Hodgkin lymphoma in very young children: Clinical characteristics and outcome of treatment

In developed nations, Hodgkin lymphoma (HL) is rare in \u3c5-year olds and represent a minority in developing countries. Little is reported about the biology and behavior of these very young patients compared with older children. 18.75% of our pediatric HL patients (0 - 14 years) were \u3c5 years at diagnosis. This group had more boys, similar incidence of B-symptoms and stage distribution, less mediastinal involvement and bulky disease, and more mixed cellularity subtype than older children. Treatment included chemotherapy (CT; n = 55), combined modality therapy (CMT; n = 12) and XRT only (n = 2). Ten-year EFS and OS was 81.5% and 90.4%, respectively, versus 75.5% and 90.5% for older children (p \u3e 0.5). A trend toward better survival was seen with CMT, using very LD-XRT, than with CT (OS 100% vs. 86.4%[p = 0.3]; EFS 90.9% vs. 81.0%[p = 0.4]). Although CT could be effective in a subset of LR patients, LD-XRT may be needed to effectively treat most of these patients. This dose reduction may reduce XRT-related toxicity, which can be significant in very young children

Pediatric Hodgkin lymphoma patients treated with ABVD chemotherapy with or without low-dose radiation therapy

The ABVD protocol is probably the most effective chemotherapy (CTX) regimen for the treatment of Hodgkin Lymphoma (HL), however the dose and volume, and indeed the need for radiation therapy (XRT) in combination remains uncertain. Pediatric patients (0–14 years) at our institution have been treated with ABVD either with or without XRT, based on the treating physician’s decision. Patients receiving XRT were usually given one or two cycles less of CTX than those without. Since 1998 we have used 1500cGy as the dose of XRT, however the field was determined by the radiation oncologist. Between 1990 and 2003, 152 patients were treated according to the ABVD protocol. Of these, 64 were treated with CTX alone, while 88 also received radiation as consolidation therapy (Combined modality therapy; CMT). Of those who received XRT 63 were administered a dose of 1500cGy. The remaining 25 received various higher doses (1655cGy: 1, 2400cGy: 9, 2500cGy: 10, 3500cGy: 3, 3680cGy: 1, 3980cGy:1). Patients who were treated with CMT were older (mean age 9.2 v. 7.4 years; p\u3c0.05), had less B-symptoms (10.2% v. 26.6%; p\u3c0.05), but not more bulky disease (43.2% v. 34.4%; p=0.2). CTX group had more patients with stage III and IV disease, while CMT group had more stage II disease (p\u3c0.05). With a median follow-up of 4 years, the actuarial overall survival (OS) and event free survival (EFS) at 5 years for all the patients is 97.3% and 88.0%, respectively. The OS and EFS for the patients treated with CTX and CMT were 95.3% v. 98.8% (p=0.4) and 85.1% v. 90.2% (p=0.3), respectively. We next looked at the patients who received only 1500cGy of radiation therapy. Of the 63 patients, 29 received extended field radiation (EFXRT) and 34 involved field radiation (IFXRT). Patients who received radiation were administered a median of two cycles of ABVD less than those who did not (median 4 v. 6 cycles; mean 4.3 v. 5.1, p\u3c0.05). OS at 5 years for the patients treated by CTX v. CMT/EFXRT v. CMT/IFXRT is 95.3%, 96.6% and 100%, respectively (p=0.3). The EFS for the same groups is 85.1%, 86.2% and 90.1% (p=0.4).Conclusion: Pediatric patients with HL can be treated successfully with minimal or no XRT. These results need to be confirmed in a prospective clinical trial

Clinical characteristics and outcome of pediatric patients with stage IV Hodgkin lymphoma

