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Types of Lymphoma > Childhood Lymphomas

Last update: 03/12/2014

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Overview | Types | Treatment Information | Clinical Trials | Resources & Research News

TOPICS SEARCH: PubMed: Diagnosis | Review | Therapies | Prognosis 

Investigational therapies for childhood lymphomas:
All phases | Phase I | Phase II | Phase III

Overview of Childhood Lymphomas

Lymphoma is rare in children:

Incidence and Age Distribution of Specific Types of Childhood NHL per million person-years  cancer.gov

Prognosis is very good for most types:

JAMA 2010: Differences in Non-Hodgkin Lymphoma Survival Between Young Adults and Children jamanetwork.com

Selected snips:

"Significant progress in survival from cancer has been achieved in the United States in the past 30 years, especially in childhood cancer for which the 5-year relative survival rate has increased from around 60% in the 1970s to currently more than 80%.

For young adults, however, progress is more difficult to characterize because cancer rates for this population are often combined with overall adult cancer rates.

Children were defined as those aged 0 to 19 years at diagnosis and young adults were defined as those aged 20 to 29 at diagnosis.

Among the 2,442 persons younger than 30 years diagnosed with NHL from 1992 to 2001,
40% were children aged 0 to 19 years and
60% were young adults (aged 20-29 years) (Table 1).

More males than females were diagnosed with NHL (66% vs 34%, respectively; P = .02).

For all cases, 12% were non-Hispanic black and 21% were Hispanic; there were no differences in race/ethnicity distribution by age group.

Thirty-three percent of all persons were diagnosed with stage I disease,
20% with stage II,
11% with stage III, and
36% with stage IV.

There were modest differences in stage distribution by age group (P = .02).

A higher proportion of young adults were diagnosed at stage I (35%) and a lower percentage were diagnosed at stage IV (34%) compared with childhood NHL cases (30% and 38%, respectively).

Among all persons,
75% had aggressive subtypes of NHL,
11% had indolent subtypes of NHL, and
14% had unspecified subtypes of NHL.

There were clear differences between children and young adults by histologic subtype of NHL
(P ≤ .001).

Nearly half (49%) of young adults were diagnosed with diffuse large B-cell lymphoma, compared with 28% of children.

After diffuse large B-cell lymphoma, the other most common histologic subtypes of NHL diagnosed in young adults were
the indolent subtype (16%) and
not otherwise specified (16%);

among children, the other common subtypes were
lymphoblastic (24%) and
Burkitt (22%) lymphomas. "

Also See
Pediatric Non-Hodgkin Lymphoma
emedicine.medscape.com/

 

The above posted to lymphomation.org

 

About Lymphoma

Overview of genes and cancer

Lymphoma is a cancer

About Lymphoma - general

Characteristics of NHL
  Cell type | Histology | Grading | Staging

 Ann Arbor Staging 
  Extranodal notations 

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Extranodal (beyond nodal) sites indicates that the disease has extended beyond the lymph system. 
Also see 
Extranodal Lymphomas
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Childhood Lymphomas

Approximately 60% of pediatric lymphomas are non-Hodgkin's lymphomas (NHL), with the remainder being Hodgkin's lymphomas. Extranodal involvement (outside the lymph system) at diagnosis is common.  Lymphoma is the third most common childhood cancer. (SEER Cancer Statistics Review.)

The prognosis and survival for all the subtypes of childhood NHL has improved dramatically, despite the fact that it is commonly diagnosed at an advanced stage. 

What is lymphoma?

Lymphoma is a blood cell cancer affecting lymphocytes -- a type of white blood cell that help to fight infection.  It occurs when this type of cell is damaged in ways that affect how the cells grows and survive.

Blood cell development; drawing shows the steps a blood stem cell goes through to become a red blood cell, platelet, or white blood cell. A myeloid stem cell becomes a red blood cell, a platelet, or a myeloblast, which then becomes a granulocyte (the types of granulocytes are eosinophils, basophils, and neutrophils). A lymphoid stem cell becomes a lymphoblast and then becomes a B-lymphocyte, T-lymphocyte, or natural killer cell.... The damaged cells reproduce and do not die off as do normal lymphocytes. The abnormal cells accumulate and eventually form tumors, most typically in the lymphatic system (lymph nodes, bone marrow, spleen) but also in other parts of the body such as the skin.


