Pediatric Hodgkin and non-Hodgkin lymphomas differ from adult cases in biology and management, yet there is a lack of survival analysis tailored to pediatric lymphoma. We analyzed lymphoma data from 1975 to 2018, comparing survival trends between 7,871 pediatric and 226,211 adult patients, identified key risk factors for pediatric lymphoma survival, developed a predictive nomogram, and utilized machine learning to predict long-term lymphoma-specific mortality risk. Between 1975 and 2018, we observed substantial increases in 1-year (19.3%), 5-year (41.9%), and 10-year (48.8%) overall survival rates in pediatric patients with lymphoma. Prognostic factors such as age, sex, race, Ann Arbor stage, lymphoma subtypes, and radiotherapy were incorporated into the nomogram. The nomogram exhibited excellent predictive performance with area under the curve (AUC) values of 0.766, 0.724, and 0.703 for one-year, five-year, and ten-year survival, respectively, in the training cohort, and AUC values of 0.776, 0.712, and 0.696 in the validation cohort. Importantly, the nomogram outperformed the Ann Arbor staging system in survival prediction. Machine learning models achieved AUC values of approximately 0.75, surpassing the conventional method (AUC =  ~ 0.70) in predicting the risk of lymphoma-specific death. We also observed that pediatric lymphoma survivors had a substantially reduced risk of lymphoma after ten years b,ut faced an increasing risk of non-lymphoma diseases. The study highlights substantial improvements in pediatric lymphoma survival, offers reliable predictive tools, and underscores the importance of long-term monitoring for non-lymphoma health issues in pediatric patients.

BACKGROUND: To evaluate the efficacy of PET/CT using18F-FDG (18F-fluorodeoxyglucose) as a radiotracer compared to conventional bone marrow biopsy (BMB) in detecting infiltration to bone marrow (BM) in pediatric patients with lymphoma at the time of initial diagnosis.
METHODS: 66 pediatric patients with lymphoma (47Hodgkin\'s and 19non-Hodgkin\'s lymphoma) were referred for initial staging by18F-FDG PET/CT study. All patients underwent bilateral iliac BMB and 18F-FDG PET/CT scan with no more than 2 weeks interval in-between. Follow-up for at least 6 months was used as a reference standard to compare diagnostic performance between two modalities in detecting bone marrow infiltration (BMI).
RESULTS: Sensitivity, specificity, accuracy, as well as positive and negative predictive values of 18F-FDG PET/CT in detecting BMI were (80%, 86%, 85%, 63%, and 94%) in contrast to BMB (80%, 53%, 59%, 33%, and 90%) respectively. 18F-FDG PET/CT was concordant to BMB in 39/66 patients (59%).
CONCLUSIONS: 18F-FDG PET/CT was more accurate and specific, with higher predictive values than BMB in detecting BMI during initial staging of pediatric lymphoma. In most pediatric lymphoma patients, 18F-FDG PET/CT can be used instead of BMB to determine BMI during their initial staging process.

OBJECTIVE: Survivors after pediatric Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL) are with lifetime risk for second primary malignancy (SPM). This necessitates a thorough analysis to better understand the potential long-term health implications for these individuals.
METHODS: We used a US-wide population-based cancer registry data to quantify the SPM risk and identify its incidence patterns among pediatric lymphoma patients.
RESULTS: We observed 4.74-fold (95% CI 4.27-5.25) and 3.40-fold (95% CI 2.78-4.10) increased risks of SPM in survivors after pediatric HL and NHL, respectively. Through over 40 years\' follow-up, the cumulative incidence of SPM for pediatric lymphoma was persistently increasing, and here we firstly report the high 40-year cumulative incidence rates of SPM, 22.2% for HL and 12.6% for NHL, suggesting that SPM accounts for a great proportion of deaths among survivors. Of 6805 pediatric lymphomas, 462 (6.36%) developed a SPM, especially second breast and thyroid cancer, followed by hematologic neoplasms including leukemia and NHL. The competing risk analysis demonstrated gender, lymphoma subtype and radiotherapy were significantly associated with SPM. Different risk patterns of SPM were identified between pediatric HL and NHL. Chemotherapy accelerated SPM development but did not increase its incidence risk.
CONCLUSIONS: Overall, patients after pediatric lymphoma can be with high lifetime risk of SPM, and more attention should be paid to SPM-related signs for early detection and intervention.