Background and objectives: While treatment outcomes for patients with Hodgkin lymphoma (HL) have improved remarkably, patients with disseminated disease still have a poorer outcome. Stage IV HL is often reported with other \u27advanced stage\u27 categories, confusing the specific contribution of disease dissemination to the outcome. This single-institution report looks at characteristics and outcomes of this specific category.Patients and methods: The medical records of pediatric HL patients (\u3c 14 years) from 1975 through 2003 were retrospectively reviewed and the data analyzed.Results: Stage IV patients (n = 67) had more poor-risk characteristics than patients in stages I-III (n = 300) (B symptoms 86.6% vs. 19.3%, bulky disease 57.6% vs. 45.5% and mediastinal mass 77.6% vs. 29.7%; P \u3c .001 for all characteristics). The liver was the most common extralymphatic site (in 51.5% of patients with stage IV disease. Stage IV patients received chemotherapy (CT) alone (n = 55) or combined modality therapy (CMT) (n = 12). Fifty-four patients (80.6%) achieved complete remission, 2 (3%) partial remission, 10 (14.9%) had progressive disease and 1 was lost to follow up. Overall survival was 79.4% and event-free survival (EFS) was 63.9% at 5 years. There was a non-significant benefit for CMT (OS = 91.7% v. 77.1%, P = .3; EFS = 70.7% v. 62.7%, P = .3). Ten of 12 relapsed and only 1 of 10 progressive disease patients were salvaged. On multivariate analysis, failure to achieve complete remission with CT was associated with a poorer outcome.Conclusion: Stage IV disease is associated with poor risk features and confers a worse outcome than stage I-III disease. Achievement of complete remission with CT is an important prognostic feature. Slow responders may require novel and/or aggressive therapy to achieve complete remission

Hodgkin lymphoma in very young children can be treated successfully without radiation therapy

Hodgkin Lymphoma (HL) is rarely seen in \u3c5-year olds in developed nations. Even in developing countries, where a tendency towards younger age of presentation has been shown, this represents a minority of cases. Little is known about the biology and behavior of these very young patients with HL as compared to older children.|Methods: We retrospectively reviewed HL cases diagnosed and treated at our institution between 1975 and 2003. HL was diagnosed histopathologically and staged clinically. The pediatric age group ranged from 0–14 years. Treatment strategy for these very young children was focused on the elimination of radiation therapy (XRT).Results: 69/368 (18.75%) patients were less than 5 years at diagnosis. When compared to older patients, there was a trend towards male predominance (M:F 4.31 v. 2.65; p=0.2), but no difference in the incidence of B-symptoms (26.1% v. 32.9%; p=1.0) and stage distribution (p=1.0). There was less mediastinal involvement (p=0.025) or bulky disease (p=0.01) in the younger patients. These patients had more mixed cellularity and less nodular sclerosis subtype (p=0.025). Fifty-five were treated with chemotherapy (CTX) alone, 12 with combined modality therapy (CMT) and 2 with XRT only. 35/55 CTX patients were treated with ABVD (20 per standard schedule, 13 modified and 2 unknown), 12 MOPP and 8 with hybrid or combination CTX (4 MOPP/ABVD, 3 COPP/ABVD and 1 unspecified). All CMT patients received ABVD (9 standard and 3 modified) and XRT (1500cGy/IF for 5, 1500cGy/EF for 4 and 2400cGy/EF, 2720cGy/IF and 3060cGy/IF for one each). The two XRT alone patients had stage I cervical disease and received 3900cGy and 3250cGy IFXRT. At ten years the EFS and OS for these patients under 5-years of age was 81.5% and 90.4%, respectively, compared to 75.5% and 90.5% for the children between 5 and 14 years of age (p\u3e0.5 for both comparisons). OS (86.4% v. 100%; p=0.3, Log Rank test) and EFS (81.0% v. 90.9%; p=0.4, Log Rank test) for CTX v. CMT groups were not statistically significantly different. The CTX group had more B-symptoms (29.1% v. 16.1%) and higher stage disease (stage III/IV 47.3% v. 25%; stage IV 12.73% v. 0%).Conclusions: HL patients \u3c5years old do not present with higher risk disease than older children. They can successfully be treated without XRT using CTX alone. XRT can be reserved for treating the few who relapse. This may result in reduction in XRT related toxicity, which can be significant in these very young children