Stage I Childhood NHL
Stage II Childhood NHL
Stage III Childhood NHL
Stage IV Childhood NHL

The most widely used staging scheme for childhood non-Hodgkin lymphoma (NHL) is that of the St. Jude Children’s Research Hospital (Murphy Staging).[1]

In stage I childhood NHL, a single tumor or nodal area is involved, excluding the abdomen and mediastinum.

In stage II childhood NHL, disease extent is limited to a single tumor with regional node involvement, two or more tumors or nodal areas involved on one side of the diaphragm, or a primary gastrointestinal tract tumor (completely resected) with or without regional node involvement.

In stage III childhood NHL, tumors or involved lymph node areas occur on both sides of the diaphragm. Stage III NHL also includes any primary intrathoracic (mediastinal, pleural, or thymic) disease, extensive primary intra-abdominal disease, or any paraspinal or epidural tumors.

In stage IV childhood NHL, tumors involve bone marrow and/or central nervous system (CNS) disease regardless of other sites of involvement.

Bone marrow involvement has been defined as 5% malignant cells in an otherwise normal bone marrow with normal peripheral blood counts and smears. Patients with lymphoblastic lymphoma with more than 25% malignant cells in the bone marrow are usually considered to have leukemia and may be appropriately treated on leukemia clinical trials.

CNS disease in lymphoblastic lymphoma is defined by criteria similar to that used for acute lymphocytic leukemia (i.e., white blood cell count of at least 5/μL and malignant cells in the cerebrospinal fluid [CSF]). For any other NHL, the definition of CNS disease is any malignant cell present in the CSF regardless of cell count. The Berlin-Frankfurt-Munster (BFM) group analyzed the prevalence, clinical pattern, and outcome of CNS involvement in NHL in over 2,500 patients.[2] Overall, CNS involvement was diagnosed in 6% of patients. Involvement by cell type was as follows:

• Burkitt lymphoma/leukemia: 8.8%
• Precursor B-cell lymphoblastic lymphoma: 5.4%
• Anaplastic large cell lymphoma: 3.3%
• T-cell lymphoblastic lymphoma: 3.7%
• Diffuse large B-cell lymphoma: 2.6%
• Primary mediastinal large B-cell lymphoma: 0%

The probability of event-free survival at 6 years for CNS-positive patients was 64% compared with 86% for CNS-negative patients. Presence of CNS involvement did not impact outcome for T-cell lymphoblastic lymphoma patients, but had significant negative impact on patients with Burkitt lymphoma/leukemia.[2]

As with histologic classification, there exist several different staging schemes for childhood NHL; none is perfect. For example, in the French Society of Pediatric Oncology and most recent international French-American-British study for B-lineage NHL, group A is completely resected stage I and II disease; group C is disease with leukemic disease (>25% marrow involvement) and/or CNS disease; and group B consists of all other patients.[3-5] For B-lineage NHL, the BFM group treats according to four risk groups: R1 is completely resected disease; R2 is unresected disease or stage III disease with lactate dehydrogenase (LDH) less than 500 u/L; R3 is stage III and LDH concentrations of 500 to 1,000 u/L or leukemic disease (>25% marrow disease) with LDH levels higher than 1,000 u/L; and R4 is stage III/IV disease or leukemic disease with LDH levels higher than 1,000 u/L and/or CNS involvement.[6] In general, treatment for childhood NHL depends on localized versus disseminated disease. Localized disease is usually defined as stage I or II disease, while stage III or IV disease is generally considered disseminated.

References

1. Murphy SB, Fairclough DL, Hutchison RE, et al.: Non-Hodgkin's lymphomas of childhood: an analysis of the histology, staging, and response to treatment of 338 cases at a single institution. J Clin Oncol 7 (2): 186-93, 1989.  [PUBMED Abstract]
   
   
2. Salzburg J, Burkhardt B, Zimmermann M, et al.: Prevalence, clinical pattern, and outcome of CNS involvement in childhood and adolescent non-Hodgkin's lymphoma differ by non-Hodgkin's lymphoma subtype: a Berlin-Frankfurt-Munster Group Report. J Clin Oncol 25 (25): 3915-22, 2007.  [PUBMED Abstract]
   
   
3. Patte C, Auperin A, Michon J, et al.: The Société Française d'Oncologie Pédiatrique LMB89 protocol: highly effective multiagent chemotherapy tailored to the tumor burden and initial response in 561 unselected children with B-cell lymphomas and L3 leukemia. Blood 97 (11): 3370-9, 2001.  [PUBMED Abstract]
   
   
4. Patte C, Auperin A, Gerrard M, et al.: Results of the randomized international FAB/LMB96 trial for intermediate risk B-cell non-Hodgkin lymphoma in children and adolescents: it is possible to reduce treatment for the early responding patients. Blood 109 (7): 2773-80, 2007.  [PUBMED Abstract]
   
   
5. Cairo MS, Gerrard M, Sposto R, et al.: Results of a randomized international study of high-risk central nervous system B non-Hodgkin lymphoma and B acute lymphoblastic leukemia in children and adolescents. Blood 109 (7): 2736-43, 2007.  [PUBMED Abstract]
   
   
6. Reiter A, Schrappe M, Tiemann M, et al.: Improved treatment results in childhood B-cell neoplasms with tailored intensification of therapy: A report of the Berlin-Frankfurt-Münster Group Trial NHL-BFM 90. Blood 94 (10): 3294-306, 1999.  [PUBMED Abstract]
   
   

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