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Childhood Non-Hodgkin Lymphoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Low-Stage Childhood NHL Treatment

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For low-stage anaplastic large cell lymphoma, the best results have come from using pulsed chemotherapy similar to mature B-cell NHL therapy. In the POG study for low-stage lymphoma using three cycles of CHOP, a 5-year event-free survival (EFS) of 88% for large cell lymphoma (anaplastic large cell lymphoma and diffuse large B-cell lymphoma) patients was reported.[3] The BFM group has used three cycles of chemotherapy following a cytoreductive prophase for completely resected stage I/II disease.[11] The FRE-IGR-ALCL99 trial used three cycles of chemotherapy following cytoreductive prophase for patients with stage I completely resected disease. The minority of stage I patients had complete resections (6 out of 36) but there were no treatment failures for these six patients. The therapy for patients without complete resection was the same as the therapy for patients with disseminated disease and the 3-year EFS (81%) and OS (97%) were not statistically different from the outcomes for patients with higher stage disease.[12][Level of evidence: 2A]

Primary cutaneous anaplastic large cell lymphoma presents a particular problem. The diagnosis can be difficult to distinguish from more benign diseases such as lymphoid papulosis.[13] Primary cutaneous anaplastic large cell lymphoma usually does not express ALK and may be treated successfully with surgical resection and/or local radiation therapy without systemic chemotherapy.[14] There are reports of surgery alone being curative for ALK-positive cutaneous anaplastic large cell lymphoma, but extensive staging and vigilant follow-up is required.

Follicular lymphoma is rare in children, with only case reports and case series to guide therapy. Case series reporting a variety of chemotherapy approaches have resulted in good outcomes.[15,16,17,18,19]

Subcutaneous mature T-cell lymphomas are very rare in children. An oral retinoid (bexarotene) has been reported to be active against subcutaneous T-cell lymphomas in children.[20]

Standard treatment options are based on histology; however, current data do not suggest superiority between regimens listed below for a specific histology.

Standard Treatment Options

Table 4. Standard Treatment Options for Low-Stage Non-Hodgkin Lymphoma

DiseaseTreatment Options
ALL = acute lymphoblastic leukemia; BFM = Berlin-Frankfurt-Munster; CCG = Children's Cancer Group.
Burkitt lymphoma or diffuse large B-cell lymphoma (DLBCL) (completely resected)GER-GPOH-NHL-BFM-95 (R1): Two cycles of chemotherapy.[5]
COG-C5961 (FAB/LMB-96)(Group A): Two cycles of chemotherapy.[6]
 
Burkitt lymphoma or DLBCL (nonresected stage I/II)GER-GPOH-NHL-BFM-95 (R2): Prephase + four cycles of chemotherapy (4-hour methotrexate infusion).[5]
COG-C5961 (FAB/LMB-96)(Group B): Prephase + four cycles of chemotherapy (reduced-intensity arm).[7]
POG-8314/POG-8719: Three cycles of chemotherapy (no radiation or maintenance therapy).[3]
 
Lymphoblastic lymphomaGER-GPOH-NHL-BFM-95: Induction, consolidation, intensification, and maintenance therapy (2 years of total therapy); ALL-type induction and consolidation, high-dose methotrexate courses × 4, and ALL-type maintenance therapy (2 years of total therapy).[8,9]
COG-A5971 (NCT00004228): Modified CCG-BFM ALL therapy; 2 years of total therapy.[10]
 
Anaplastic large cell lymphomaPOG-8314/POG-8719: Three cycles of chemotherapy (no radiation or maintenance therapy).[3]
GER-GPOH-NHL-BFM-90: Prephase + three cycles of chemotherapy (only for completely resected disease).[11]
FRE-IGR-ALCL99: Prephase + six cycles of chemotherapy (for disease not completely resected).[12]
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