Prognostic Factors and Treatment Outcome of Relapsing and Refractory Pediatric Mature B-cell Non-Hodgkin Lymphoma, Children's Cancer Hospital Egypt Experience.

BACKGROUND: Relapsed non-Hodgkin lymphoma treated by chemotherapy and hematopoietic stem cell transplantation (HSCT) has a dismal prognosis. PATIENTS: It is a retrospective study, including pediatric patients diagnosed as mature B-cell non-Hodgkin lymphoma who were primarily refractory or relapsed over 8 years at CCHE. The aim of the study was to analyze the prognostic factors and outcomes of this group of patients. Our result is, 53 of 750 (7%) patients were included. Thirty-four (64.2%) patients had Burkitt lymphoma. Forty-eight (90.6%) patients received LMB 96 protocol initially. The median delay of duration between chemotherapy cycles in first-line treatment was 37 days. Thirty-five (66%) patients relapsed, 23 (65.7%) of them relapsed early, whereas 18 (34%) had tumor progression. Thirty-one (58.5%) patients presented with stage IV at the time of relapse. rituximab, ifosfamide, carboplatin, etoposide was the second line of treatment in 42 (79.24%) patients, and complete second remission was achieved only in 13 (24.3%) patients. Allogeneic HSCT was done for 4 (7.5%) patients, and auto HSCT was done for 3 (5.7%) patients. Three years of overall survival for relapsed and progressed patients were 35.3% and 11.1%, respectively, with a P-value of 0.009. Three years overall survival for patients who underwent HSCT was 85.7% compared with 18.1% for no HSCT with a P-value of 0.007. CONCLUSIONS: The relapse rate is higher than literatures because of the delay of duration between chemotherapy cycles in first-line treatment and more advanced stage at time of relapse. Progression of the disease had a worse outcome than relapse. HSCT in patients with the second remission markedly improved the outcome.

Can FDG-PET replace biopsy for the evaluation of residual tumor in pediatric mature B-cell non-Hodgkin lymphoma?

INTRODUCTION: The aim of our study is to evaluate the role of 18 F-labeled fluorodeoxy glucose positron emission tomography (18 FDG-PET) scan for the detection of viable residual mass in pediatric mature B-cell non-Hodgkin lymphoma (NHL). This study also aims to detect the negative predictive value, positive predictive value (PPV), sensitivity, and specificity of 18 FDG-PET. PATIENTS AND METHODS: A retrospective, cross-sectional nonrandomized study was carried out. We included all patients with newly diagnosed mature B-cell NHL treated at the Children Cancer Hospital Egypt during the period between July 2007 and the end of May 2018. Patients were included in the study if they (a) had a residual tumor mass, (b) underwent an 18 FDG-PET scan, and (c) had a pathologic documentation of this residual tumor. Patients were followed up till June 2019. RESULTS: Thirty-six patients were included, for whom 39 biopsies were performed. Mean age was 7.7 years. Median follow-up period was 52.8, range 6.1 to 117 months.18 FDG-PET scan was positive (Deauville score 3, 4, or 5) in 24 of 39 patients (61.5%), while it was negative (Deauville score 1 or 2) in 15 patients (38.5%). Positive 18 FDG-PET scan and biopsy were performed in 15 of 39 samples (38.4%; true positive, TP), while they were both negative in 13 samples (33.3%; true negative). Nine patients (23%) had positive scan and a negative biopsy (false positive), while 2 patients had negative uptake and a positive biopsy (false negative, FN)). Sensitivity of the 18 FDG-PET scan was 88.2% and specificity was 59.1%. PPV was 62.5% and NPPV was 86.6%. CONCLUSION: Changing therapy on the basis of a positive finding alone at the time of evaluation is not recommended. FN results exist, so biopsy confirmation is required to avoid the missing refractory disease. If negative, 18 FDG- PET can replace a biopsy if the latter is inaccessible or carries an unnecessary risk.

Outcome of Children Treated for Infantile Hepatic Hemangioendothelioma.

PURPOSE: Infantile hepatic hemangioendothelioma (IHHE) is the most common hepatic vascular tumor in children. We report on the treatment outcome of our large single-center experience of patients with IHHE over a 9-year period. MATERIALS AND METHODS: A retrospective analysis of all IHHE patients treated at the Children Cancer Hospital Egypt from April 2008 through April 2017. RESULTS: In total, 28 patients (18 females, 10 males) were diagnosed with IHHE with a median age at diagnosis of 3 months. The lesions were multifocal (n=12), focal (n=10), and diffuse (n=6). Six (21.4%) patients initially had low T3 and T4. Eleven patients did not receive any treatment, whereas 1 patient underwent resectional surgery. Sixteen patients received drug treatment, 9 of whom responded well to first-line propranolol/prednisolone, whereas 7 patients needed salvage treatment. Twenty-five patients are alive, whereas 3 patients have died. CONCLUSIONS: Overall, patients with IHHE do well, a significant percentage of whom do not require drug therapy, particularly for those with small focal lesions. In patients with multifocal/diffuse disease, there is a high incidence of low T3 and T4 and while some of these patients did well without additional therapy, those with rapidly progressive lesions during treatment may do poorly.