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).

Hayase T, Niijima H, Tanaka D, et al.
Successful allogeneic bone marrow transplantation with reduced intensity conditioning regimen for a pediatric relapsed ALK positive anaplastic large cell lymphoma.
Rinsho Ketsueki. 2016; 57(7):859-63 [PubMed] Related Publications
Pediatric anaplastic large cell lymphoma (ALCL) is a chemosensitive malignancy, but about 30% of patients experience relapse. In most of these patients, a second complete remission is obtainable with salvage chemotherapy, though relapse free survival rates are as low as 30-60%. Herein, we report a 6-year-old boy with relapsed anaplastic lymphoma kinase (ALK) positive ALCL successfully treated with vinblastine monotherapy followed by allogeneic hematopoietic stem cell transplantation (allo-HSCT), with a reduced intensity conditioning (RIC) regimen, from his father. One HLA locus from the father was mismatched. The boy had neither severe graft-versus-host disease nor transplantation related complications. He is currently well and has remained disease free for 10 months, to date, since transplantation. Allo-HSCT with a RIC regimen may be a promising treatment strategy for relapsed ALK positive ALCL based on obtaining graft-versus lymphoma effects as well as reducing transplantation-related mortality.

Porpaczy E, Mayerhoefer M, Salzer-Muhar U, Jaeger U
Lymphoma in Danon disease with chronic rhabdomyolysis treated with EPOCH-R: A case report.
Medicine (Baltimore). 2016; 95(29):e4237 [PubMed] Free Access to Full Article Related Publications
Rare disorders often represent a challenge for clinicians and require close collaboration of an interdisciplinary team.We present the complex case of a 22-year-old male with Danon disease and late-onset of posttransplant lymphoproliferative disorder after heart transplantation. The critical aspects of his condition were: pre-existing rhabdomyolysis; infiltration of muscle and gut with lymphoma; advanced clinical stage with bulky disease; nonresponsiveness to the reduction of immunosuppression and rituximab monotherapy; expected cardiotoxicity of anthracyclines. Therefore, the patient was treated with the EPOCH-R protocol, which includes continuous administration of doxorubicin over 4 days, instead of R-CHOP, in which the anthracycline is given in a short single infusion. Complete remission was achieved after the third cycle; rhabdomyolysis did not increase and heart function was not affected. The patient received a total of 6 cycles and is still in metabolic complete remission.We conclude that patients with Danon disease can be treated with anthracycline-containing chemotherapy and that continuous infusion of EPOCH-R does not exacerbate pre-existing rhabdomyolysis.

Related: Cyclophosphamide Doxorubicin Etoposide Rituximab (Mabthera) Vincristine

Burkhardt B, Yavuz D, Zimmermann M, et al.
Impact of Fc gamma-receptor polymorphisms on the response to rituximab treatment in children and adolescents with mature B cell lymphoma/leukemia.
Ann Hematol. 2016; 95(9):1503-12 [PubMed] Related Publications
Recent studies in adult lymphoma patients have indicated a correlation between polymorphisms of Fc gamma-receptors (FcγRs, encoded by the respective FCGR genes) and the response to rituximab treatment. In vitro, cells expressing FcγRIIIa-158V mediate antibody-dependent cellular cytotoxicity (ADCC) more efficiently than cells expressing FcγRIIIa-158F. The impact of the FCGR2A-131HR polymorphism is unclear. In this study, the FCGR polymorphisms FCGR3A-158VF and FCGR2A-131HR were analyzed in pediatric patients with mature aggressive B cell non-Hodgkin lymphoma/leukemia (B-NHL). Pediatric patients received a single dose of rituximab monotherapy. Response was evaluated on day 5 followed by standard chemotherapy for B-NHL. Among 105 evaluable patients, a response to rituximab was observed in 21 % of those homozygous for FcγRIIa-131RR (5/24) compared to 48 % of patients who were HH and HR FcγRIIa-131 allele carriers (18/34 and 21/47, respectively; p = 0.044). Among patients with the FCGR3A-158 polymorphism, those homozygous for the FF genotype had a significantly favorable rituximab response rate of 59 % (22/37) compared to 32 % in patients who were FcγRIIIa-158VV and FcγRIIIa-VF allele carriers (2/9 and 20/59, respectively; p = 0.022). A stringent phase II response evaluation of children and adolescents with B-NHL after one dose of rituximab monotherapy showed a significant association between the rituximab response rate and FCGR polymorphisms. These findings support the hypothesis that FCGR polymorphisms represent patient-specific parameters that influence the response to rituximab.