Pediatric-type follicular lymphoma is an uncommon and newly recognized entity of lymphoid neoplasm commonly encountered in the young population. Despite its indolent clinical course and localized nodal involvement, it has been characterized by its high-grade histopathological features. The overlapping features between this disease and several entities have made approaching this unique entity significantly challenging, with all such features being reflected in the strict diagnostic criteria highlighted by the WHO 2016 lymphoid malignancy classification. Despite its characteristic high-grade histology, its cure rates have remained high, with relapse and transformation rarely occurring. Interestingly, several cases have achieved remission following nodal disease resection, possibly eliminating the need for chemotherapy and radiation and preventing long-term morbidities from later approaches in disease survivors.

UNASSIGNED: Burkitt lymphoma (BL) is a common tumor of childhood that usually arises in the abdomen or pelvis in its sporadic form. In a minority of cases, BL can present with CNS involvement, usually as a secondary site. Rarely, BL can arise primarily in the epidural space and present with back pain, or less commonly, acute myelopathy. This presentation is a surgical emergency and requires vigilant management.
UNASSIGNED: We describe a case of pediatric BL arising primarily within the epidural space and presenting with progressive difficulty walking in a 3-year-old boy. Progression to complete inability to walk, absent lower extremity deep tendon reflexes, and new urinary incontinence prompted MRI of the spine, which showed a lesion extending from T5 to T10 and wrapping around the anterior and posterior portions of the spine with evidence of spinal cord compression. The patient underwent decompressive laminectomies from T5 to T10 and partial debulking of the posterior portions of the tumor. Microscopic examination showed a prominent \"starry sky\" pattern with abundant mitotic figures. Immunohistochemistry confirmed the diagnosis of BL. The patient is 10 months post-op and continues to undergo chemotherapy with partial neurologic improvement. He was free of recurrence 10 months post-operative.
UNASSIGNED: This appears to be the youngest described patient presenting with acute myelopathy in primary paraspinal BL. Management should include surgical decompression of the spinal cord followed by one of the various described chemotherapeutic regimens. Preoperative staging and neurologic function correlate with prognosis.

BACKGROUND: Although primary cutaneous B-cell lymphomas (PCBCL) comprise 25% of all cutaneous lymphomas, their incidence in the pediatric population is unknown, and the information on pediatric PCBCL has mostly been gathered from individual case reports or series from single centers.
METHODS: This was a population-based, retrospective cohort study of patients in 18 cancer registries in the United States diagnosed between 2000 to 2016 through the Surveillance, Epidemiology, and End Results (SEER) program. Age-adjusted incidence rates were calculated for PCBCL in pediatric (<20 years) and adult (≥20 years) populations. Demographic, clinical, and pathological characteristics of PCBCL were compared between the two groups.
RESULTS: A total of 48 pediatric and 5128 adult PCBCL cases were included. Median age at diagnosis was 16.5 years and 65 years in the two groups, respectively. The major histologic subtypes of pediatric cases were marginal zone lymphoma (77.1%), followed by diffuse large B-cell lymphoma (12.5%) and follicle center lymphoma (10.4%), which were equally distributed in adults. The age-adjusted pediatric PCBCL incidence rate (per 1,000,000 person-years) was 0.12 (95% CI 0.09-0.16). The incidence in the adult population was approximately 40-fold higher than the one observed in the pediatric group (IRR 41.4, 95% CI 31.2-56.2). All 48 pediatric cases were alive during a median follow-up time of 48 months.
CONCLUSIONS: Pediatric PCBCL is a very rare disease affecting mostly adolescents of both sexes. The major histologic subtype is marginal zone lymphoma, and the prognosis is favorable.

Non-anaplasticperipheral T-cell lymphomas (PTCL) are rare tumors in children, adolescents, and young adults (CAYA) with poor prognosis and scarce genetic data. We analyzed lymphoma tissue from 36 patients up to 18 years old with PTCL, not otherwise specified (PTCL-NOS), hepatosplenic T-cell lymphoma, Epstein-Barr virus (EBV)-positive T-lymphoproliferative diseases, subcutaneous panniculitis-like T-cell lymphoma, and other PTCL types. Twenty-three patients (64%) had at least one genetic variant detectable, including TET2, KMT2C, PIK3D, and DMNT3A. TP53 and RHOA variants, commonly found in adults, were not identified. Eight of 20 (40%) CAYA PTCL-NOS had no detectable mutations. The genetic findings suggest that CAYA PTCL differ from adult cases.

