Rare Non-Hodgkin Lymphoma in Childhood; A Single Center Experience
10.15264/cpho.2016.23.2.116
- Author:
Hyun jin KIM
1
;
Darae LEE
;
Kyung Nam KOH
;
Sung Han KANG
;
Hyery KIM
;
Ho Joon IM
;
Jong Jin SEO
Author Information
1. Division of Pediatric Hematology/Oncology, Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea. jjseo@amc.seoul.kr
- Publication Type:Multicenter Study
- Keywords:
Lymphoma;
Non-Hodgkin;
Children;
Treatment
- MeSH:
B-Lymphocytes;
Burkitt Lymphoma;
Child;
Chungcheongnam-do;
Cohort Studies;
Disease-Free Survival;
Drug Therapy;
Humans;
Incidence;
Lymphoma;
Lymphoma, B-Cell;
Lymphoma, Large-Cell, Anaplastic;
Lymphoma, Non-Hodgkin;
Lymphoma, T-Cell;
Lymphoma, T-Cell, Peripheral;
Peripheral Blood Stem Cell Transplantation;
Precursor Cell Lymphoblastic Leukemia-Lymphoma;
Retrospective Studies;
T-Lymphocytes
- From:Clinical Pediatric Hematology-Oncology
2016;23(2):116-124
- CountryRepublic of Korea
- Language:English
-
Abstract:
BACKGROUND: Among pediatric non-Hodgkin lymphomas, there are 4 major subtypes: Burkitt lymphoma, lymphoblastic lymphoma, diffuse large B-cell lymphoma, and anaplastic large-cell lymphoma. Understanding of other rare subtypes derives only from small pediatric case series. We report our institutional experience with rare pediatric NHLs.METHODS: Thirty-six cases of rare NHL subtypes diagnosed at the Asan Medical Center from 1995 to 2015 were evaluated. We retrospectively reviewed the clinical and pathologic features and outcomes of these patients, excluding peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS), and extranodal NK/T-cell lymphoma (ENKL), on which we have previously reported.RESULTS: There were 23 cases of T-cell lineage (13 PTCL, 6 ENKL, 2 subcutaneous panniculitis-like T-cell lymphoma, 1 primary cutaneous CD4+2016-11-22 small/medium sized T-cell lymphoma, 1 enteropathy-associated T-cell lymphoma) and 13 cases of B-cell lineage lymphoma (5 marginal zone lymphoma, 6 primary mediastinal large B-cell lymphoma, 2 immunoblastic and plasmablastic lymphoma). All patients were treated with chemotherapy with or without surgery, except 4 out of 5 patients with marginal zone lymphoma who received surgery only. Two patients died and 6 patients relapsed. One patient with primary mediastinal large B-cell lymphoma received autologous peripheral blood stem cell transplantation. The 5-year overall survival and event-free survival rates of rare pediatric NHL excluding PTCL, NOS, and ENKL was 80.0% and 72.0%, respectively.CONCLUSION: Children diagnosed with rare pediatric NHL other than PTCL, NOS, and ENKL showed variable incidence and treatment outcomes. Multicenter studies in larger cohorts are needed for better understanding of these rare NHL subtypes in childhood.