Related: Rituximab (Mabthera)

Mellgren K, Attarbaschi A, Abla O, et al.
Non-anaplastic peripheral T cell lymphoma in children and adolescents-an international review of 143 cases.
Ann Hematol. 2016; 95(8):1295-305 [PubMed] Related Publications
Peripheral T cell lymphomas (PTCL) are rare in children and adolescents, and data about outcome and treatment results are scarce. The present study is a joint, international, retrospective analysis of 143 reported cases of non-anaplastic PTCL in patients <19 years of age, with a focus on treatment and outcome features. One hundred forty-three patients, between 0.3 and 18.7 years old, diagnosed between 2000 and 2015 were included in the study. PTCL not otherwise specified was the largest subgroup, followed by extranodal NK/T cell lymphoma, hepatosplenic T cell lymphoma (HS TCL), and subcutaneous panniculitis-like T cell lymphoma (SP TCL). Probability of overall survival (pOS) at 5 years for the whole group was 0.56 ± 0.05, and probability of event-free survival was (pEFS) 0.45 ± 0.05. Patients with SP TCL had a good outcome with 5-year pOS of 0.78 ± 0.1 while patients with HS TCL were reported with 5-year pOS of only 0.13 ± 0.12. Twenty-five percent of the patients were reported to have a pre-existing condition, and this group had a dismal outcome with 5-year pOS of 0.29 ± 0.09. The distribution of non-anaplastic PTCL subtypes in pediatric and adolescent patients differs from what is reported in adult patients. Overall outcome depends on the subtype with some doing better than others. Pre-existing conditions are frequent and associated with poor outcomes. There is a clear need for subtype-based treatment recommendations for children and adolescents with PTCL.

Maciejka-Kemblowska L, Chaber R, Wrobel G, et al.
Clinical features and treatment outcomes of peripheral T-cell lymphoma in children. A current data report from Polish Pediatric Leukemia/Lymphoma Study Group (PPLLSG).
Adv Med Sci. 2016; 61(2):311-316 [PubMed] Related Publications
PURPOSE: Peripheral T-cell lymphomas (PTCL) are lymphoproliferative disorders derived from post-thymic cells, that occur extremely rarely in children. The optimal treatment of pediatric PTCL remains still unclear.
PATIENTS AND METHODS: Ten children with PTCL from 3 up to 18 years of age registered by the Polish Pediatric Leukemia/Lymphoma Study Group (PPLLSG) were retrospectively analyzed. All patients were treated with different regimens including protocols: for lymphoblastic lymphoma in 7 cases, for anaplastic large cell lymphoma in 1, CHOP in 1. Five of the 10 patients with PTCL were classified as stage II; 4 as stage III and 1 as stage IV due to extralymphatic organs (bone marrow) involvement. Four histological subtypes of PTCL were recognized: extranodal NK/T-cell lymphoma, nasal type (ENTNT), peripheral T-cell lymphoma not otherwise specified (PTCL-NOS), subcutaneous panniculitis-like T-cell lymphoma (SPL), Sezary syndrome (SS). After first-line therapy 9 patients initially achieved complete remission, 4 relapsed, 5 died. One patient achieved remission spontaneously. Three children (1 with stage IV and 2 in relapse) underwent high-dose chemotherapy with allogeneic bone marrow stem cell transplantation and all of them are alive and in CR.
RESULTS: The cumulative probability of 5-year overall survival (OS) for our whole group was 63.9% (95%CI: 35.2-88.2%) with a median follow-up time of 48.4 months (range 24-90+ months). The 5-year event free survival (EFS) was 81%. PTCLs are a heterogeneous and rare group of childhood NHLs.
CONCLUSIONS: According to our experience the standard chemotherapy for precursor lymphomas seems to be a beneficial treatment option for children with PTCL. Allogeneic stem cell transplantation may improve the outcome in selected patients.

Related: Leukemia Childhood Leukaemia Leukemia - Molecular Biology

Wieser I, Wohlmuth C, Nunez CA, Duvic M
Lymphomatoid Papulosis in Children and Adolescents: A Systematic Review.
Am J Clin Dermatol. 2016; 17(4):319-27 [PubMed] Related Publications
BACKGROUND: Lymphomatoid papulosis (LyP) is a lymphoproliferative disorder that is rare among adults and even rarer among children. In adults, LyP is associated with an increased risk of secondary lymphomas.
OBJECTIVE: The aim of this systematic review was to describe the clinical and histopathological features of LyP in children, to assess the risk of associated lymphomas, and to compare the disease to the adult form.
METHODS: A systematic review was conducted using the MEDLINE (PubMed), EMBASE, Scopus, and Cochrane databases from inception to June 2015. Articles were included if data were extractable from studies, case series, and single reports of pediatric LyP patients.
RESULTS: A total of 251 children and adolescents with LyP were identified, with the mean age at diagnosis being 9.3 ± 4.6 years (n = 187). The female to male ratio was 1:1.4, and the majority of children reported on were Caucasian (n = 74, 85.1 %). The predominant histologic subtype was type A (n = 106, 79.1 %). Clinically, LyP lesions presented as erythematous papules or nodules, appearing preferentially on the extremities and the trunk. LyP has to be differentiated from pityriasis lichenoides (PL) and primary cutaneous anaplastic large cell lymphoma (ALCL). PL and associated lymphomas were diagnosed before, with, and after LyP in 19 and 14 cases, respectively. Of the 14 subjects with associated lymphomas, two children developed systemic ALCL.
CONCLUSION: LyP has to be differentiated from ALCL to avoid erroneous treatments. Due to the increased risk of development of non-Hodgkin lymphomas, lifelong follow-up and proper patient counseling are warranted.

