Childhood Non-Hodgkin's Lymphoma
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The lymphatic system helps the body fight infection. There are two main types of cancer associated with the lymphatic system: Hodgkin's Disease and Non-Hodgkin's Lymphoma (NHL). Both are rare in children aged under 3, and are more common in older children and adults. More boys than girls have childhood Hodgkin's disease.Childhood non-Hodgkin's lymphoma is a disease in which cancer (malignant) cells are found in the lymphatic system.

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Latest Research Publications

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Latest Research Publications

This list of publications is regularly updated (Source: PubMed).

Gabali A, Ross CW, Edwards PC, et al.
Pediatric extranodal marginal zone B-cell lymphoma presenting as amyloidosis in minor salivary glands: a case report and review of the literature.
J Pediatr Hematol Oncol. 2013; 35(3):e130-3 [PubMed]
We report an unusual case of an extranodal marginal zone B-cell lymphoma (EMZL) arising in the labial minor salivary gland in an immunocompetent 11-year-old boy. The initial histopathologic review favored localized amyloidosis. However, further evaluation supported the diagnosis of low-grade B-cell lymphoma with plasmacytic differentiation, surrounded by deposits of AL κ-type amyloid. Clinical management consisted of excision with no recurrence at 1-year follow-up. This case demonstrates that a diagnosis of lymphoma must be considered in cases of amyloidosis associated with minor salivary gland involvement, even in children. In addition, we provide a literature review of extranodal marginal zone B-cell lymphoma arising in salivary glands.


Sandlund JT, Choi JK
Pediatric MLBL: challenges remain.
Blood. 2013; 121(2):245-6 [PubMed]
In this issue of Blood, Gerrard et al report the outcome and histologic classification of children and adolescents with mediastinal large B-cell lymphoma (MLBL), and highlight the need for new treatment strategies.


Hesseling P, Israels T, Harif M, et al.
Practical recommendations for the management of children with endemic Burkitt lymphoma (BL) in a resource limited setting.
Pediatr Blood Cancer. 2013; 60(3):357-62 [PubMed]
Treatment recommendations for endemic Burkitt lymphoma (BL) in settings with only minimum requirements for curative treatment (PODC setting 1) are described. The reported cure rate for endemic BL is usually <50%. Facilities within setting 1 differ. Three treatment schedules are proposed based on: (1) when accurate staging is not possible, (2) when staging is possible and for (3) relapses and poor responders to primary therapy. A literature review and personal experience were used to formulate the recommendations. Recorded 1-year event free survival was 48% for treatment 1, 61% for treatment 2, and 35% for the rescue treatment.


Liu Q, Salaverria I, Pittaluga S, et al.
Follicular lymphomas in children and young adults: a comparison of the pediatric variant with usual follicular lymphoma.
Am J Surg Pathol. 2013; 37(3):333-43 [PubMed] Article available free on PMC after 01/03/2014
Follicular lymphoma (FL), a common lymphoma in adults, occurs rarely in pediatric and young adult patients. Most pediatric cases have been described as grade 3, but the criteria to distinguish the pediatric variant of FL (PFL) from usual FL (UFL) seen in adults are not well defined. We undertook a study of FL in patients under the age of 30. We identified 63 cases, which were analyzed by morphology, immunohistochemistry, and polymerase chain reaction analysis of IGH@ and IGK@ clonality. These data were correlated with clinical findings including stage, treatment, and outcome. Among the 63 cases, 34 cases were classified as PFL: 22 presenting in lymph nodes, 8 in the Waldeyer ring, and 4 in the testis. Clonal immunoglobulin gene rearrangement was detected in 97% of PFL cases, but fluorescence in situ hybridization analysis showed an absence of the BCL2/IGH@ translocation in all cases tested. Twenty-nine cases were classified as UFL, 28 of which presented in lymph nodes. The nodal PFLs were observed exclusively in male patients in both children and young adults with a median age of 15 years. They showed marked head/neck predilection, blastoid cytologic features with a high proliferation rate, lack of BCL2 protein and t(14;18), low clinical stage at presentation, and good prognosis. PFLs involving the Waldeyer ring were distinguished by MUM1 expression, 50% (3/6) of which carried IRF4 breaks. BCL2 expression was common (63%) in the absence of BCL2/IGH@ translocation. UFLs were more common in female patients, exclusively in young adults (median age, 24 y), with no cases reported in patients under the age of 18. Twenty-five of 29 cases were of grade 1-2, and 4 cases were classified as grade 3A. They exhibited a higher clinical stage at presentation. Eighty-three percent expressed BCL2. Our results indicate that histologic and immunophenotypic criteria can reliably separate PFL and UFL and that UFL is exceptionally rare in the pediatric age group. PFL associated with particular anatomic sites have distinctive features and should be evaluated separately in future clinical and biological studies.


