Prognostic Factors and Treatment Outcomes of Adult Patients With Rhabdomyosarcoma After Multimodality Treatment

Abstract

Background: Adults with rhabdomyosarcoma (RMS) have a worse clinical outcome compared to pediatric cases. In the present study, the failure pattern and clinical outcome of adult patients with RMS who received multimodality treatment at our Institution was assessed. Patients and Methods: Data were retrospectively recorded and analyzed from 20 adult patients, aged 19 years or more, who were treated for RMS at our Institution between 2004 and 2015. Disease-free (DFS) and overall (OS) survival after starting treatment were calculated using the Kaplan-Meier method. The relationship of these outcome measures with the following variables was then assessed: Primary site, tumor stage, lymph node involvement, histological subtype, radiotherapy (RT), and duration of chemotherapy. Results: Sixteen patients had localized RMS, and four had metastatic disease. For the whole patient cohort, the 3-year DFS and OS rates were 20%, and 45%, respectively. Patients with alveolar histological subtype had a better 3-year OS than those with other subtypes (p=0.038). The median OS rates for those with localized and metastatic disease were 53.2 (95% confidence interval(CI)=14.7-91.8) months, and 21.7 (95% CI=0-45.7) months, respectively (p=0.047). In patients with localized RMS, those who received RT (n=13) had a better median DFS (24.6 versus 6.0 months, p=0.009) and OS (53.2 versus 11.4 months, p=0.009) than those who did not (n=3).For patients receiving RT, concurrent chemotherapy with vincristine and cyclophosphamide (n=11) was associated with better 3-year DFS (36.4% versus 0%, p<0.001) and OS (81.8% versus 0%, p<0.001) compared with RT alone (n=2). Administration of chemotherapy for more than 19 weeks significantly correlated with better 3-year DFS (44% versus 0%, p=0.001) and OS (53.3% versus 0%, p<0.001) in those with localized RMS. Conclusion: In addition to staging and histological subtype, our results indicate that concurrent chemoradiotherapy and longer duration of chemotherapy were associated with significantly improved DFS and OS in adult patients with localized RMS.Rhabdomyosarcoma (RMS) is the most common soft-tissue malignancy, accounting for 4-5% of cancer cases in children (1). In Taiwan, soft-tissue sarcoma accounted for approximately 40% of all pediatric cancers diagnosed between 1996 and 2010, with RMS comprising 6% of all pediatric soft-tissue sarcomas (2). RMS is far less common in adults (2). Although most cases occur in children younger than 10 years, adult RMS still accounted for 41% of all RMS cases in the Surveillance, Epidemiology and End Results (SEER) database between 1973 and 2005 (3). Pediatric patients with RMS are typically treated within clinical studies or by standard protocols. In the United States, the Intergroup Rhabdomyosarcoma Study Group (IRSG; now the Children's Oncology Group Soft-Tissue Sarcoma Committee) organized a series of studies incorporating multimodality treatment (chemotherapy, surgery, and radiotherapy) to treat pediatric RMS, and the 5-year overall survival (OS) after treatment cle...

Rhabdomyosarcoma in Adults: A Retrospective Analysis of Case Records Diagnosed between 1979 and 2018 in Western Denmark

“…In our analysis, anatomical location divided into “favourable” and “unfavourable” failed to reach statistical significance; this was also the case in a study by Hawkins et al [ 1 ]. Nevertheless, the primary tumour location is still clinically meaningful, as tumours occurring in, for example, the retroperitoneum, can become quite large before producing signs and symptoms [ 22 ]. The reason for the low percentage of patients receiving chemotherapy in our study (54%) ( Table 5 ) could be the predominance of pleomorphic subtype in our cohort (44%), as the majority of this subgroup would be treated as nonrhabdomyosarcoma with surgery only or with a combination of radiotherapy, as pleomorphic rhabdomyosarcoma has been shown not to be as responsive to chemotherapy as the embryonal or alveolar subtypes [ 18 , 22 ].…”

The VAC regimen for adult rhabdomyosarcoma: Differences between adolescent/young adult and older patients

“…Randomized clinical trials have recently shown that long‐term maintenance therapy with relatively low‐dose chemotherapy provided longer survival in pediatric RMS cases . There are retrospective data indicating that long‐term treatments are related to the long survival of adult RMS patients, and thus long‐term low‐dose chemotherapy might be promising as a new strategy for adult RMS patients in the future.…”

Evaluation of clinical efficacy of Chemotherapy for Rhabdomyosarcoma in children

“…RMS grows rapidly, has a high degree of malignancy, and is sensitive to chemotherapy and radiotherapy, but the effect of single treatment is poor; therefore, comprehensive treatment such as surgery, chemotherapy and radiotherapy are needed. 6 , 7…”