Pediatric follicular lymphoma – a clinico-pathological study of a population-based series of patients treated within the Non-Hodgkin’s Lymphoma - Berlin-Frankfurt-Münster (NHL-BFM) multicenter trials

• Published in: Haematologica (Roma) Vol. 95; no. 2; pp. 253 - 259
• Main Authors: OSCHLIES, Ilske, SALAVERRIA, Itziar, SIEBERT, Reiner, KLAPPER, Wolfram, MAHN, Friederike, MEINHARDT, Andrea, ZIMMERMANN, Martin, WOESSMANN, Wilhelm, BURKHARDT, Birgit, GESK, Stefan, KRAMS, Matthias, REITER, Alfred
• Format: Journal Article
• Language: English
• Published: Pavia Ferrata Storti Foundation 01.02.2010
• Subjects: Adolescent; Biological and medical sciences; Child; Child, Preschool; Chromosome aberrations; Cohort Studies; Cytogenetic Analysis; Female; Gene Expression; Hematologic and hematopoietic diseases; Humans; Immunohistochemistry; Infant; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymphoma, Follicular - genetics; Lymphoma, Follicular - immunology; Lymphoma, Follicular - pathology; Lymphoma, Follicular - therapy; Lymphoma, Large B-Cell, Diffuse - diagnosis; Male; Medical genetics; Medical sciences; Original; Proto-Oncogene Proteins c-bcl-2 - genetics; Treatment Outcome; Europe; Berlin; Germany; Chromosomal aberration; Human; Pediatrics; Hematology; Multicenter study; B cell neoplasm; t(14;18); Malignant hemopathy; Lymphoid neoplasm; Non Hodgkin lymphoma; pediatric follicular lymphoma; Abnormal chromosome E18; Treatment; Lymphoproliferative syndrome; pediatric diffuse large B-cell lymphoma; Abnormal chromosome D14; Follicular lymphoma; Abnormal chromosome; Diffuse large B cell lymphoma; childhood lymphoma; Child; Cancer; Chromosome translocation
• ISSN: 0390-6078; 1592-8721
• DOI: 10.3324/haematol.2009.013177

Background Pediatric follicular lymphoma has recently been recognized as a novel variant of follicular lymphoma in the World Health Organization classification of lymphomas. Given the rarity of the disease, histopathological and genetic data on this type of lymphoma are still scarce. We analyzed 25 cases of pediatric follicular lymphoma (patients aged <or=18 years) by morphology, immunohistochemistry and interphase fluorescence in situ hybridization. All patients analyzed were treated within Non-Hodgkin's Lymphoma--Berlin-Frankfurt-Münster (NHL-BFM) multicenter trials, and the cohort was representative of the German population. The genetic hallmark of adult follicular lymphoma, t(14;18)(q32;q21), was not detectable in any of the pediatric cases, although BCL2 protein was expressed in 55% of the latter cases. No correlation was found between BCL2 protein expression and outcome. Chromosomal breaks in the immunoglobulin heavy chain gene (IGH) and the BCL6 locus were detected in 5 of 17 and 1 of 18 cases, respectively. Patients with pediatric follicular lymphoma had long event-free survival and, in contrast to adult follicular lymphoma, the clinical course was not dominated by relapses. A simultaneous diffuse large B-cell lymphoma was frequently detected at initial diagnosis in children but did not indicate an aggressive clinical course. Conclusions Our data suggest that pediatric follicular lymphoma is a disease that differs from its adult counterpart both genetically and clinically.