Limited stage I disease is not necessarily indicative of an excellent prognosis in childhood anaplastic large cell lymphoma (ALCL)
Attarbaschi, Andishe × Mann, Georg Rosolen, Angelo Williams, Denise Uyttebroeck, Anne Marky, Ildiko Lamant, Laurence Horibe, Keizo Wrobel, Grazyna Beishuizen, Auke Wössmann, Wilhelm Reiter, Alfred Mauguen, Audrey Le Deley, Marie-Cecile Brugières, Laurence #
Data on incidence, characteristics and prognosis in stage I childhood anaplastic large cell lymphoma (ALCL) are scarce. Out of 463 patients (pts.) enrolled into the international trial ALCL99, 36 (8%) cases had stage I disease and were treated with a prephase followed by either 3 chemotherapy courses in case of initial complete resection (6 pts.) or otherwise by 6 courses (30 pts.). Disease localization was peripheral lymph nodes in 26, soft tissue mass in 8, solitary bone and bronchial disease in one patient each. Of the 6 patients with complete resection none suffered from relapse while of the 30 patients 9 (30%) relapsed including in all cases a new site of disease involvement and 3 of 5 ALK- patients. In summary, the failure rate for non-completely resected disease was similar to patients with stage II and III/IV disease. Whether ALK-negativity contributed to this moderate outcome has to be proven prospectively. This study was registered at www.clinicaltrials.gov as NCT00006455.