Global variation in lymphoma incidence by type and age at diagnosis, region, sex, and Human Development Index (HDI) categories has not been reported, may shed light on potential biologic mechanisms and identify areas for targeted interventions.
Using the Cancer Incidence in Five Continents data from 1988 to 2012, we identified Hodgkin (HL), non-Hodgkin (NHL), and Burkitt lymphoma (BL) diagnosed in children aged 0-19 years. We estimated incidence rates (IRs; cases/million) and average annual percent change in incidence (AAPC; 95 % CI) by geographic region, sex, and HDI for each age group (0-9years and 10-19 years).
There were 42,440 NHL, 38,683 H L, and 7703 included. Southern European (SE) 10-19-year-olds (yo) had the highest IR of NHL (19.6 cases/million) in 2008-2012. HL IRs for 0-9yo were <6 cases/million and >25 cases/million for 10-19yo in European regions and Oceania (OC). BL IRs were generally <5cases/million. Northern Europe (NE), SE, and OC 10-19yo had significantly increased APPCs in incidence for all lymphomas with the largest increases in BL (NE AAPC: 7.69 %; 95 % CI: 5.27, 10.16; SE AAPC: 5.21 %; 95 % CI: 3.26, 7.19; OC AAPC: 3.97 %; 95 % CI: 3.26, 4.70). BL incidence increased among males of all ages by approximately 2 %. NHL and BL incidence increased significantly among 10-19yo in very high HDI countries by approximately 3 %.
Southern and Northern Europe and Oceania displayed increased incidence of all lymphomas studied from 1988 to 2012. BL incidence significantly increased in 8 of 15 global regions, males, and higher HDI countries over the study period. Mechanisms underlying these increases remain to be elucidated.

The aim of this study was to analyze whether the baseline metabolic parameters of 18F-FDG PET/CT in pediatric patients with Burkitt lymphoma (BL) can predict treatment response and prognosis. We retrospectively analyzed 68 pediatric patients with BL who underwent PET/CT before treatment. PET images were analyzed semi-quantitatively by measuring the maximum standardized uptake (SUVmax), total metabolic tumor volume (tMTV), and total lesion glycolysis (TLG). Survival curves were plotted according to the Kaplan-Meier method. Univariate and multivariate Cox proportional hazards regression models were used to assess the relation between potential variables and outcomes. tMTV and TLG were significantly lower in patients with complete response compared with those with partial response at the end of treatment. PET metabolic parameters (tMTV and TLG) were the independent prognostic values for outcome. TMTV and TLG were significantly connected with treatment response and prognosis in pediatric with BL.

BACKGROUND: Cancer is the leading cause of disease-related mortality among children, 0-14 years, and lymphoma, a malignant neoplasm of the lymphoid cells, mostly lymphatic B and T cells is common among children. The current study aimed to assess the cumulative incidence (CmI), mortality, and survival in pediatric lymphoma.
METHODS: A retrospective cohort was utilized to examine children, 0-19 years with lymphoma for CmI, mortality and survival from the Surveillance, Epidemiology, and End Results (SEER) data. The variables assessed included social determinants of health, namely urbanity, median household income, and race. While chi square was used to characterize study variables by race, binomial regression was employed for mortality risk. The Cox proportional hazard model was used for survival modeling.
RESULTS: The CmI was higher among white children (76.67%) relative to Black/African American (AA, 13.44%), American Indian/Alaskan Native (AI/AN, 0.67%), as well as Asian/Pacific Islander (A/PI, 7.53%). With respect to mortality, there was excess mortality among Black/AA children compared to white children, Risk Ratio (RR) = 1.54, 95% CI, 1.33-1.79. Relative to whites, Blacks were 52% more likely to die, Hazard Ratio (HR) = 1.52, 95% CI, 1.30-1.78. Survival disadvantage persisted among Blacks/AA after controlling for the other confoundings, adjusted hazard ratio (aHR) = 1.54, 99% CI, 1.24-1.91.
CONCLUSIONS: In a large cohort of children with lymphoma, Black/AA children relative to whites presented with survival disadvantage, which was explained by urbanity and median household income, suggestive of transforming the physical and social environments in narrowing the racial differences in pediatric lymphoma survival in the US.