Malignant, infectious, inflammatory Thymoma, thyroid dz or cancer, teratoma, rhabdomyosarcoma, germ cell tumor or T cell lymphoblastic lymphoma
17-40% of children with HL have a mediastinal mass on CXR at initial presentation; can initially c/o dysphagia, dyspnea, cough, stridor, or SVC syndrome
Fewer than 20% of children with HL have the classic fever and night sweats seen in adults. Pts can also present with pruritis, urticaria, fatigue postulated to be secondary to cytokine release from HRS cells
Most children and adolescents with HL have EXCELLENT prognosis with current therapy regimens. Overall 5 year survival rate for early disease >90%. The overall 5 year survival rate in patients with advanced stage disease treated with chemo + radiation ~93%
A majority of these late complications are dose and volume related to the amount of chemo +/- radiation received
Using gene-expression profiling – analyzed 130 frozen samples obtained from patients with classic HL during diagnostic LN bx to determine which cellular signatures were correlated with treatment outcome. Then they confirmed these findings in an independent cohort of 166 patients using immunohistochemical analysis for CD68 markers on macrophages – which per the study can be done by all pathology departments
Along with Lewis et al - Pollard et al 2009; Mantovani et al 1992 Possibly, the number of tumor associated macrophages leads to a poor response to treatment because their increased number signals a deregulation of apoptosis in Reed-Sternberg cells that inhibits cell death in response to cytotoxic agents????
FUTURE = Catering therapy!!!
Pediatric Case Management By Richard Lirio,MD 16 th June 2010
The most widely used staging system is the Ann Arbor staging system.
Stage I - Single lymph node region or single extranodal site
Stage II - Two or more lymph node regions on the same side of the diaphragm
Stage III - Lymph node regions on both sides of the diaphragm
Stage IV - Diffuse or disseminated involvement of one or more extralymphatic organs (liver, bone marrow, lung) or tissues with or without associated lymph node involvement (The spleen is considered a nodal site.)
Standard treatment regimens for pediatric Hodgkin lymphoma are as follows:
Early or favorable disease (stage IA or IIA with <3 nodal sites)
2-4 chemotherapy cycles without alkylators (VAMP; etoposide, bleomycin, vinblastine, and prednisone [EBVP]; OEPA; or ABVE) plus low-dose, involved-field radiation of 15-30 Gy or 6 chemotherapy cycles (alternating COPP and ABVD or derivatives of these regimens) and no irradiation. The use of very limited doses of chemotherapy (2-3 cycles) should be administered only as part of a clinical trial.
Intermediate-risk disease (stage IA, IIA, or IIA bulky disease with extension or > 3 nodal sites):
4-6 chemotherapy cycles (OPPA and COPP, Stanford V) plus low-dose, involved-field radiation of 15-30 Gy or 6 chemotherapy cycles (alternating COPP and ABVD or their derivatives) or a dose-intense, hybrid regimen (eg, Stanford V, ABVE-PC, or BEACOPP) and no irradiation.
Advanced or unfavorable disease (stages IIB, IIIB, or IV):
6-8 chemotherapy cycles (OPPA and/or COPP, ABVE-PC, BEACOPP) plus low-dose involved-field radiation of 15-30 Gy or 6-8 chemotherapy cycles (alternating COPP and ABVD or their derivatives) or a dose-intense, hybrid regimen (ABVE-PC or BEACOPP) and no irradiation.
"An increased number of tumor-associated macrophages was strongly associated with shortened survival in patients with classic Hodgkin's lymphoma, and provides a new biomarker for risk stratification"
The tumor-associated macrophages were identified by a single marker of CD68+ cells
Steidl et al. Tumor-Associated Macrophages and Survival in Classic Hodgkin’s Lymphoma. N Engl J Med. 2010; Vol 362, No 10:875-85