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Campuzano-Zuluaga G, Ortiz D, Peng JH, et al.
Primary Mediastinal Large B-Cell Lymphoma With Translocations Involving BCL6 and MYC (Double-Hit Lymphoma).
Am J Clin Pathol. 2016; 145(5):710-6 [PubMed] Related Publications
OBJECTIVES: Primary mediastinal large B-cell lymphomas (PMLBCLs) are aggressive lymphomas with characteristic clinical, morphologic, and immunophenotypic features. "Double-hit" (DH) lymphomas are B-cell neoplasms characterized by a translocation involving MYC and either BCL2 or BCL6 In the indexed literature, there are no reported cases of PMLBCL associated with DH or triple-hit events.
METHODS: Herein, we present a case of a 15-year-old girl with PMLBCL who had typical clinical, morphologic, and immunophenotypic features.
RESULTS: Fluorescent in situ hybridization studies showed rearrangements involving MYC and BCL6 We also excluded the possibility of a reciprocal t(3;8) (3q27;8q24) BCL6/MYC translocation.
CONCLUSIONS: This case expands the current spectrum of lymphomas subtypes in which DH can be found and supports the rationale for cytogenetic testing for DH abnormalities in all cases of aggressive large B-cell lymphomas regardless of subtype.

Related: FISH BCL6 MYC

Bhaskar MK, Ojha B, Jaiswal M, Sagar M
Extra-axial primary non-Hodgkin's CNS lymphoma mimicking meningioma, in a 5-year-old immunocompetent child: a rare entity.
BMJ Case Rep. 2016; 2016 [PubMed] Related Publications
We describe a case of a 5-year-old immunocompetent girl who presented with features of raised intracranial pressure with left eye ptosis of 1-month duration. CT scan and MRI of the brain showed an extra-axial, intensely contrast enhancing lesion in the left temporoparieto-occipital region, consistent with meningioma. On open tissue biopsy and immunohistochemistry staining, a diagnosis of B cell non-Hodgkin's lymphoma was made. Six cycles of chemotherapy with cyclophosphamide, adriamycin, vincristine and prednisolone regimen were given and showed a good clinical outcome without any recurrence during follow-up of 5 months.

Related: Brain and Spinal Cord Tumours Primary CNS Lymphoma Brain and CNS Tumors

ter Haar E, Labarque V, Tousseyn T, Mekahli D
Severe acute kidney injury as presentation of Burkitt's lymphoma.
BMJ Case Rep. 2016; 2016 [PubMed] Related Publications
We discuss a case of acute kidney injury (AKI) at a very young age caused by primary lymphomatous renal infiltration due to Burkitt's lymphoma and analyse the literature on this rare condition. At presentation, clinical examination showed impressive bilateral nephromegaly and hypertension. Blood analysis indicated severe AKI, mild anaemia and normal serum electrolytes. There were no signs of tumour lysis syndrome. Urine sediment was normal, with neither haematuria nor proteinuria. Abdominal ultrasound demonstrated bilateral renal enlargement (+12 SD), with increased corticomedullar differentiation. MRI demonstrated the presence of a homogenous renal enlargement with features of an infiltrative lesion. Ultimately, microscopic and immunohistochemical analysis of the renal biopsy confirmed the diagnosis of Burkitt's lymphoma. Early and aggressive therapy is the key to ensure a good outcome.

Zah E, Lin MY, Silva-Benedict A, et al.
T Cells Expressing CD19/CD20 Bispecific Chimeric Antigen Receptors Prevent Antigen Escape by Malignant B Cells.
Cancer Immunol Res. 2016; 4(6):498-508 [PubMed] Article available free on PMC after 01/06/2017 Related Publications
The adoptive transfer of T cells expressing anti-CD19 chimeric antigen receptors (CARs) has shown remarkable curative potential against advanced B-cell malignancies, but multiple trials have also reported patient relapses due to the emergence of CD19-negative leukemic cells. Here, we report the design and optimization of single-chain, bispecific CARs that trigger robust cytotoxicity against target cells expressing either CD19 or CD20, two clinically validated targets for B-cell malignancies. We determined the structural parameters required for efficient dual-antigen recognition, and we demonstrate that optimized bispecific CARs can control both wild-type B-cell lymphoma and CD19(-) mutants with equal efficiency in vivo To our knowledge, this is the first bispecific CAR capable of preventing antigen escape by performing true OR-gate signal computation on a clinically relevant pair of tumor-associated antigens. The CD19-OR-CD20 CAR is fully compatible with existing T-cell manufacturing procedures and implementable by current clinical protocols. These results present an effective solution to the challenge of antigen escape in CD19 CAR T-cell therapy, and they highlight the utility of structure-based rational design in the development of receptors with higher-level complexity. Cancer Immunol Res; 4(6); 498-508. ©2016 AACR