Giulino-Roth L, Wang K, MacDonald TY, et al.
Targeted genomic sequencing of pediatric Burkitt lymphoma identifies recurrent alterations in antiapoptotic and chromatin-remodeling genes.
Blood. 2012; 120(26):5181-4 [PubMed] Article available free on PMC after 20/12/2013
To ascertain the genetic basis of pediatric Burkitt lymphoma (pBL), we performed clinical-grade next-generation sequencing of 182 cancer-related genes on 29 formalin-fixed, paraffin embedded primary pBL samples. Ninety percent of cases had at least one mutation or genetic alteration, most commonly involving MYC and TP53. EBV(-) cases were more likely than EBV(+) cases to have multiple mutations (P < .0001). Alterations in tumor-related genes not previously described in BL were identified. Truncating mutations in ARID1A, a member of the SWI/SNF nucleosome remodeling complex, were seen in 17% of cases. MCL1 pathway alterations were found in 22% of cases and confirmed in an expanded panel. Other clinically relevant genomic alterations were found in 20% of cases. Our data suggest the roles of MCL1 and ARID1A in BL pathogenesis and demonstrate that comprehensive genomic profiling may identify additional treatment options in refractory disease.


Al Mahmoud R, Weitzman S, Schechter T, et al.
Peripheral T-cell lymphoma in children and adolescents: a single-institution experience.
J Pediatr Hematol Oncol. 2012; 34(8):611-6 [PubMed]
BACKGROUND: Peripheral T-cell lymphoma (PTCL) is rare in pediatric patients and is associated with worse outcome compared with other pediatric non-Hodgkin lymphomas. We report our institutional experience over a 10-year period.
METHODS: Patients were identified through the institutional oncology database. Data were abstracted through a detailed retrospective review of patient charts. Those with isolated cutaneous T-cell lymphoma were excluded from this analysis.
RESULTS: Thirteen patients were diagnosed with PTCL during the 10-year period. All presented with advanced disease. According to the WHO PTCL classification, 7 patients had PTCL not otherwise specified, 3 had hepatosplenic T-cell lymphoma and 3 had T-cell posttransplant lymphoproliferative disease. Six of the patients had previously received or were receiving immunosuppressive therapy at the time of their diagnosis. Patients were treated with various chemotherapeutic regimens, including B-cell non-Hodgkin lymphomas and T-cell acute lymphoblastic leukemia-like therapy. Patients who had refractory or recurrent disease were changed to alternative therapy. Eight patients underwent stem cell transplantation, 7 allogeneic and 1 autologous. Of the 13 patients, 7 died. Three patients died from disease progression, 3 died from treatment-related mortality, and 1 patient died from cardiac rejection. Six patients (46%) are alive and disease free at a median of 21 months (2 to 79 mo).
CONCLUSIONS: Children with PTCL have an unfavorable outcome. A standard approach to management of pediatric PTCL needs to be established to improve outcome. Because of the rarity of this condition, this will require collaborative studies.


Minnicelli C, Barros MH, Klumb CE, et al.
Relationship of Epstein-Barr virus and interleukin 10 promoter polymorphisms with the risk and clinical outcome of childhood Burkitt lymphoma.
PLoS One. 2012; 7(9):e46005 [PubMed] Article available free on PMC after 20/12/2013
Epstein-Barr virus (EBV) is an important environmental factor associated to the development of Burkitt lymphoma (BL) in endemic and intermediate risk regions. However, little is known about the contribution of genetic constitution to the development and clinical response of the disease. The aim of this work was to investigate the role of EBV and Interleukin 10 (IL10) single nucleotide polymorphisms (-1082A/G, -819C/T, -592C/A) and microsatellites (IL10.R and IL10.G) in susceptibility and clinical outcome in pediatric BL patients, in a region with intermediate EBV association frequency. The frequencies of IL10 promoter Single nucleotide polymorphisms -1082A/G, -819C/T, -592C/A, and IL10.R and IL10.G microsatellites were compared in 62 pediatric patients and 216 healthy donors. IL10 -1082GG and GCC/GCC genotypes were more frequent in patients than in controls, and associated to a higher risk of BL development (GG genotype OR 2.62, 95% CI, 1.25-5.51; P = 0.008; Pc = 0.024). EBV was detected in tumor samples by EBER-ISH in 54.1% of cases. EBV+ patients exhibited a better event free survival (EFS) (P = 0.019) than EBV- patients. Carriers of IL10 R3-GCC had worse EFS (P = 0.028). Our results suggest a risk effect and an independent prognostic value of IL10 polymorphisms and EBV in childhood BL patients.


