title: Childhood malignant non-Hodgkin lymphomas of uncommon histology.
abstract: 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.
authors.affiliation: Department of Hematology-Oncology, St Jude Children's Research Hospital, Memphis, TN.
authors.forename: R C
authors.initials: RC
authors.lastname: Ribeiro
authors.forename: C H
authors.initials: CH
authors.lastname: Pui
authors.forename: S B
authors.initials: SB
authors.lastname: Murphy
authors.forename: J J
authors.initials: JJ
authors.lastname: Shuster
authors.forename: E V
authors.initials: EV
authors.lastname: Hvizdala
authors.forename: J
authors.initials: J
authors.lastname: Falletta
authors.forename: M P
authors.initials: MP
authors.lastname: Link
authors.forename: M P
authors.initials: MP
authors.lastname: Sullivan
authors.forename: T E
authors.initials: TE
authors.lastname: Pick
authors.forename: J T
authors.initials: JT
authors.lastname: Sandlund
mesh_terms: 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