Related: Cytokines

Robaina MC, Faccion RS, Mazzoccoli L, et al.
miR-17-92 cluster components analysis in Burkitt lymphoma: overexpression of miR-17 is associated with poor prognosis.
Ann Hematol. 2016; 95(6):881-91 [PubMed] Related Publications
Burkitt lymphoma (BL) is an aggressive B cell lymphoma characterized by the reciprocal translocation of the c-Myc gene with immunoglobulin genes. Recently, MYC has been shown to maintain the neoplastic state via the miR-17-92 microRNA cluster that suppresses chromatin regulatory genes and the apoptosis regulator Bim. However, the expression and prognostic impact of miR-17-92 members in pediatric BL (pBL) are unknown. Therefore, we investigated miR-17, miR-19a, miR-19b, miR-20, and miR-92a expression and prognostic impact in a series of 41 pBL samples. In addition, Bim protein expression was evaluated and compared to miR-17, miR-19a, miR-19b, miR-20, and miR-92a levels and patient outcomes. The expression of miR-17-92 members was evaluated by qPCR and Bim protein by immunohistochemistry. Log-rank test was employed to assess prognostic impact. We found that upregulated expression of miR-17 and miR-20a correlates with lack of pro-apoptotic Bim expression. Patients bearing tumors with upregulated miR-17 displayed decreased overall survival (OS), and multivariate analysis revealed that miR-17 was a significant predictor of shortened OS. Using hairpin inhibitors, we showed that inhibition of miR-17 resulted in enhanced Bim expression in a BL cell line overexpressing the miR-17-92 cluster. Our results describe for the first time miR-17, miR-19a, miR-19b, miR-20a, and miR-92a expression profiles in pBL. The prognostic impact of miR-17 should be validated in a larger series, and may provide new therapeutic avenues in the era of anti-miRNA therapy research. Additional functional studies are further required to understand the specific role of miR-17-92 cluster members in BL.

Related: MicroRNAs

Grasso D, Borreggine C, Ladogana S, et al.
Sporadic Burkitt's lymphoma/acute B-cell leukaemia presenting with progressive proptosis and orbital mass in a child.
Neuroradiol J. 2016; 29(3):231-5 [PubMed] Article available free on PMC after 01/06/2017 Related Publications
Burkitt's lymphoma (BL) is an aggressive B-cell non-Hodgkin lymphoma that is found predominantly in children, with the highest incidence occurring in Africa. The sporadic form occurs in non-endemic areas and typically involves the ileo-caecum and the bowel, whereas orbital and paranasal sinus involvement is rare. Here, we present an unusual case of sporadic BL in a Caucasian male child with rapidly progressive painful proptosis of the right eye. Magnetic resonance imaging showed an oval-shaped, extraconal mass in the supero-lateral part of the right orbit that deformed and dislocated the eyeball antero-inferiorly. The patient underwent anterior orbitotomy, and a biopsy of the excised tissue revealed a starry-sky appearance characteristic of BL. Postoperative aggressive chemotherapy was initiated with a good response after one week.

Leiba M, Leiba A, Keinan-Boker L, et al.
Adolescent weight and height are predictors of specific non-Hodgkin lymphoma subtypes among a cohort of 2,352,988 individuals aged 16 to 19 years.
Cancer. 2016; 122(7):1068-77 [PubMed] Related Publications
BACKGROUND: The age-adjusted annual incidence of non-Hodgkin lymphoma (NHL) has risen worldwide. This trend may be affected by the secular increase in height and the sharp upswing in adolescent overweight; these drive increased insulinlike growth factor 1 and chronic inflammation, which may play an etiologic role. This study examined the association of the body mass index (BMI) and height of adolescents with NHL subtypes, which have been insufficiently evaluated.
METHODS: Health-related data on 2,352,988 Israeli adolescents, aged 16 to 19 years, who were examined between 1967 and 2011 were linked to the Israel National Cancer Registry to derive the NHL incidence up to December 31, 2012 (4021 cases). Cox proportional hazards modeling was used to estimate the multivariate-adjusted hazard ratio (HR) for NHL subtypes associated with the BMI and height of adolescents.
RESULTS: Adolescent overweight and obesity were associated with an HR of 1.25 (95% confidence interval [CI], 1.13-1.37; P = 1.14 × 10(-5) ) for NHL in comparison with normal weight. There was a graded association of height with NHL (P = 4.29 × 10(-9) ), with the tallest adolescents (≥ 95th percentile vs 25th to < 50th percentiles [US Centers for Disease Control and Prevention]) exhibiting an HR of 1.28 (95% CI, 1.04-1.56). Marginal zone lymphoma, primary cutaneous lymphoma (PCL), and diffuse large B-cell lymphoma (DLBCL) showed the strongest associations for overweight/obesity, and DLBCL and PCL showed the strongest associations for height.
CONCLUSIONS: The findings of this large cohort study add to the growing body of evidence showing that higher body weight and taller stature during adolescence are associated with an increased risk of NHL and may modestly contribute to its increasing incidence. Further studies are needed to elucidate the mechanisms linking anthropometric measures and NHL risk.