Hojsak I, Gagro A, Petković I, et al.
Chromosomal aberrations in peripheral blood lymphocytes in patients with newly diagnosed celiac and Crohn's disease.
Eur J Gastroenterol Hepatol. 2013; 25(1):22-7 [PubMed]
OBJECTIVES: The aims of this research were to determine the number of chromosomal aberrations in peripheral blood lymphocytes and to evaluate the number of circulating lymphocytes with CD103, integrin expressed on intraepithelial lymphocytes and preserved in enteropathy-associated T-cell lymphoma, in patients with newly diagnosed Crohn's disease, celiac disease, and healthy controls.
METHODS: During the period of 30 months, we included 44 patients. Chromosome aberrations were analyzed in peripheral blood lymphocytes by a single cytogeneticist. Multicolor flow cytometric was used for immunophenotyping of peripheral blood lymphocytes.
RESULTS: We found a significantly higher number of chromosomal aberrations/100 metaphases in the celiac and Crohn's disease group compared with the controls (P=0.01) and they also had a significantly higher number of aberrant cells compared with the controls (P<0.001). There was no statistically significant difference between the groups with respect to the percentage of CD103+ and CD8+CD103+ cells between groups (P=0.16 and 0.41, respectively) and no correlation between the total number of chromosomal aberrations and the percentage of CD103+ and CD8+CD103+ cells (P=0.06 and 0.06, respectively).
CONCLUSION: Patients with active celiac and newly diagnosed Crohn's disease, before treatment initiation, have a significantly increased number of chromosomal aberrations in peripheral blood lymphocytes. No dissemination of intraepithelial cells in the blood and correlation to the chromosomal aberration was found.


Tekkeşin F, Pınarlı FG, Kaya Z, et al.
High-risk ALK negative anaplastic large-cell lymphoma presenting with hypereosinophilic syndrome in a 2.5-year-old child.
Pediatr Hematol Oncol. 2012; 29(8):686-90 [PubMed]
The hypereosinophilic syndromes (HES) are characterized by prolonged nonreactive peripheral blood hypereosinophilia with tissue damage. The lymphocytic HES variant can precede malignant clonal T-cell disease in adults but it is extremely rare to be the presenting feature of lymphomas in children. Here we present a 2.5-year-old boy with HES and mediastinal T-cell anaplastic lymphoma kinase (ALK) negative systemic anaplastic large-cell lymphoma. Mature and immature eosinophils without blasts were shown on bone marrow aspiration while biopsy revealed malignant infiltration. The patient responded well to initial corticosteroid therapy, but high-risk features make a challenge of finding the cure in this extremely rare case.


Foyil KV, Bartlett NL
Brentuximab vedotin and crizotinib in anaplastic large-cell lymphoma.
Cancer J. 2012 Sep-Oct; 18(5):450-6 [PubMed]
Systemic anaplastic large-cell lymphoma (ALCL) is a rare, mature T-cell non-Hodgkin lymphoma. Anaplastic large-cell lymphoma cells express the surface antigen CD30, and more than half express the anaplastic lymphoma kinase (ALK) protein. These 2 proteins provide unique therapeutic targets in ALCL. Remission rates in ALCL with combination chemotherapy are approximately 80%, but relapse after first-line therapy is common. Brentuximab vedotin is a US Food and Drug Administration-approved, antibody-drug conjugate that combines an anti-CD30 antibody with monomethylauristatin E, a potent antimicrotubule agent. Response rates to brentuximab vedotin in patients with relapsed/refractory ALK and ALK ALCL have exceeded 80% with frequent complete responses and a median duration of response greater than 1 year. Brentuximab vedotin in combination with chemotherapy is being explored as a first-line therapy in ALCL. Crizotinib is an inhibitor of ALK tyrosine kinase that has been approved for the treatment of ALK non-small cell lung cancer. Successful treatment of ALK ALCL with crizotinib has been reported in pediatric patients and small case series leading to ongoing trials in relapsed/refractory ALCL. Brentuximab vedotin and crizotinib represent major advances in the treatment of ALK and ALK ALCL and will likely result in marked improvement in prognosis for this subset of aggressive lymphomas.


Cohen M, De Matteo E, Narbaitz M, et al.
Epstein-Barr virus presence in pediatric diffuse large B-cell lymphoma reveals a particular association and latency patterns: analysis of viral role in tumor microenvironment.
Int J Cancer. 2013; 132(7):1572-80 [PubMed]
Non-Hodgkin's lymphoma represents 6-10% of pediatric malignancies, and diffuse large B-cell lymphoma (DLBCL) is one of the three major subtypes. The 2008 WHO classification included a new entity, Epstein-Barr virus (EBV)-positive DLBCL of the elderly, affecting patients >50 years. It has been demonstrated that EBV may play a role in tumor microenvironment composition, disturbing antitumor immune response and disease progression. As most studies were performed in adults, our aim was to assess EBV presence and latency pattern, as well as T-cell microenvironment in a pediatric DLBCL series of Argentina. The study was conducted on formalin-fixed paraffin-embedded biopsies from 25 DLBCL patients. EBV-encoded small nuclear early regions (EBERs) expression was performed by in situ hybridization, whereas EBV gene expression was analyzed using real-time PCR. Epstein-Barr virus latent membrane proteins (LMP)1, LMP2A, CD3, CD4, CD8 and Foxp3 expression were assessed by immunohistochemistry (IHC). Forty percent of cases showed EBV expression, with a significantly higher incidence among patients <10 years (p = 0.018), and with immunosuppressed (p = 0.023). T-cell subsets were not altered by EBV presence. Full EBV latency antigen expression (latency type III) was the most frequently pattern observed, together with BZLF1 lytic gene expression. One patient showed II-like pattern (LMP1 without LMP2A expression). Based exclusively on IHC, some patients showed latency II/III (EBERs and LMP1 expression) or I (EBERs only). These findings suggest that EBV association in our series was higher than the previously demonstrated for elderly DLBCL and that EBV latency pattern could be more complex from those previously observed. Therefore, EBV could be an important cofactor in pediatric DLBCL lymphomagenesis.


