Non-anaplastic peripheral t-cell lymphoma in childhood and adolescence: a Children's Oncology Group study.
BACKGROUND
Peripheral T-cell lymphomas (PTCL) other than anaplastic large cell lymphoma (ALCL) are rare in young patients. While a high proportion of adults with PTCL have poor risk disease, pediatric PTCL is not well characterized. This study examines the outcome of localized and advanced PTCL in pediatric patients treated in standardized fashion.


METHODS
We identified 20 pediatric patients diagnosed with PTCL whose tumor cells did not express CD30 and/or ALK, as determined by immunohistochemistry, between 1992 and 2000 on one of two treatment protocols for localized NHL (POG 9219) or advanced stage large cell lymphoma (POG 9315). All cases were centrally reviewed.


RESULTS
The median age was 12.6 (range 0.7-16.9)-9 male and 11 female. Histological subtypes in the WHO Classification included PTCL, unspecified (12), extra-nodal NK/T-cell lymphoma of nasal type (4), subcutaneous panniculitis-like T cell lymphoma (1) and enteropathy-type T-cell lymphoma (1). Two cases exhibited both T-cell and histiocyte markers and were reclassified as histiocytic sarcoma per the WHO, although T-lineage remains possible. Of 10 patients with localized disease, only two relapsed and 9 survive. Of 10 patients with advanced disease, six relapsed and five (50%) survive.


CONCLUSIONS
These results suggest that localized PTCL in children and adolescents is frequently cured with modern therapy, but that advanced stage cases may require novel therapy.
Hutchison
Robert E
RE
SUNY Upstate Medical University Department of Pathology, Syracuse, New York, USA. hutchisb@upstate.edu
Laver
Joseph H
JH
Chang
Myron
M
Muzzafar
Tariq
T
Desai
Sunil
S
Murphy
Sharon
S
Schwenn
Molly
M
Shuster
Jonathan
J
Link
Michael P
MP
D000293:Adolescent; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D016411:Lymphoma, T-Cell, Peripheral / Q000145:classification* / Q000188:drug therapy / Q000401:mortality* / Q000473:pathology; D008297:Male; D012008:Recurrence; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome
D000970:Antineoplastic Agents