Variant histology, IgD and CD30 expression in low‐risk pediatric nodular lymphocyte predominant Hodgkin lymphoma: A report from the Children's Oncology Group

Abstract

Background Histologic prognostic factors have been described for nodular lymphocyte predominant Hodgkin lymphoma (NLPHL). This study examines histologic and immunophenotypic variants in a clinical trial for pediatric NLPHL. Procedure One hundred sixty-eight cases of localized NLPHL were examined for histologic variants, CD30 and immunoglobulin D (IgD) expression, and outcome. Histologic types were scored categorically as 0 = 0, 1 ≤ 25%, and 2 > 25% of the sample. Results Fifty-eight (35.1%) cases showed only typical nodular with or without serpiginous histology (types A and B). The remainder showed mixtures of histologies. The numbers of patients with score 2 are 85 (50.6%) type A, 21 (12.5%) type B, 46 (27.4%) with extranodular large B cells (type C), 3 with T-cell-rich nodular pattern (type D), 55 (32.7%) with diffuse T-cell-rich (type E) pattern, and 2 (1.2%) with diffuse B-cell pattern (type F). Higher level of types C (P = 0.048) and D (P = 0.033) resulted in lower event-free survival (EFS). Cytoplasmic IgD was found in 65 of 130 tested (50%), did not significantly associate with EFS but positively correlated with types C and E histology (P < 0.0001) and negatively correlated with types A (P = 0.0003) and B (P = 0.006). Seventeen (10%) expressed CD30, with no adverse effect. Conclusions Variant histology is common in pediatric NLPHL, especially types C and E, which are associated with IgD expression. Type C variant histology and possibly type D are associated with decreased EFS, but neither IgD nor CD30 are adverse features. Variant histology may warrant increased surveillance, but did not affect overall survival.

Unfavorable presentation but comparable outcome: Presentation and outcome of children with nodular lymphocyte predominant Hodgkin lymphoma from India

“…European cohorts have noted an increased incidence of advanced stage disease, poorer response to chemotherapy, and increased recurrences in variant histology NLPHL as compared to patients with typical NLPHL, although the influence on overall survival is inconsistent 26,30,32,[35][36][37][38][39][40]. Our study also noted the higher incidence of unfavorable presentation and poorer EFS in children with variant NLPHL histology.…”

“…Our study also noted the higher incidence of unfavorable presentation and poorer EFS in children with variant NLPHL histology. A larger prospective study of pediatric NLPHL found type C and possibly type D variant histology was associated with decreased EFS 36. Our study found Fan D and E histology to have the lowest 5 year EFS, with Fan C histology having lower overall survival.…”

“…Pediatric NLPHL is a rare tumor and a fascinating entity, and understanding of its biology is constantly evolving . Incorporation of these advances in histopathology into risk stratification, as well as treatment and follow‐up as part of large collaborative trials will help us further understand this rare disease and refine treatment strategies.…”

“…Pediatric NLPHL is a rare tumor and a fascinating entity, and understanding of its biology is constantly evolving. 30,[36][37][38][39][40] Incorporation of these advances in histopathology into risk stratification, as well as treatment and follow-up as part of large collaborative trials will help us further understand this rare disease and refine treatment strategies. It will also be interesting to see if the biology of NLPHL and response to treatment is different in India compared to other parts of the world.…”

Adolescent and young adult Hodgkin lymphoma: Raising the bar through collaborative science and multidisciplinary care

“…According to the Cooperative Human Tissue Network, 57% of the expected number of biologic specimens is banked for AYAs with cancer . Differences in HL histologic subtypes have been reported across the age spectrum . Approximately 45% of children <15 years have nodular sclerosing (NS) histology versus 80% of AYAs; mixed cellularity (MC) histology is more common in younger patients versus AYAs (∼35% vs. 15%), and nodular lymphocyte predominant HL is less common in AYAs versus older patients .…”