Ceppi F, Weitzman S, Woessmann W, et al.
Safety and efficacy of intrathecal rituximab in children with B cell lymphoid CD20+ malignancies: An international retrospective study.
Am J Hematol. 2016; 91(5):486-91 [PubMed] Related Publications
Central nervous system (CNS) involvement in patients with mature B non-Hodgkin lymphoma, post-transplantation proliferative disorder and acute lymphoblastic leukemia confers a significantly inferior prognosis as compared to patients without CNS disease. Intrathecal (IT) or intraventricular administration of rituximab is an option for this group of patients. We report 25 children with CNS involvement of CD20+ B lymphoid malignancies who received in total 163 IT/intraventricular rituximab doses. The median number of doses received by each patient was 6, with a median dose of 25 mg. The most common adverse events were Grades 1 and 2 peripheral neuropathies in five patients (20%), allergy in two patients, and headache in two patients. These events were self-limited, occurring in the 48 hours after treatment and resolving within 24 hr. Three patients presented with more severe though transient side effects, one with a Grade III neuropathy and two with seizure. Eighteen patients (72%) of those treated with IT/intraventricular rituximab, with or without other CNS directed treatment, achieved a CNS remission. This case series suggests that IT/intraventricular rituximab has therapeutic efficacy and relatively limited toxicity. Prospective trials of IT/intraventricular rituximab for patients with CNS involvement of CD20 + B lymphoid malignancies are warranted.

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Ghoshal A, Salins N, Damani A, et al.
Medical Management of Pediatric Malignant Bowel Obstruction in a Patient with Burkitt's Lymphoma and Ataxia Telangiectasia Using Continuous Ambulatory Drug Delivery System.
J Pain Palliat Care Pharmacother. 2016; 30(1):44-8 [PubMed] Related Publications
Malignant bowel obstruction (MBO) is commonly seen in patients with advanced abdominal cancers. The incidence of pediatric MBO in a patient with Burkitt's lymphoma and ataxia telangiectasia is rare, with no published case reports till now. Conservative management of inoperable MBO results in relief of symptoms and improves quality of life. An 11-year-old boy with Burkitt's lymphoma and ataxia telangiectasia was referred to pediatric palliative care with MBO. The objective of this report is to demonstrate conservative management of pediatric MBO using continuous ambulatory drug delivery system. The patient was initiated on continuous ambulatory drug delivery (CADD) system for symptom relief. MBO was reversed with conservative management and the child was discharged on self-collapsible portable elastomeric continuous infusion pump under the supervision of a local family physician. The child remained comfortable at home for 4 weeks until his death. His parents were satisfied with the child's symptom control, quality of life, and were able to care for the child at home. In a resource-limited setting, managing patients at home using elastomeric continuous infusion pumps instead of expensive automated CADD is a practical pharmacoeconomic approach.

Related: Ataxia-Telangiectasia Ataxia Telangiectasia

Meznarich J, Miles R, Paxton CN, Afify Z
Pediatric B-Cell Lymphoma With Lymphoblastic Morphology, TdT Expression, MYC Rearrangement, and Features Overlapping With Burkitt Lymphoma.
Pediatr Blood Cancer. 2016; 63(5):938-40 [PubMed] Related Publications
Burkitt lymphoma (BL) and B-lymphoblastic lymphoma are subtypes of pediatric non-Hodgkin lymphoma with different presenting features, treatment, and outcomes. This case report documents a 5-year-old female who presented with B-cell lymphoma with lymphoblastic morphology, terminal deoxynucleotidyl transferase expression, MYC rearrangement, and features overlapping with BL. Genomic microarray analysis identified a gain on the long arm of chromosome 1 without other definitive changes. She was treated according to a BL protocol and remains in remission 16-months after initial diagnosis.

Related: Chromosome 1 MYC

Fedhila-Ben Ayed F, Douira-Khomsi W, Rhayem S, et al.
Burkitt lymphoma in a child with Bloom syndrome.
Arch Pediatr. 2016; 23(4):382-4 [PubMed] Related Publications
BACKGROUND: Bloom syndrome is a rare disease characterized by chromosomal instability and increased risk of developing lymphoma.
OBSERVATION: We report on a case of Bloom syndrome in a 5-year-old boy with Burkitt lymphoma. The diagnosis was suspected by growth retardation, repeated respiratory infections, facial telangiectasia, and a low immunoglobulin level, then confirmed cytogenetically by sister chromatid exchanges. Chemotherapy was poorly tolerated, which required reducing the doses. Unfortunately, it was not sufficient to control the neoplasm and the patient died 14 months after diagnosis.
CONCLUSIONS: Cancers in Bloom syndrome are a challenge since the potentially life-threatening side effects of the chemotherapy may require modifications in standard treatment such as dose reduction, which can compromise the tumor prognosis.

