, Department of Pediatric Hematology and Oncology, Justus-Liebig-University, Feulgenstr. 12, D-35385 Giessen, Germany; email: alfred.reiter{at}paediat.med.uni-giessen.de.

Purpose: Primary mediastinal large B-cell lymphoma with sclerosis (PMLBL) is a rare entity of non-Hodgkin’s lymphoma (NHL) arising from thymic mature B cells. Optimal treatment strategies remain to be established, especially in pediatric patients.

Patients and Methods: This study analyzes clinical characteristics and treatment outcome of 30 pediatric patients with PMLBL, diagnosed in multicenter therapy NHL–Berlin-Frankfurt-Münster Group (BFM) trials. Treatment was stratified by stage and serum lactate dehydrogenase (LDH) and consisted of four to six 5-day courses of chemotherapy using steroids, oxazaphosphorine alkylating agents, methotrexate, cytarabine, etoposide, and doxorubicin. Radiation was not part of the protocol.

Results: From April 1986 to August 1999, 1,650 patients with newly diagnosed NHL were enrolled in the NHL-BFM trials; 30 patients (1.8%) had PMLBL. Median age was 14.3 years (range, 1.4 to 16.7 years); 15 patients were male and 15 patients were female. With a median observation time of 5 years (range, 1 to 12 years), probability of event-free survival (pEFS) at 5 years was 0.70 (SE, 0.08). Two patients erroneously diagnosed as T-cell NHL received non–B-cell therapy and died from progress of disease. Events in 28 patients receiving B-cell therapy included early progress during therapy (n = 1) and relapse (n = 6). Residual mediastinal masses were present in 23 patients after two courses of therapy and in 15 patients after the end of therapy. LDH 500 U/L was associated with increased risk of failure in multivariate analysis.

Conclusion: PMLBL mainly is found in adolescents. Dose-intense chemotherapy including high-dose methotrexate yields a pEFS at 5 years of 0.70 (SE, 0.08). LDH is of prognostic value in pediatric patients with PMLBL.

Supported by the Deutsche Krebshilfe, Bonn, Germany, grant no. M 109/91/Re1; T 12/96Re I.

CiteULike    Complore    Connotea    Del.icio.us    Digg    Facebook    Reddit    Technorati    Twitter    What's this?

This article has been cited by other articles:

				A. Reiter Diagnosis and Treatment of Childhood Non-Hodgkin Lymphoma Hematology, January 1, 2007; 2007(1): 285 - 296. [Abstract] [Full Text] [PDF]

				W. Woessmann, K. Seidemann, G. Mann, M. Zimmermann, B. Burkhardt, I. Oschlies, W.-D. Ludwig, T. Klingebiel, N. Graf, B. Gruhn, et al. The impact of the methotrexate administration schedule and dose in the treatment of children and adolescents with B-cell neoplasms: a report of the BFM Group Study NHL-BFM95 Blood, February 1, 2005; 105(3): 948 - 958. [Abstract] [Full Text] [PDF]