Elli M, Dagdemir A, Bozkurt C, et al.
Serum osteopontin and CD44 levels in lymphoreticular malignancies in children.
Bratisl Lek Listy. 2012; 113(9):534-8 [PubMed]
OBJECTIVE: Osteopontin (OPN) is an adhesive glycoprotein that interacts with a variety of cell surface receptors, including several integrins and CD44. OPN is expressed and secreted by numerous human malignancies. CD44 play an important role in tumor growth and metastasis. We aimed to evaluate serum levels of osteopontin and CD44 in patients with lymphorethicular malignancies in childhood.
METHODS: We studied serum levels of CD44 and OPN levels of 54 patients (26, 18 and 10 patients with non-Hodgkin's lymphoma (NHL), Hodgkin's lymphoma (HL) and acute lymphoblastic leukemia (ALL), respectively) at the diagnosis.
RESULTS: The mean levels of OPN were significantly higher in patients (5.42±8.24 ng/ml) than in controls (3.89 ±1.96 ng/ml). The mean levels of CD44 levels were also significantly higher in patients (3.82±2.31 ng/ml) than in controls (1.96±0.62 ng/ml), and significantly higher in the advanced stages than in early stages. The mean levels of the CD44 in NHL, HL and ALL were 3.49±2.00, 3.56±1.74, and 5.15±3.50 respectively. OPN and CD44 levels were found to be increased in parallel (p=0.003). A more advanced disease and/or poor prognostic factors were seen in 9 patients who had both serum CD44 and OPN levels higher than 2SD of the control.
CONCLUSION: Elevated levels of both CD44 and OPN at the diagnosis may predict an unfavorable outcome in childhood leukemias and lymphomas (Tab. 2, Fig. 3, Ref. 44).


Pongpruttipan T, Cook JR, Reyes-Mugica M, et al.
Pulmonary extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue associated with granulomatous inflammation in a child with chromosome 22q11.2 deletion syndrome (DiGeorge syndrome).
J Pediatr. 2012; 161(5):954-8 [PubMed]
Patients with immunodeficiency disorders have an increased incidence of lymphoproliferative disorders; however, only 4 such patients with DiGeorge/chromosome 22q11.2 deletion syndrome have been reported. We report a case of a pulmonary Epstein-Barr virus-negative extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue in a child with this syndrome.


Ziade F, von der Weid N, Beck-Popovic M, Nydegger A
Burkitts's lymphoma--an atypical presentation.
BMC Pediatr. 2012; 12:113 [PubMed] Article available free on PMC after 20/12/2013
BACKGROUND: In female adolescents and young adults, malignancies of the genital tract are the most frequent type of cancer, closely followed by Hodgkin's and non-Hodgkin's lymphomas.
CASE PRESENTATION: We report an unusual case of sporadic Burkitt's lymphoma (BL) presenting with massive bilateral ovarian infiltration, peritoneal carcinomatosis and diffuse nodular lesions of the stomach and the intestine mimicking Krukenberg tumor. Diagnostic biopsies were obtained by endoscopy of the upper gastrointestinal tract. With intensive chemotherapy, complete remission was rapidly achieved, without life-threatening tumor lysis syndrome.
CONCLUSION: Besides metastatic gastric adenocarcinoma, BL is an important differential diagnosis in adolescents presenting with Krukenberg tumor.


Louissaint A, Ackerman AM, Dias-Santagata D, et al.
Pediatric-type nodal follicular lymphoma: an indolent clonal proliferation in children and adults with high proliferation index and no BCL2 rearrangement.
Blood. 2012; 120(12):2395-404 [PubMed]
Pediatric follicular lymphoma (PFL) is a variant of follicular lymphoma (FL) presenting as localized lymphadenopathy in children. Unlike conventional adult FL, PFL typically does not recur or progress. Clear diagnostic criteria for PFL are lacking, and it is uncertain whether this indolent lymphoma is defined by age or may occur in adults. We analyzed 27 FL in patients < 40 years of age and found that all 21 cases that lacked a BCL2 gene abnormality (BCL2-N; P < .0001) and had > 30% Ki67 fraction (high proliferation index, HPI; P = .0007) were stage I and did not progress or recur; in comparison, all 6 cases with BCL2 rearrangement and/or PI < 30% were stage III/IV, and 5 of 6 recurred or progressed. In a separate cohort of 58 adult FL (≥ 18 years of age), all 13 BCL2-N/HPI cases were stage I, and none progressed or relapsed, whereas 11 of 15 stage I cases with BCL2 gene abnormality and/or LPI relapsed or progressed (P = .0001). The adult and pediatric BCL2-N/HPI FL cases had similar morphologic features. Our results confirm the highly indolent behavior of PFL and suggest that these are characterized by HPI and absence of BCL2 gene abnormality. PFL-like cases also occur in adults and are associated with indolent behavior in this patient population.