Related: Bloom Syndrome

Sharapova SO, Chang EY, Guryanova IE, et al.
Next generation sequencing revealed DNA ligase IV deficiency in a "developmentally normal" patient with massive brain Epstein-Barr virus-positive diffuse large B-cell lymphoma.
Clin Immunol. 2016; 163:108-10 [PubMed] Related Publications
INTRODUCTION: Here we present an unusual case of DNA ligase IV deficiency syndrome without dysmorphic facial findings and microcephaly complicated with Epstein-Barr virus-associated large B-cell lymphoma with the right lung involvement and a massive brain tumor lesion in a two-year-old female.
METHODS: PID panel was used for sequencing 55 genes. Most genes have >98% exon coverage including splicing sites. LIG4 gene has 100% exon and splicing site coverage. This was used in Ion Torrent PGM system, the library kit was made by Agilent with Haloplex technology. The sequence analysis software was Alamut, direct sequencing of LIG4 gene was performed after NGS results.
RESULT: We identified three heterozygous mutations in LIG4 gene c.2736+3delC and c.8 C>T (p.A3V) inherited from mother and c.26C>T (p.T9I) - from father after PID panel sequencing and some additional polymorphisms in ATM, NOD2 and NLRP3 genes.
CONCLUSION: This case broadens the clinical spectrum of DNA ligase IV deficiency.

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Gupta V, Arava S, Bakhshi S, et al.
Subcutaneous Panniculitis-Like T-Cell Lymphoma with Hemophagocytic Syndrome in a Child.
Pediatr Dermatol. 2016 Mar-Apr; 33(2):e72-6 [PubMed] Related Publications
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) characterized by subcutaneous infiltration of pleomorphic T-cells of the α/β phenotype rarely affects children. Development of hemophagocytic syndrome (HPS) leads to a poor prognosis with this otherwise indolent lymphoma. We report a case of SPTCL in a 5-year-old child complicated by HPS treated successfully with combination chemotherapy. We discuss the potential pitfalls in reaching an early diagnosis and challenges in its management. Previously reported cases of SPTCL with HPS in children are briefly reviewed.

Barrett MM, Strikwerda AM, Somers K, et al.
Lymphomatoid Papulosis Type D: Report of a Case in a Child and Review of the Literature.
Pediatr Dermatol. 2016 Mar-Apr; 33(2):e52-6 [PubMed] Related Publications
Lymphomatoid papulosis (LyP) is a cutaneous CD30-positive T-cell lymphoproliferative disorder that occurs primarily in adults and presents with crops of papules that become necrotic and spontaneously regress. It is classified according to the histopathologic findings; currently recognized subtypes include A, B, C, D, and E. LyP is uncommon in children. Herein we describe a child with an unusual clinical presentation of LyP type D and review the literature of reported cases in children.

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Bălănescu RN, Bălănescu L, Cordoş I, et al.
Unusual finding of a mediastinal T-cell lymphoma in a 13-year-old patient - a case report.
Rom J Morphol Embryol. 2015; 56(4):1541-3 [PubMed] Related Publications
T-cell lymphoblastic lymphoma is an aggressive malignancy that represents 85% of all lymphoblastic lymphomas. It usually occurs in late childhood, adolescence and young adulthood with a 2:1 male preponderance and it presents with pleural effusion and respiratory symptoms and in rare cases vena cava syndrome can be encountered. We present the case of a 13-year-old patient who was referred to our clinic from a local hospital where he was diagnosed with a mediastinal tumor. The patient presented with thoracic pain, fever, coughing and fatigability for a month prior to admission, after having underwent surgery for abdominal pain (appendectomy). On admission to our hospital, a thoracic computed tomography (CT) scan was performed and showed the presence of an anterior mediastinal mass measuring 109/76/140 mm, well defined, which came in close contact with the superior vena cava, the ascending aorta and the pulmonary artery, right pleural effusion and a collapsed lung on the right side. The decision was taken to perform a tumor biopsy and a right pleural drain was placed. The patient's post-operative evolution was favorable with the remission of the respiratory symptoms. The histopathological result showed the presence of T-cell lymphoblastic lymphoma and the patient was then transferred to the oncology ward where he underwent chemotherapeutic treatment, with a favorable outcome. T-cell lymphoblastic lymphoma is an aggressive type of lymphoma and it is usually hard to diagnose considering the fact that the symptoms are often vague. It is essential to establish the diagnosis without delay and start appropriate chemotherapeutic treatment.

Shaver AC, Zimmerman D, Liu M, et al.
Concurrent and Clonally Related Pediatric Follicular Lymphoma and Burkitt Lymphoma in a 5-Year-Old Boy.
Lab Med. 2016; 47(1):43-7 [PubMed] Related Publications
Pediatric follicular lymphoma shares morphologic similarities with the adult form of the disease but lacks other classic features of adult lymphoma, including t(14;18) translocation, BCL2 overexpression, and transformation to aggressive higher-grade lymphoma. Herein, we report a novel case in which a 5-year-old boy (ethnicity unknown) had follicular lymphoma, along with concurrent high-grade and clonally related disease that fulfilled all of the morphologic, immunophenotypic, and genetic criteria for Burkitt lymphoma, including a t(8;14) translocation involving the MYC gene. To our knowledge, this case is the first reported instance of transformation of follicular lymphoma of any sort into true Burkitt lymphoma and the first reported instance of acquisition of MYC abnormalities in pediatric follicular lymphoma.