In the News

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Anaplastic large cell lymphoma - Clinical Oncology News: Study Shows Brentuximab Also Effective in Kids
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Pediatric Blood and Cancer: Gonadal function and parenthood 20 years after treatment for childhood lymphoma: A cross-sectional study

Childhood lymphomas usually fall into
four broad categories: 

Common Subtypes of
Childhood Lymphoma
(rare lymphomas)
Incidence
within
childhood
types
Current 5-year
survival estimates
 
Small non-cleaved cell lymphoma

Burkitt's and non-Burkitt's 
 
~ 40%
 90 to 95%
 
Lymphoblastic Lymphoma
 
~30%
85 to 90%
Large B- cell Lymphoma (B-LCL)
~20%
80 to 90%
  
 
Anaplastic large cell lymphoma (ALCL)
~10%
100%
event free survival in recent report for early stage disease * 

but also

Pediatric Hodgkins Lymphoma

   

* Children with ALCL respond well to treatment but are susceptible to relapse [4a]

Most common areas of extranodal presentation are:
bullet

head & neck

bullet

abdomen

bullet

chest

Incidence and prognosis

The diagnosis of a lymphoma in children is rare:

"There are about 500 new cases of non-Hodgkin's lymphoma diagnosed each year in kids in the United States. It may occur at any age during childhood, but is rare before age 3. NHL is slightly more common than Hodgkin's disease in kids younger than 15 years old." 1

"In children with NHL, 5-year survival is about 90% for those with Stage I or Stage II at the time of diagnosis, and close to 70% for those with more advanced Stage III or IV disease."  1
 

  1. Childhood Cancer: Lymphoma kidshealth.org 
  2. The impact of age and gender on biology, clinical features and treatment outcome of non-Hodgkin lymphoma in childhood and adolescence.  
    Br J Haematol. 2005 Oct;131(1):39-49. PMID: 16173961
  3. The incidence of pediatric lymphomas is low - SEER: http://seer.cancer.gov/publications/childhood/lymphomas.pdf

Recommended resources:

bullet
AMA 2010 full text:
Differences in Non-Hodgkin Lymphoma Survival Between Young Adults and Children jamanetwork.com
bullet
Pediatric Non-Hodgkin Lymphoma emedicine.medscape.com/
bullet
Outcomes in poor countries:
Pediatric B-cell lymphoma - by subtype
http://bit.ly/buMFH8
bullet
Children's Oncology Group
(800) 458-6223 (Toll Free, U.S. and Canada)

Centers by state: curesearch.org
bullet
Guidance for Parents on Childhood cancers by NCI  
Large PDF file
bullet
A parent's guide to children's cancer  CancerBACUP
bullet
Childhood Non-Hodgkin's Lymphoma  Cancer.gov | indianpediatrics.net
bullet
About (all types)  KidHealth | lymphomainfo.net 
bullet
Cellular Classification  Cancer.gov
bullet
Mortality: improving trend  FDA.gov

 

Childhood Small non-cleaved cell lymphoma
 - Burkitt's or non-Burkitt's

Here we will provide links to resources and news specific to this subtype of childhood lymphoma.  

Also review the Resources & Research News and Clinical Trials sections below.

RESOURCES
bullet
About Stage I and II  - includes standard &  investigational options  Cancer.gov 
bullet
About Stage III and IV  - includes standard &  investigational options  Cancer.gov 
bullet
Childhood Non-Hodgkin Lymphoma Treatment (PDQ) 
Last Modified: 2/12/2008  Health Professional Version Cancer.gov 

Cellular Classification and Clinical Presentation
        Burkitt and Burkitt-like lymphoma/leukemia
        Diffuse large B-cell lymphoma
        Lymphoblastic lymphoma
        Anaplastic large cell lymphoma
        Lymphoproliferative disease associated with immunodeficiency in children
        Rare non-Hodgkin lymphoma occurring in children

OUTCOMES:

bullet
Long-term results of the first Italian Association of Pediatric Hematology and Oncology protocol 
for the treatment of pediatric B-cell non-Hodgkin lymphoma (AIEOP LNH92).
Cancer. 2004 Jul 15;101(2):385-94. PMID: 15241838
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Non-Hodgkin's lymphoma: treatment and outcome of children with advanced disease  Gan To Kagaku Ryoho. 1999 Jul;26(8):1050-5. Review. Japanese. PMID: 10431576  PubMed
  
"In recent years, the results of treating children with advanced non-Hodgkin's lymphomas have improved markedly. Among patients with small non-cleaved cell lymphoma (both Burkitt's and Burkitt-like lymphomas according to the Revised European American Lymphoma Classification) in particular, about 80% could be cured by a short intensive polychemotherapy containing cyclophosphamide, high-dose methotrexate, and high-dose cytarabine."
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Long term survival of children with Burkitt lymphoma in Malawi after cyclophosphamide monotherapy. Med Pediatr Oncol. 2003 Jan;40(1):23-5. PMID: 12426682  PubMed

Childhood Lymphoblastic Lymphoma

Here we will provide links to resources and news specific to this subtype of childhood lymphoma.

 Also review the Resources & Research News and Clinical Trials sections below.

RESOURCES

bullet
Getting closer to curing pediatric acute lymphoblastic lymphoma http://bit.ly/35rUl3 
bullet
Disseminated Childhood Lymphoblastic Lymphoma (for healthcare professionals)  Cancer.gov
bullet
About Stage 1 and II - includes includes standard &  investigational options  Cancer.gov 
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About Stage III and IV - includes includes standard &  investigational options  Cancer.gov 
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A Comparison of Allogeneic and Autologous Bone Marrow Transplant for Lymphoblastic Lymphoma. Blood. 2002 Nov 27 [epub ahead of print] PMID: 12456505  PubMed

 

Childhood Large B-cell Lymphoma

Here we will provide links to resources and news specific to this subtype of childhood lymphoma. 

