Note-For the unusual patients with small mediastinal masses (<10 cm or <1/3 of the maximal chest diameter) & with stage I disease, treatment with 3 cycles of CHOP could be considered. If a complete remission is achieved after three cycles, this could be followed by involved field radiation.
REF:NEJM 339: 21-26, 1998
All other patients to receive 6 cycles of chemotherapy. If the gallium/PET scan remains positive or a significant residual mass is present (> 20% of the original volume), the risk for recurrence is high. Therefore, consolidation with XRT or HDT is recommended.
Most patients with PMBL will initially respond to treatment with shrinkage of the tumor, but rapid disease progression between treatment cycles is not uncommon.
A retrospective analysis of 35 patients with PMBL treated with high-dose cyclophosphamide, carmustine, and etoposide (CBV) + ABMT was done. Patients with primary refractory disease had 58% long-term DFS, & patients with relapsed disease had 27% long-term DFS. The strongest predictor of PFS was chemotherapy responsiveness immediately before transplant. But even in chemotherapy-refractory patients, 33% long-term survival was observed. Therefore, although HDC & ABMT are not usually recommended in refractory NHL, PMBL may be an exception.