Mendiratta V, Gandhi N, Rana S, et al.
Isolated cutaneous involvement in a child with nodal anaplastic large cell lymphoma.
Indian J Dermatol Venereol Leprol. 2016 Jan-Feb; 82(1):53-6 [PubMed] Related Publications
Non-Hodgkin lymphoma is a common childhood T-cell and B-cell neoplasm that originates primarily from lymphoid tissue. Cutaneous involvement can be in the form of a primary extranodal lymphoma, or secondary to metastasis from a non-cutaneous location. The latter is uncommon, and isolated cutaneous involvement is rarely reported. We report a case of isolated secondary cutaneous involvement from nodal anaplastic large cell lymphoma (CD30 + and ALK +) in a 7-year-old boy who was on chemotherapy. This case is reported for its unusual clinical presentation as an acute febrile, generalized papulonodular eruption that mimicked deep fungal infection, with the absence of other foci of systemic metastasis.

Related: Cyclophosphamide Epirubicin Vincristine

O'Suoji C, Welch JJ, Perkins SL, et al.
Rare Pediatric Non-Hodgkin Lymphomas: A Report From Children's Oncology Group Study ANHL 04B1.
Pediatr Blood Cancer. 2016; 63(5):794-800 [PubMed] Related Publications
BACKGROUND: Non-Hodgkin lymphoma (NHL) is a relatively common malignancy in pediatric patients; however, a small subgroup have unusual lymphoma subtypes for the pediatric population.
PROCEDURE: The Children's Oncology Group Rare and Cutaneous NHL registry's (protocol ANHL 04B1) main objectives were to determine the pathologic, biologic, and clinical features of rare and cutaneous pediatric NHL and establish a bank of centrally reviewed tissue specimens. We report the clinical data, treatment data, and outcome for rare pediatric NHL.
RESULTS: In 101 lymphomas, there is a 97.8% concordance between the reviewing study pathologists and an 87.6% concordance between the central and institutional pathology review. Samples in the specimen bank include primary tumor tissue that is snap frozen, in paraffin blocks, or H&E-stained and unstained paraffin slides as well as blood, serum, and bone marrow. This descriptive analysis shows that children with pediatric follicular lymphoma, mucosa-associated lymphoid tissue, nodal marginal zone lymphoma, primary cutaneous, primary central nervous system lymphoma, and subcutaneous panniculitis-like T-cell lymphomas have 100% survival at a median of 2 years from enrollment. There are early deaths, mostly from progressive disease, in subjects with peripheral T-cell (not otherwise specified), NKT, and hepatosplenic T-cell lymphomas.
CONCLUSIONS: This registry provides high-quality biologic specimens with clinical data to investigators working on the biology of these unusual pediatric diseases.

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McEachron TA, Kirov I, Wungwattana M, et al.
Successful Treatment of Genetically Profiled Pediatric Extranodal NK/T-Cell Lymphoma Targeting Oncogenic STAT3 Mutation.
Pediatr Blood Cancer. 2016; 63(4):727-30 [PubMed] Related Publications
Extranodal natural killer (NK)/T-cell lymphoma (ENKTCL) is a distinct type of non-Hodgkin lymphoma predominantly observed in Asian and Latin American adult males. A 12-year-old Hispanic female diagnosed with ENKTCL was enrolled in our genomic profiling research protocol. We identified specific somatic alterations consistent with diagnosis of ENKTCL as well as oncogenic mutations in MAP2K1 and STAT3. To our knowledge, this is the first report of an immunophenotypically confirmed and genetically profiled case of ENKTCL in a female pediatric patient in the United States, including its unique treatment and favorable outcome.

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Meleshko AN, Kharuzhyk SA, Piatrouskaya NA
Response monitoring in follicular lymphoma by synchronous evaluation of minimal residual disease and diffusion-weighted MRI.
Exp Oncol. 2015; 37(4):295-7 [PubMed] Related Publications
AIM: To demonstrate quantitative assessment of tumor response to treatment in patients with follicular lymphoma using parallel monitoring of minimal residual disease (MRD) and diffusion-weighted MRI (MRI-DWI) derived apparent diffusion coefficient (ADC).
MATERIALS AND METHODS: Two patients with follicular lymphoma were undergone synchronous evaluation of MRD and MRI-DWI at definite time points before, during and after chemotherapy. MRD level was calculated in diagnostic and follow up samples relative to the highest level of amplification of the target. Allele-specific primer for clonal IgH gene rearrangement was used as a target for real-time quantitative polymerase chain reaction (PCR). 1.5 Tesla scanner was used for MRI-DWI. The largest non necrotic lymph node was chosen for serial ADC measurement.
RESULTS: In first patient MRD reduced drastically in blood after chemotherapy but persisted at low level in bone marrow. Whole body MRI-DWI demonstrated regression of most of tumor lesions except one -marginally enlarged iliac lymph node and allowed to predict tumor progression in this particular anatomical site based on low ADC value after treatment. In the second patient all three methods (MRD, ADC and radiologic evaluation) gave concordant result of complete tumor response and patient remained in a clinical remission during follow-up time.
CONCLUSION: Quantitative PCR measurement may detect very low level of MRD in patients with follicular lymphoma which may have prognostic value. Combination of both PCR-based MRD and quantitative evaluation of MRI-DWI derived ADC provides safe added-value disease monitoring in lymphoma.