Kolenova A, Mejstrikova E, Fidlerova K, et al.
Uncommon leukemic case of anaplastic large cell lymphoma diagnosed through a typical chromosomal abnormality t(2;5) with a null phenotype and aberrant expression of NG2 and CD13.
Bratisl Lek Listy. 2012; 113(7):404-8 [PubMed]
Anaplastic large cell lymphoma represents approximately 10-15 % of pediatric non-Hodgkin lymphomas. Leukemic presentation is very rare, and in particular, the null phenotype ALCL without typical anaplastic morphology together with aberrant expression of CD13 and/or CD11b represents a diagnostic challenge. We report a case of a 9 year-old boy with leukemic presentation of ALCL with the typical translocation t(2;5)(p23;q35); in this patient, the only positive antigens identified by immunophenotyping were CD13, NG2 HLA-DR, and CD38. To our knowledge, aberrant expression of NG2 has never been reported in ALCL cases (Tab. 1, Fig. 6, Ref. 20).


Wang YF, Yang YL, Gao ZF, et al.
Clinical and laboratory characteristics of systemic anaplastic large cell lymphoma in Chinese patients.
J Hematol Oncol. 2012; 5:38 [PubMed] Article available free on PMC after 20/12/2013
BACKGROUND: Systemic anaplastic large cell lymphoma (S-ALCL) is a rare disease with a highly variable prognosis and no standard chemotherapy regimen. Anaplastic lymphoma kinase (ALK) has been reported as an important prognostic factor correlated with S-ALCL in many but not all studies. In our study, we retrospectively analyzed 92 patients with S-ALCL from the Peking University Lymphoma Center for clinical and molecular prognostic factors to make clear the role of ALK and other prognostic factors in Han Chinese S-ALCL.
RESULTS: The majority of Chinese S-ALCL patients were young male patients (median age 26, male/female ratio 1.7) and the median age was younger than previous reports regardless of ALK expression status. The only statistically significant different clinical characteristic in S-ALCL between ALK positive (ALK+) and ALK negative (ALK-) was age, with a younger median age of 22 for ALK+ compared with 30 for ALK-. However, when pediatric patients (≤ 18) were excluded, there was no age difference between ALK+ and ALK-. The groups did not differ in the proportion of males, those with clinical stage III/IV (49 vs 51%) or those with extranodal disease (53 vs 59%). Of 73 evaluable patients, the 3-year and 5-year survival rates were 60% and 47%, respectively. Univariate analysis showed that three factors: advanced stage III/IV, lack of expression of ALK, and high Ki-67 expression, were associated with treatment failure in patients with S-ALCL. However, ALK expression correlated with improved survival only in patients younger than 14 years, while not in adult patients. In multivariate analysis, only clinical stage was an independent prognostic factor for survival. Expressions of Wilms tumor 1 (WT1) and B-cell lymphoma 2 protein (BCL-2) correlated with the expression of ALK, but they did not have prognostic significance. High Ki-67 expression was also a poor prognostic factor.
CONCLUSIONS: Our results show that ALK expression alone is not sufficient to determine the outcome of ALCL and other prognostic factors must be considered. Clinical stage is an independent prognostic factor. Ki-67 expression is a promising prognostic factor.


Nakatani K, Nakamoto Y, Watanabe K, et al.
Roles and limitations of FDG PET in pediatric non-Hodgkin lymphoma.
Clin Nucl Med. 2012; 37(7):656-62 [PubMed]
PURPOSE: The usefulness of 18F fluorodeoxyglucose (FDG) positron emission tomography (PET) in pediatric Hodgkin lymphoma (p-HL) has been well demonstrated; however, pediatric non-Hodgkin lymphoma (p-NHL) has distinct characteristics from p-HL and adult NHL. We assessed roles of FDG PET in p-NHL.
MATERIALS AND METHODS: Nineteen patients with p-NHL underwent 80 scans. Scans for staging (group A, n=6) and response assessment (group B, n=42) were compared with conventional imaging modalities (CIMs). Scans within group B for end-chemotherapy assessment (subgroup B+, n=11) and for post-therapeutic surveillance (group C, n=32) were analyzed for diagnostic performance.
RESULTS: In group A, PET and CIM demonstrated comparable results. In group B, PET diagnoses were concordant with CIM in 21 and discordant in 11 studies. Of the discordant cases, PET suggested remnant lesions in 5 cases, whereas CIM suggested lesions in 6 cases. PET modified therapeutic strategy in 4 cases by detecting new extranodal lesions. In subgroup B+, sensitivity, specificity, and accuracy for predicting relapse were 50%, 71%, and 64%, respectively. In group C, sensitivity, specificity, and accuracy were 100%, 87%, and 88%, respectively, but positive predictive value was 33%.
CONCLUSIONS: The role of FDG PET in p-NHL may be limited, unlike with p-HL or adult NHL. Nevertheless, FDG PET may serve complementarily in detecting unexpected lesions that can emerge in p-NHL.


