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. The BFM group has used three cycles of chemotherapy following a cytoreductive prophase for completely resected stage I/II disease. 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.[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. 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. 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.
is rare in children, with only case reports and case series to guide therapy. Although outcome is generally very good, treatments range from surgery only to multiagent chemotherapy and even autologous blood or marrow transplant.[15,16,17,18,19,20] It appears that for pediatric patients without the BCL2 rearrangement and a high proliferative index, surgical resection with no further treatment is sufficient for completely resected, localized disease. For those with tumors that have the BCL2 rearrangement, treatment like that of adult patients with follicular lymphoma is preferred (refer to the PDQ summary on Adult Non-Hodgkin Lymphoma Treatment for more information).
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.
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