Nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL) is a rare variant of Hodgkin's lymphoma (HL) in children. Since specific immunohistochemical staining has become available, NLPHL can be separated from classical Hodgkin's lymphoma (cHL) more accurately. Scarce information is available about pediatric NLPHL treated with chemotherapy only. Therefore, clinical characteristics, treatment, response and outcome of seven pediatric NLPHL patients, median age 9.2 years (range 7.5 - 14.2 years), diagnosed between 1986 - 2003 among 58 HL patients, uniformly treated in a single center with chemotherapy only, were evaluated. The median follow-up time was 4.2 years (range 2.1 - 10.2 years). NLPHL patients were stage I (n = 5), II (n = 2), whereas cHL, median age 11.4 years (range 3.3 - 15.9 years), were stage I (n = 8), II (n = 17), III (n = 9), IV (n = 1). Upfront treatment of NLPHL patients consisted of six courses of epirubicin, bleomycin, vinblastine and dacarbazine (EBVD) without radiotherapy, whereas cHL patients received six courses of EBVD (n = 14) or 4 - 6 courses of EBVD/MOPP (mitoxin, oncovin, procarbazine, prednison; n = 21). Chemotherapy was used as primary treatment thereby aiming to avoid radiotherapy with potential serious side effects to growing jaws and thyroid. All seven patients reached complete remission (CR). Four patients relapsed, of which three locally. These three were salvaged with second-line chemotherapy without radiation therapy (RT) and are in second CR. One patient relapsing with stage III disease was salvaged by EBVD/MOPP followed by autologous BMT and is also in second CR for 36 months. The event-free survival (EFS) is 43% and overall survival (OS) 100%. This study shows that apart from histology, immunohistochemistry (ICH) is required for diagnosing NLPHL. Moreover, it illustrates that although cure of pediatric NLPHL is feasible with chemotherapy only, high dosages of cytotoxic drugs are necessary as salvage treatment in a relatively high proportion of patients after relapse.

Objective Long-term outcomes of patients with ruptured hepatocellular carcinoma (rHCC) remain scant. This study aimed to assess disease-free survival (DFS) and overall survival (OS) after surgical resection of rHCC compared to non-ruptured HCC (nrHCC). Methods Patients with rHCC and nrHCC were collected from 8 centers in Europe, Asia, and North America. Resected rHCC patients were matched 1:1 to patients undergoing surgery for nrHCC using propensity score and nearest-neighbor method (matching criteria: age, tumor size, cirrhosis, Child-Pugh score, Barcelona Clinic Liver Cancer stage, resection status, grade, and microvascular invasion). Survival rates were calculated using Kaplan-Meier method. Results A total of 2033 patients were included: 226 rHCC patients (172 operated: 68 with upfront surgery and 104 after embolization) and 1807 nrHCC patients. Median DFS and OS of rHCC patients (all treatments confounded) were 10 months (95% CI 7–13) and 22 months (95% CI 13–31). Prognostic factors for worse OS among rHCC patients were absence of preoperative arterial embolization (HR 2.3, 95% CI 1.2–4.6, p=0.016), cirrhosis Child B/C (HR 2.4, 95% CI 1.1–5.4, p=0.040), and R1/R2 margins (HR 2, 95% CI 1–5, p=0.049). Survivals were similar between Western and Eastern rHCC patients. After propensity score matching, 106 rHCC patients and 106 nrHCC patients displayed similar characteristics. Patients with rHCC had shorter median DFS (12 months, 95% CI 7–17 vs. 22 months, 95% CI 12–32, p=0.011), but similar median OS compared to nrHCC patients (43 months, 95% CI 21–65 vs. 63 months, 95% CI 21–105, p=0.060). Conclusion In this large dataset including Eastern and Western patients, rHCC was associated with shorter DFS compared to nrHCC, while OS was similar.