Second‐line therapy in young patients with relapsed or refractory orbital rhabdomyosarcoma

Authors:
Publisher website
Google Scholar
Abstract
Objective: Localized orbital rhabdomyosarcoma (oRMS) has an overall favourable prognosis with more than 90% of survival. Little is known about the best strategy in recurrent/refractory (R/R) cases. The purpose is to examine the characteristics of patients with R/R‐oRMS, focusing on local therapy.Methods: This is bicentric retrospective study. Analysis is of young patients (<30 years) with R/R‐oRMS who were treated from 1989 to 2018 at the Institut Curie and Gustave Roussy Cancer Campus, France.Results: Twenty‐seven out of 162 patients (17%) with oRMS presented with R/R disease. 6 of these patients had alveolar RMS (22%), 3 of whom had initial parameningeal extension (11%). During first‐line treatment, 18 patients (67%) had orbital radiotherapy. Median age at R/R was 10 years (ranges: 4–28) after a delay of 19 months from diagnosis (ranges: 3–40). Tumoral events were local relapses (22 cases), local progression (3 cases) or regional relapses (2 cases). Second‐line treatments included chemotherapy (27 cases), radiotherapy (16 cases), surgery (exenteration; 8 cases) and metastasis/ nodal removal (3 cases). After a median follow‐up of 99 months (range: 10–306), 4 patients died and 23 are in complete remission (CR) without treatment. One patient had subsequent relapse treated with exenteration and brachytherapy until a new tumour remission. Five‐year event‐free and overall survivals after first tumour event are, respectively, 84.4% (95% confidence interval: 71.5%–98.8%) and 85.8% (95% confidence interval: 72.1%–100.0%)Conclusion: R/R‐oRMS is a rare situation. Second‐line therapy is efficient in this location, sometime at the cost of lifesaving mutilating surgery. Second‐line local therapy needs therefore to consider local radiotherapy if possible or complete wide surgery.

This publication has 28 references indexed in Scilit: