title: Limited stage I disease is not necessarily indicative of an excellent prognosis in childhood anaplastic large cell lymphoma.
abstract: Data on incidence, characteristics, and prognosis in stage I childhood anaplastic large cell lymphoma are scarce. Of 463 patients enrolled in the international ALCL99 trial, 36 (8%) had stage I disease and were treated with a prephase chemotherapy, followed by either 3 chemotherapy courses in case of initial complete resection (6 patients) or otherwise by 6 courses of chemotherapy (30 patients). Disease localization was to the peripheral lymph nodes in 26, soft tissue mass in 8, and solitary bone and bronchial disease in 1 patient each. Of the 6 patients with complete resection, none experienced relapse, whereas of the 30 remaining stage I patients, 9 (30%) relapsed, including in all cases a new site of disease involvement and including 3 of 5 anaplastic lymphoma kinase-negative patients. In summary, the failure rate for incompletely resected stage I disease was similar to that for patients with stage II and stage III/IV disease. Whether anaplastic lymphoma kinase negativity contributed to this moderate outcome has to be proven prospectively. This study was registered at www.clinicaltrials.gov as NCT00006455.
authors: Paediatric Hematology and Oncology, St Anna Children's Hospital, Vienna, Austria. andishe.attarbaschi@stanna.at
authors: Andishe
authors: A
authors: Attarbaschi
authors: Georg
authors: G
authors: Mann
authors: Angelo
authors: A
authors: Rosolen
authors: Denise
authors: D
authors: Williams
authors: Anne
authors: A
authors: Uyttebroeck
authors: Ildiko
authors: I
authors: Marky
authors: Laurence
authors: L
authors: Lamant
authors: Keizo
authors: K
authors: Horibe
authors: Grazyna
authors: G
authors: Wrobel
authors: Auke
authors: A
authors: Beishuizen
authors: Wilhelm
authors: W
authors: Wössmann
authors: Alfred
authors: A
authors: Reiter
authors: Audrey
authors: A
authors: Mauguen
authors: Marie-Cecile
authors: MC
authors: Le Deley
authors: Laurence
authors: L
authors: Brugières
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D005260:Female; D006801:Humans; D007390:International Agencies; D017728:Lymphoma, Large-Cell, Anaplastic / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology*; D008297:Male; D009367:Neoplasm Staging; D011379:Prognosis; D011446:Prospective Studies; D015996:Survival Rate