par Réguerre, Yves;Martelli, Hélne;Rey, Annie;Rogers, Timothy;Gaze, Mark;Arush, Myriam Weyl Ben;Devalck, Christine ;Oberlin, Odile;Stevens, Michael;Orbach, Daniel
Référence European journal of cancer, 48, 13, page (2020-2027)
Publication Publié, 2012-09
Référence European journal of cancer, 48, 13, page (2020-2027)
Publication Publié, 2012-09
Article révisé par les pairs
Résumé : | Background: Localised pelvic rhabdomyosarcomas (pRMS) are rare tumours with a poorer prognosis than the majority of RMS. This study analysed patient outcome according to the type of local therapy delivered and the effect of disease-related factors on prognosis. Patients and methods: 97 children with localised pRMS were enrolled in the SIOP-MMT84, 89 and 95 studies. After primary surgery or biopsy, all children received ifosfamide/actinomycin/vincristine- based chemotherapy. Radiotherapy and surgery were planned in patients failing to achieve complete remission. Results: Median age at diagnosis was 52 months [5 months-18 years]. IRS staging was I for five patients, II for 15 and III for 77. Patients had embryonal RMS (N = 41), alveolar RMS (N = 29), botryoid RMS (N = 3), or not otherwise specified RMS (N = 24). Outcome: 87 patients achieved local control (90%), 37 relapsed (43%), mainly locally (84%). With a median follow-up of more than 10 years [4-22 years], 5-year OS was 66% (95% CI: 56-75%) and EFS was 52% (95% CI: 42-61%). Among the 18 IRS-I/II patients treated without radiotherapy, 15 survived. Seven out of the 20 IRS-III patients treated without local therapy died. In multivariate analysis, IRS staging, age greater than 10 years and lymph node involvement had a negative impact on OS. Perineal/perianal locations had a trend towards a worse prognosis. Conclusion: pRMS still have a relatively poor prognosis. Radiotherapy or brachytherapy is necessary for all IRS-III patients including those with radiological complete remission after neoadjuvant chemotherapy with or without surgery. Radiotherapy may be withheld in IRS-I patients and children under 3 years with IRS-II pRMS. © 2012 Elsevier B.V. All rights reserved. |