Padmanabhan MY, Pandey RK, Kumar A, Radhakrishnan A
Dental management of a pediatric patient with Burkitt lymphoma: a case report.
Spec Care Dentist. 2012; 32(3):118-23 [PubMed]
Recent advancements in the field of cancer diagnosis and chemotherapy have led to higher rates of survival for children with malignant tumors. More than 75% of pediatric patients diagnosed with malignancies survive more than 5 years. These advancements have resulted in additional responsibilities for dental professionals to diagnose, evaluate, prevent, stabilize, and manage the oral and dental problems that can compromise the quality of life of these children. This case report highlights the importance of professional and self-administered oral prophylactic measures and patient compliance in the successful oral rehabilitation of a pediatric patient with Burkitt lymphoma.


Lee DW, Barrett DM, Mackall C, et al.
The future is now: chimeric antigen receptors as new targeted therapies for childhood cancer.
Clin Cancer Res. 2012; 18(10):2780-90 [PubMed]
Improved outcomes for children with cancer hinge on the development of new targeted therapies with acceptable short-term and long-term toxicity. Progress in basic, preclinical, and clinical arenas spanning cellular immunology, gene therapy, and cell-processing technologies have paved the way for clinical applications of chimeric antigen receptor-based therapies. This is a new form of targeted immunotherapy that merges the exquisite targeting specificity of monoclonal antibodies with the potent cytotoxicity, potential for expansion, and long-term persistence provided by cytotoxic T cells. Although this field is still in its infancy, clinical trials have already shown clinically significant antitumor activity in neuroblastoma, chronic lymphocytic leukemia, and B-cell lymphoma, and trials targeting a variety of other adult and pediatric malignancies are under way. Ongoing work is focused on identifying optimal tumor targets and elucidating and manipulating both cell- and host-associated factors to support expansion and persistence of the genetically engineered cells in vivo. In pediatric oncology, CD19 and GD2 are compelling antigens that have already been identified for targeting pre-B acute lymphoblastic leukemia and neuroblastoma, respectively, with this approach, but it is likely that other antigens expressed in a variety of childhood cancers will also soon be targeted using this therapy. The potential to target essentially any tumor-associated cell-surface antigen for which a monoclonal antibody can be made opens up an entirely new arena for targeted therapy of childhood cancer.


Ferreira JM, Klumb CE, de Souza Reis R, et al.
Lymphoma subtype incidence rates in children and adolescents: first report from Brazil.
Cancer Epidemiol. 2012; 36(4):e221-6 [PubMed]
BACKGROUND: Lymphoma is the third most common pediatric malignancy. The purpose of this study was to analyze the incidence rates of lymphoma in children and adolescents in Brazil.
METHODS: All cases of Hodgkin lymphoma (HL), non-Hodgkin lymphoma (NHL), and Burkitt lymphoma (BL) were extracted from 14 population-based cancer registries (PBCRs) from 2000 to 2005, and included children and adolescents 0-19 years old. Analyses included age-adjusted incidence rates (AAIRs) and age-specific incidence rates (ASIRs) by each PBCR. A social exclusion index (SEI) was built and used as proxy for socioeconomic status (SES) levels. Correlations between SES and incidence rates were investigated using Spearman's test.
RESULTS: The median incidence of lymphoma was 22.7/million. AAIRs of lymphomas varied from 12.9 (Salvador) to 34.5 per million (São Paulo). Median AAIR was 8.8/million, 9.8/million, and 2.9/million for NHL, HL, and BL, respectively. In all PBCRs except that of Recife, AAIR was slightly higher in males than females. The median ASIR was highest for HL (18.5/million) at 15-19 years for both genders. For NHL there were two peaks for ASIR: 11.1/million (1-4 years of age) and 13.2/million (15-19 years of age). The median ASIR for BL was highest among children aged 1-4 years (4.7/million) and in males. Higher SEI correlated with higher incidence of HL (P = 0.06), whereas rates of NHL and BL did not correlate with SEI. Borderline different incidence rates were observed in HL correlated with cities with higher SEIs.
CONCLUSION: Incidence rates of lymphomas in Brazil do not differ compared to rates reported worldwide, although SES differences deserve further investigation.


Pleasant L, Skinner S, Pulliam J, et al.
Burkitt lymphoma in a child with atopic dermatitis and a 7-year history of regular topical tacrolimus use.
Cutis. 2012; 89(3):117-20 [PubMed]
We describe the case of a boy who presented with abdominal Burkitt lymphoma; he had been regularly using tacrolimus ointment 0.1% for severe recurrent atopic dermatitis for 7 years immediately prior to developing cancer. We present his medical history and review the current knowledge regarding a link between topical tacrolimus and malignancy risk.


