Children and adolescents with early-stage nodular lymphocyte-predominant Hodgkin lymphoma (nLPHL) have a 5-year event-free survival (EFS) of about 77% after complete lymph node resection and around 89% with anthracycline-containing chemotherapy. We investigated whether patients with early-stage nLPHL can be successfully treated with surgical resection alone or with low-intensity, anthracycline-free CVP chemotherapy for those with unresectable nodal disease. EuroNet-PHL-LP1 was a prospective phase III trial enrolling patients under 18 years with stage IA/IIA nLPHL. Completely resected lymph nodes led to active surveillance. Patients with unresectable disease received three cycles of CVP (cyclophosphamide, vinblastine, prednisone). In CVP-cohort-1, treatment cessation required a negative 18F -FDG-PET. An amendment in 2014 omitted PET for end-of-treatment response; CT and/or MRI alone defined response in CVP-cohort-2. The primary endpoint was 5-year EFS, with death, relapse, second malignancy and PET-positivity counting as events; for CVP-cohort-2, progression-free survival (PFS) was primary. Among 267 registered patients (2009-2018), 247 were evaluable. Seventy-eight underwent resection only; their 5-year EFS/PFS was 79.5%. Six of 18 patients who relapsed after surgical resection re-entered the CVP arm. In CVP-cohort-1, 51/82 (62%) achieved a complete metabolic response (CMR). 5-year EFS was 56.4%, while 5-year PFS in the CMR group was 91.3%. In contrast, PFS in 84 CVP-cohort-2 patients was 64.7%. During CVP, 68.3% of patients experienced grade 3-4 neutropenia. No treatment-related deaths were reported. Excellent outcomes were achieved after complete resection and with low-intensive, anthracycline-free chemotherapy if 18F-FDG-PET showed CMR after chemotherapy. This strategy now constitutes the EuroNet-PHL standard of care for early-stage nLPHL. The trial was registered under EudraCT-No.: 2007-004092-19.
Shankar et al. (Wed,) studied this question.
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