Bedekovics T, Hussain S, Feldman AL, Galardy PJ
UCH-L1 is induced in germinal center B cells and identifies patients with aggressive germinal center diffuse large B-cell lymphoma.
Blood. 2016; 127(12):1564-74 [PubMed] Article available free on PMC after 24/03/2017 Related Publications
Gene expression profiling has identified 2 major subclasses of diffuse large B-cell lymphoma (DLBCL). Cases resembling germinal center (GC) B cells (GCB-DLBCL) generally occur in younger patients, have a distinct molecular pathophysiology, and have improved outcomes compared with those similar to activated post-GC cells (activated B-cell DLBCL). We previously found that the ubiquitin hydrolase UCH-L1 is frequently overexpressed in mature B-cell malignancies and is a potent oncogene in mice. The cause for its overexpression in lymphoma, and whether it impacts the outcome of patients with DLBCL is unknown. Here, we show that UCH-L1 reflects GC lineage in lymphoma and is an oncogenic biomarker of aggressive GCB-DLBCL. We find that UCH-L1 is specifically induced in GC B cells in mice and humans, and that its expression correlates highly with the GCB subtype in DLBCL. We also find that UCH-L1 cooperates with BCL6 in a mouse model of GC B-cell lymphoma, but not with the development of multiple myeloma derived from post-GC cells. Despite the typically good outcomes of GCB-DLBCL, increased UCHL1 identifies a subgroup with early relapses independent of MYC expression, suggesting biological diversity in this subset of disease. Consistent with this, forced Uchl1 overexpression had a substantial impact on gene expression in GC B cells including pathways of cell cycle progression, cell death and proliferation, and DNA replication. These data demonstrate a novel role for UCH-L1 outside of the nervous system and suggest its potential use as a biomarker and therapeutic target in DLBCL.

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Chapuy B, Roemer MG, Stewart C, et al.
Targetable genetic features of primary testicular and primary central nervous system lymphomas.
Blood. 2016; 127(7):869-81 [PubMed] Article available free on PMC after 24/03/2017 Related Publications
Primary central nervous system lymphomas (PCNSLs) and primary testicular lymphomas (PTLs) are extranodal large B-cell lymphomas (LBCLs) with inferior responses to current empiric treatment regimens. To identify targetable genetic features of PCNSL and PTL, we characterized their recurrent somatic mutations, chromosomal rearrangements, copy number alterations (CNAs), and associated driver genes, and compared these comprehensive genetic signatures to those of diffuse LBCL and primary mediastinal large B-cell lymphoma (PMBL). These studies identify unique combinations of genetic alterations in discrete LBCL subtypes and subtype-selective bases for targeted therapy. PCNSLs and PTLs frequently exhibit genomic instability, and near-uniform, often biallelic, CDKN2A loss with rare TP53 mutations. PCNSLs and PTLs also use multiple genetic mechanisms to target key genes and pathways and exhibit near-uniform oncogenic Toll-like receptor signaling as a result of MYD88 mutation and/or NFKBIZ amplification, frequent concurrent B-cell receptor pathway activation, and deregulation of BCL6. Of great interest, PCNSLs and PTLs also have frequent 9p24.1/PD-L1/PD-L2 CNAs and additional translocations of these loci, structural bases of immune evasion that are shared with PMBL.

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Aboobaker S, Tinley C
Primary Orbital Lymphomatoid Granulomatosis in a 1-Year-Old Child.
Middle East Afr J Ophthalmol. 2015 Oct-Dec; 22(4):517-9 [PubMed] Article available free on PMC after 24/03/2017 Related Publications
Lymphomatoid granulomatosis (LyG) is a rare, angiocentric, angiodestructive, B-cell lymphoproliferative disease, presenting with pulmonary involvement in more than 80% of cases. We describe a case of primary orbital LyG in a 1-year-old child.

Awasthi S, Rothe MJ, Eichenfield LF
Atopic dermatitis: Kids are not just little people.
Clin Dermatol. 2015 Nov-Dec; 33(6):594-604 [PubMed] Related Publications
The approach to children and adults with atopic dermatitis is similar. In both age groups, failure to respond to conventional therapy should prompt evaluation for complicating factors such as secondary infection and secondary ACD. Immunologic, metabolic, genetic, and nutritional disorders should be considered in the differential diagnosis of refractory pediatric atopic dermatitis. Cutaneous T cell lymphoma (CTCL), cutaneous drug reactions, other spongiotic dermatoses, psoriasis, dermatomycosis, and infestations should be considered in the differential of refractory atopic dermatitis in adults. Systemic therapies prescribed to both children and adults with severe atopic dermatitis include oral corticosteroids, cyclosporine, methotrexate, azathioprine, and mycophenolate mofetil.

Related: Cutaneous T-cell lymphoma

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