Gaiser T, Geissinger E, Schattenberg T, et al.
Case report: A unique pediatric case of a primary CD8 expressing ALK-1 positive anaplastic large cell lymphoma of skeletal muscle.
Diagn Pathol. 2012; 7:38 [PubMed] Article available free on PMC after 20/12/2013
Primary involvement of skeletal muscle is a very rare event in ALK-1 positive anaplastic large cell lymphoma (ALCL). We describe a case of a 10-year old boy presenting with a three week history of pain and a palpable firm swelling at the dorsal aspect of the left thigh. Histological examination of the lesion revealed a tumoral and diffuse polymorphic infiltration of the muscle by large lymphoid cells. Tumor cells displayed eccentric, lobulated "horse shoe" or "kidney-shape" nuclei. The cells showed immunohistochemical positivity for CD30, ALK-1, CD2, CD3, CD7, CD8, and Perforin. Fluorescence in situ hybridization analysis revealed a characteristic rearrangement of the ALK-1 gene in 2p23 leading to the diagnosis of ALK-1 positive ALCL. Chemotherapy according to the ALCL-99-NHL-BFM protocol was initiated and resulted in a complete remission after two cycles. This case illustrates the unusual presentation of a pediatric ALCL in soft tissue with a good response to chemotherapy.


Thakral C, Hutchison RE, Shrimpton A, et al.
ALK+ anaplastic large cell lymphoma exhibits phosphatidylinositol-3 kinase/Akt activity with retained but inactivated PTEN--a report from the Children's Oncology Group.
Pediatr Blood Cancer. 2012; 59(3):440-7 [PubMed] Article available free on PMC after 01/09/2013
BACKGROUND: ALK+ anaplastic large cell lymphoma (ALCL) is usually a disease of young patients. We investigated phosphatidylinositol-3 kinase (PI3K)/Akt pathway-associated factors in pediatric cases and cell lines.
PROCEDURE: Patient materials consisted of tissue slides of ALK+/CD30+ ALCL from 33 patients treated on Pediatric Oncology Group protocols (9219, n = 8 and 9315, n = 25). Slides were examined by immunohistochemistry for phospho(p)-Akt and PTEN, the primary feedback regulator of the pathway, as well as for p27kip1 and stathmin-1. ALCL cell lines SUDHL-1 and Karpas-299 were examined for ALK, pALK, pAkt, p27/Kip1, PTEN, pPTEN, CD30, pSTAT3, and pSTAT5; ALK inhibition was performed using compound PF-2341066 and PTEN genes were sequenced.
RESULTS: A majority of patients expressed pAkt, PTEN, and stathmin, with p27kip1 levels less than controls. Cell lines showed expression of ALK, pALK, pSTAT3, pSTAT5, CD30, pAkt, PTEN, and pPTEN, with p27 slightly less than positive controls, and germline PTEN DNA. There was evidence of phosphorylated PTEN (pPTEN) associated with inhibited function. Pharmacologic inhibition of activated ALK diminished pSTAT3, pSTAT5, and CD30 expression but not pAkt or pPTEN in cultured cell lines.
CONCLUSION: We conclude that the PI3K/Akt pathway is activated in many, though not all, pediatric ALK+ ALCL. Our data suggest that activation of this pathway involves post-translational regulation of PTEN. Pharmacologic inhibition of activated ALK does not reduce modest levels of activated Akt as it does with the more abundant levels of activated STAT3 or STAT5. Future therapy of ALCL might, in selected patients, best combine agents inhibiting PI3K/Akt with those targeting ALK.


Mbulaiteye SM, Anderson WF, Ferlay J, et al.
Pediatric, elderly, and emerging adult-onset peaks in Burkitt's lymphoma incidence diagnosed in four continents, excluding Africa.
Am J Hematol. 2012; 87(6):573-8 [PubMed] Article available free on PMC after 01/06/2013
Burkitt's lymphoma (BL) in the general population and immunosuppressed persons with AIDS in the United States was characterized by three age-specific incidence peaks near 10, 40, and 70 years. We hypothesized that BL from different geographical areas may exhibit pediatric, adult, and elderly age incidence peaks. We investigated this hypothesis using data on 3,403 cases obtained from the International Agency for Research on Cancer (1963-2002). Data from Africa were sparse or incomplete, and thus were excluded. Age-standardized rates (ASRs) and age-specific incidence rates were calculated, supplemented with the calculations performed using age-period-cohort (APC) models. The ASR rose 5.3% (95% confidence interval [CI], 5.0-5.6) per year in males and 4.6% (95% CI, 4.5-4.8) in females. The ASR increased gradually in children, steeply in adults and most rapidly in the elderly both in males and in females. Overall, BL male/female ASR ratio was 2.5, but it declined from 3.1 (95% CI, 3.0-3.3) for pediatric BL to 2.3 (95% CI, 2.2-2.4) for adult BL and 1.5 (95% CI, 1.4-1.6) for elderly BL. Age-specific incidence peaks occurred near 10 and 70 years in all regions and periods. A peak near 40 years of age emerged in the mid-1990s, particularly in men. Findings using APC models confirmed those based on the standard analyses. Our findings, based on the international BL cases, support our hypothesis that BL is multimodal and that BL peaks at different ages may be clues to differences in the etiology and/or biology of BL at those ages.


