Thoracic Sarcomas in Children

Abstract

ObjectiveThis study reviews the experience of the Intergroup Rhabdomyosarcoma Studies (IRS) 11 and III with thoracic sarcomas. Summary Background DataThoracic sarcomas in children present special problems with histologic diagnosis, total resection of tumor, and local control. Previous studies have shown high rates of local and distant relapse. MethodsData from evaluable patients enrolled in IRS II and IRS IlIl were reviewed. Progression-free and overall survival were estimated by the Kaplan-Meier method and statistical differences were calculated by the log-rank test. The multivariate regression analysis of data was performed using the Cox proportional hazards model. ResultsA total of 84 patients presented with thoracic sarcomas during IRS 11 (51 patients) and IRS III (33 patients). Of these, 76 were chest wall tumors, 3 were lung tumors, 4 were pleural tumors, and 1 arose from the heart. The average age at diagnosis was 8.5 years (range, 1 month-20.5 years). Thirty-three patients had alveolar histology, 17 had embryonal histology, and 12 had extraosseous Ewing's sarcoma, with indeterminate, undifferentiated, and pleomorphic histologies in the remaining 22 patients. Thirteen patients presented as group (localized disease completely resected), 18 as group 11 (microscopic residual or nodal disease), 31 as group III (gross residual disease or biopsy only), and 22 as group IV (distant metastatic disease). Sixty patients (71 %) achieved a complete response. Thirty-nine patients had a local relapse, and 22 had a distant relapse. Thirty-five patients (42%) survived with a mean follow-up of 1.8 years (range 0.7-11.7 years); 49 (58%) have died with an average survival of 1.1 years (range 0.3-11.7 years). Four patients (5%) died of infection or treatment complications. Progression-free survival was not significantly associated with histology, site, clinical group, or IRS study. Overall survival was significantly associated with clinical group, size, and local or distant recurrence, but not with histology or IRS study by univariate analysis. In a multivariate analysis, only clinical group and local and distant recurrence showed statistical significance (p < 0.002, <0.006, and <0.001, respectively).

Predictors of outcome in children and adolescents with rhabdomyosarcoma of the trunk—the St Jude Children's Research Hospital experience

“…Truncal RMS has long been deemed to have a poor prognosis relative to RMS at other sites [2]. Our institutional experience confirms this finding and has identified several new factors which may have prognostic implications in the management of patients with truncal RMS.…”

“…Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in the pediatric age group. However, RMS in the trunk is rare, comprising only 4% to 7% of all RMS in the Intergroup Rhabdomyosarcoma Study (IRS)-I,-II, and -III reports [1][2][3]. Primary site of tumor has long been deemed a prognostic determinant in previous studies [4], and the truncal site has been associated with a poor prognosis [1,2].…”

“…Several factors associated with unfavorable outcome after treatment of truncal RMS have been previously identified, including advanced stage at presentation, alveolar histology, and local and distant tumor recurrence [2,4]. The unfavorable outcome of truncal RMS may be explained in several ways.…”

“…Because these tumors produce symptoms only when large or advanced, they may be detected later than tumors at other sites. Diffuse involvement of the pleura and peritoneum makes complete surgical resection very difficult, which also may contribute to inferior outcomes [2]. Although gross total resection (GTR) is the goal of surgical therapy for RMS, the unique anatomy of the truncal region often mandates major chest wall or abdominal wall resection and reconstruction.…”

Chest Wall And Lung Tumors

“…IRS'nin çalışmalarında rabdomiyosarkomların %3.7'si göğüs duvarı kaynaklıdır. Bu grupta yaş ortalamı 8.5 yıl olup, hastaların yarısından fazlasında alveolar histoloji saptanmıştır (16). Tedavide multimodal tedavi uygulanmaktadır.…”