Also review the Resources & Research News and Clinical Trials sections below.

RESOURCES
bullet
About Stage 1 and II - includes includes standard &  investigational options  cancer.gov 
bullet
About Stage III and IV - includes includes standard &  investigational options  cancer.gov 
bullet
Childhood and adolescent large-cell lymphoma (LCL): A review of the children's cancer group experience. 
Am J Hematol. 2003 Jan;72(1):53-63.  PMID: 12508269  PubMed

 

Childhood Anaplastic large cell lymphoma (ALCL)

Also see Anaplastic large cell lymphoma

Here we will provide links to resources and news specific to this subtype of childhood lymphomas. 

Also review the Resources & Research News and Clinical Trials sections below.

RESOURCES

bullet
About Stage 1 and II - includes includes standard &  investigational options  cancer.gov 
bullet
About Stage III and IV Ki+ anaplastic large cell lymphoma  cancer.gov
bullet
Childhood Anaplastic large cell lymphoma  PubMed abstracts
bullet
ASCO 2003 - SGN-30 is a genetically engineered antibody in clinical trials for the treatment of patients with CD30+ hematologic malignancies such as Hodgkin's disease, anaplastic large cell lymphoma and other types of lymphomas.  ASCO
bullet
A population-based study of pediatric anaplastic large cell lymphoma. 
Cancer. 2002 Mar 15;94(6):1830-5. PMID: 11920547 PubMed
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Pediatric anaplastic large cell lymphoma; results of a randomized phase III pediatric oncology group trial of APO versus APO+intermediate dose methotrexate/high dose ARA-C (POG #9315)  ASCO 2003 | Slides asco.org
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Anaplastic large cell lymphoma in childhood: analysis of 72 patients treated on The United Kingdom Children's Cancer Study Group chemotherapy regimens. Br J Haematol. 2002 Jun;117(4):812-20. Review. PMID: 12060115  PubMed
Treatments
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Treatment

Improvements in prognosis and survival for childhood lymphomas have resulted primarily from use of multi-agent chemotherapies. Recently "More recent shorter and intense therapy appears to be associated with superior event-free survival." [2]

Use of Rituxan in cd-20 positive childhood lymphomas is under investigation and is considered to have potential to improve outcomes even more.
 
bullet
General Principles of Treatment  ncbi.nlm.nih.gov/books 
bullet
ASCO 2003  Pediatric Lymphomas
bullet
Questions for your doctor  Patients Against Lymphoma
General | Treatment & Side Effects | Tests

Clinical Trials
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Clinical Trials

See Studies for in ClinicalTrials.gov
Resources & Research News
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Resources & Research News

  1. Childhood and adolescent large-cell lymphoma (LCL): A review of the children's cancer group experience. Am J Hematol. 2003 Jan;72(1):53-63.
    PMID: 12508269  PubMed
     
    " In summary, more recent shorter and intense therapy appears to be associated with superior event-free survival for children and adolescents with disseminated LCL. Large numbers of patients treated with shorter and intense therapy are required to confirm these preliminary observations."
  2. Abdominal presentation of B-cell non-Hodgkin's lymphoma (B-NHL) - surgical treatment and its results. Report of the Polish Paediatric Leukaemia/Lymphoma Study Group Med Wieku Rozwoj. 2000;IV(1 Suppl 2):57-66. Polish. PMID: 12021463  PubMed
  3. Improved treatment results of non-Hodgkin's lymphoma in children: a report from the Children's Cancer and Leukemia Study Group of Japan.
    Int J Hematol. 1995 Feb;61(2):85-96. PMID: 7734716  PubMed
  4. Non-Hodgkin's lymphoma arising in bone in children and adolescents is associated with an excellent outcome: a Children's Cancer Group report.
    J Clin Oncol. 2002 May 1;20(9):2293-301. PMID: 11981000  PubMed
  5. Guidance for Parents on Childhood cancers by NCI  -  Large PDF file | PDF-Help
  6. A parent's guide to children's cancer  CancerBACUP
  7. Natural Killer Lymphoma/Leukemia: An Uncommon Pediatric Case with Indolent Course. Leuk Lymphoma. 2004;45(8):1687-1689. PMID: 15370226 | Related articles
  8. Pediatric follicular lymphomas, marginal zone lymphomas, and marginal zone hyperplasia. Am J Clin Pathol. 2004 Dec;122 Suppl:S98-109. Review. PMID: 15690646 | Related articles
  9. Primary Non-Hodgkin's Lymphoma of Bone in Children  ejbjs.org 
  10. Prognostic factors in childhood anaplastic large cell lymphoma : results of a large European Intergroup Study. Blood. 2007 Oct 23; PMID: 17957029

    ... three factors associated with an increased risk of failure in childhood ALCL have been defined: mediastinal involvement, visceral involvement and skin lesions.
 
Disclaimer:  The information on Lymphomation.org is not intended to be a substitute for 
professional medical advice or to replace your relationship with a physician.
For all medical concerns,  you should always consult your doctor. 
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