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About
Lymphoma > Types of Lymphoma > Childhood
Lymphomas
Last update: 03/19/2008
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Investigational therapies for childhood lymphomas:
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II | Phase
III
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Overview of Childhood
Lymphomas
Extranodal
(beyond nodal) sites indicates that the disease has extended beyond the
lymph system.
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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? Briefly, lymphomas result when DNA
damage or changes occurs to an immune cell (a lymphocyte) that
alters the behavior of the cells. The damage to DNA results in the
abnormal production of proteins that prevents the cells from dying
when they should, or causes sustained rapid cell division that
produces more of its kind. These malignant cells then may accumulate
to form tumors that may enlarge the lymph nodes or spread to other
areas of the lymphatic system,
such as the spleen or bone marrow. Lymphoma can also spread or first
appear outside the lymphatic system -- and is called extranodal
disease.
Childhood lymphomas usually fall into four broad categories:
Common Subtypes of Childhood Lymphoma
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Incidence
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Current 5-year survival estimates
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~ 40%
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90 to 95%
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~30%
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85 to 90%
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~20%
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80 to 90%
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~10%
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100% event free survival in recent report for early stage
disease *
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* Children with ALCL respond well to
treatment but are susceptible to relapse [4a]
Most common areas of extranodal
presentation are:
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head & neck |
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abdomen |
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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
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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
Recommended resources:
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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:
| About Stage I
and II - includes standard & investigational
options Cancer.gov
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| About Stage III
and IV - includes standard & investigational
options Cancer.gov
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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
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Childhood Lymphoblastic Lymphoma |
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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:
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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
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Childhood Large B-cell Lymphoma |
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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:
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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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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
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Childhood Anaplastic
large cell lymphoma (ALCL) |
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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:
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About Stage 1
and II - includes includes standard &
investigational options - cancer.gov
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About Stage III and IV Ki+ anaplastic large cell lymphoma - Cancer.gov
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Childhood Anaplastic large cell lymphoma - PubMed abstracts
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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. - Buswire
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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
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Treatments
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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.
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Clinical Trials
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Resources & Research News
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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."
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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
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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
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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
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Guidance
for Parents on Childhood cancers by NCI - Large PDF
file | PDF-Help
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A parent's guide to children's cancer - CancerBACUP
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Natural Killer Lymphoma/Leukemia: An Uncommon Pediatric Case
with Indolent Course. Leuk Lymphoma. 2004;45(8):1687-1689. PMID:
15370226 | Related
articles
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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
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Primary Non-Hodgkin's Lymphoma of Bone in Children - ejbjs.org/
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.
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