Chakrabarti B, Bhaduri B, Barik S, et al.
Primary ovarian lymphoma in a child.
J Indian Med Assoc. 2011; 109(9):679-80 [PubMed]
Ovarian tumour is rarely seen in paediatric age group and primary ovarian non-Hodgkin's lymphoma is very uncommon. A child presenting with acute abdomen was detected to have bilateral solid ovarian tumours. Because of torsion of the right ovarian tumour and complete replacement of the ovarian tissue by the tumour on left side, bilateral salpingo-oophorectomy was done. Bilateral primary ovarian lymphoma was diagnosed on histopathological examination. Immunohistochemistry was suggestive of B-cell lineage. She had eight cycles of chemotherapy and at 28 months follow-up doing well without any sign of any recurrence or any evidence of metastasis.


Naithani R, Ngan BY, Roifman C, et al.
Thymic mucosa-associated lymphoid tissue lymphoma in an adolescent girl.
J Pediatr Hematol Oncol. 2012; 34(7):552-5 [PubMed]
Mucosa-associated lymphoid tissue (MALT) lymphoma is very rare in children. We report the first case of pediatric thymic MALT lymphoma in an adolescent Asian girl. She presented with chest pain, dyspnea, and low-grade fever. A large anterior mediastinal mass was biopsied that confirmed the diagnosis of MALT lymphoma with trisomy 18. The patient had secondary immunodeficiency with low NK cell count and high IgA and IgG levels. Because of the advanced stage and the presence of trisomy 18, she was treated with cyclophosphamide, vincristine, prednisone, and rituximab, followed by involved-field radiotherapy. She is currently undergoing maintenance therapy with rituximab and remains in complete remission at 13 months from diagnosis. Thymic MALT lymphoma should be suspected in any Asian child with a cystic thymic mass and autoimmune disease or hyperglobinemia. Because of the slow proliferation rate of this type of lymphoma, a long-term follow-up is needed.


Liu W, Ren J, Shu Q
Aggressive sinonasal natural killer/T-cell lymphoma mimicking refractory sinusitis in a 4-year-old boy.
Fetal Pediatr Pathol. 2012; 31(5):288-94 [PubMed]
Pediatric patients with nasal symptoms are common, and most of them usually have inflammatory diseases, such as sinusitis, chronic rhinitis, nasal polyp, or adenotonsillar hypertrophy. Rarely, however, these inflammatory symptoms may be associated with more sinister pathology. Recently, we experienced a case of a 4-year-old boy with sinonasal natural killer (NK)/T-cell lymphoma whose initial symptoms were of nasal obstruction and mucopurulent nasal discharge.


Mellgren K, Hedegaard CJ, Schmiegelow K, Müller K
Plasma cytokine profiles at diagnosis in pediatric patients with non-hodgkin lymphoma.
J Pediatr Hematol Oncol. 2012; 34(4):271-5 [PubMed]
Non-Hodgkin lymphoma (NHL) has been associated with elevated levels of inflammatory and immune-regulating cytokines, and polymorphisms in the genes encoding interleukin (IL)-10 and tumor necrosis factor (TNF)-α have been associated with increased incidence of certain subtypes of NHL. The aim of the present study was to screen for a broader spectrum of growth factors and inflammatory mediators and to compare the profiles in different subtypes of NHL in pediatric patients. Serum samples were collected at diagnosis from 31 pediatric patients diagnosed with NHL admitted at Rigshospitalet, Copenhagen, between 1995 and 2008. Cytokines and growth factors were measured in serum using the Luminex platform by application of a 30-plex kit. Levels of IL-6, IL-2R, IL-10, TNF-RI, and macrophage inflammatory protein-1α were significantly higher in patients with anaplastic large-cell lymphoma compared with patients diagnosed with B-cell lymphomas and lymphoblastic lymphomas. High levels of IL-4, IL-13, TNF-RI, and epidermal growth factor were associated with a poorer general condition at diagnosis. The present study suggests that NHL subgrouping and the general condition of pediatric patients at diagnosis are associated with plasma levels of growth factors and inflammatory mediators at presentation.


Ravikumar G, Tirumalae R, Das K
Primary pleural non-Hodgkin lymphoma in a child--an exceedingly rare disease.
J Pediatr Surg. 2012; 47(3):e29-31 [PubMed]
Primary pleural lymphomas are very rare. Two types are described in the literature: primary effusion lymphoma, in the setting of human immunodeficiency virus infection, and pyothorax-associated lymphomas, with a strong Epstein-Barr virus association. We report a rare case of a primary pleural lymphoma in a 12-year-old immunocompetent girl who presented with a hemorrhagic pleural effusion and had plaque-like thickening of the pleura. The histologic and immunophenotypic findings conformed to that of a diffuse large B-cell lymphoma (CD20 positive).


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