Childhood malignant non-Hodgkin lymphomas of uncommon histology.
Uncommon histologies were identified in 36 of 1336 cases (2.7%) of newly diagnosed childhood non-Hodgkin lymphoma (NHL). Seventeen cases were classified as follicular (six cases as mixed small and large cell, nine as large cell, and two as small non-cleaved cell) and 19 cases as diffuse (18 cases as mixed small and large cell, and one as small cell lymphocytic). The follicular pattern group included a preponderance of male patients; the median age at diagnosis was 11.7 years. These children presented primarily with low-stage disease involving lymph nodes or tonsils. All patients except one achieved complete remission and remain disease-free for 11 months to 18.8 years (actuarial 5-year event-free survival, 94%). The group with diffuse histologies was similar in sex ratio, age at diagnosis (median = 12.1 years), and nodal involvement, but tended to have more advanced-stage disease. Moreover, only 14 of 19 (74%) children with diffuse intermediate-grade histologies are alive in continuous complete remission (actuarial 5-year event-free survival, 70%). These results suggest that follicular pattern childhood NHL has an excellent prognosis, whereas cases with diffuse intermediate-grade histology are prognostically similar to those with diffuse high-grade histologies.
Ribeiro
R C
RC
Department of Hematology-Oncology, St Jude Children's Research Hospital, Memphis, TN.
Pui
C H
CH
Murphy
S B
SB
Shuster
J J
JJ
Hvizdala
E V
EV
Falletta
J
J
Link
M P
MP
Sullivan
M P
MP
Pick
T E
TE
Sandlund
J T
JT
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008224:Lymphoma, Follicular / Q000473:pathology; D008228:Lymphoma, Non-Hodgkin / Q000473:pathology* / Q000628:therapy; D008297:Male; D015996:Survival Rate