International Journal of Radiation Oncology*Biology*Physics
Clinical InvestigationAdjuvant Radiotherapy for Pediatric and Young Adult Nonrhabdomyosarcoma Soft-Tissue Sarcoma
Introduction
Sarcomas are rare in the pediatric population. The 500 children diagnosed with nonrhabdomyosarcoma soft-tissue sarcoma (NRSTS) each year in the United States represent only 4% of all malignant tumors in the pediatric population (1). Most pediatric soft-tissue sarcomas are rhabdomyosarcomas (2); however, 40% of children <5 years and 77% aged 15–19 years will have NRSTS (1). This group of tumors comprises many different histologic types. Most common in pediatric and young adult patients are malignant fibrous histiocytoma/pleomorphic sarcoma, synovial sarcoma, malignant peripheral nerve sheath tumor, and fibrosarcoma (3). Because these entities are so rare in young patients, our knowledge of their natural history and appropriate treatment is limited. Prospective randomized studies are scarce, and most have been designed to answer questions regarding systemic therapy. The standard of care for local management has been surgical excision, and the optimal role of adjuvant radiotherapy (RT) is unclear.
The purpose of the present study was to evaluate the prognostic indicators, outcomes, and complications in pediatric and young adult patients with NRSTS treated at the University of Florida with adjuvant RT.
Section snippets
Methods and Materials
Using an institutional review board-approved protocol, we retrospectively reviewed the charts of 95 patients aged ≤30 years who presented to the University of Florida with a tissue diagnosis of NRSTS and had been treated curatively between 1973 and 2007. All patients had undergone RT at our institution, and most had also undergone surgery at the University of Florida. Patients treated with surgery alone were not included in the present study. Patients who underwent RT alone for macroscopic
Local control
Of the 95 patients, 12 developed local recurrence. The median interval to local failure was 0.9 year (range, 0.2–7.4). The LC rate at 5 and 15 years was 88% and 86%, respectively (Fig. 1). All but one recurrence occurred within the first 2 years. Of the patients with local recurrence, 83% developed the recurrence in the absence of metastatic disease. On univariate analysis, a negative microscopic margin was associated with a statistically significant improvement in LC (Table 1). On multivariate
Discussion
A paucity of outcome data addressing multimodality local therapy in pediatric and young adult NRSTS patients is available. In the largest single-institution series to date, Spunt et al. (8) analyzed 121 pediatric patients with NRSTS who underwent surgical resection with or without RT between 1969 and 1996 from St. Jude's hospital. The 5-year rate of local recurrence was 13%. Local recurrence was associated with microscopically positive margins, an intra-abdominal primary tumor, and the lack of
Conclusion
Our study has contributed to understanding the expected outcomes of pediatric and young adult NRSTS patients treated with surgery and RT for local therapy. Furthermore, our study has provided a basis for identifying high-risk patients for whom treatment intensification might be justified. The priority in local management is to obtain function-preserving wide local excision. When complete excision is not achieved, patients are at an increased risk of local failure and death from the local
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Synovial sarcoma in children and adolescents
2016, Bulletin du CancerNon-rhabdomyosarcoma soft tissue sarcomas in children: A surveillance, epidemiology, and end results analysis validating COG risk stratifications
2015, International Journal of Radiation Oncology Biology PhysicsCitation Excerpt :Critical data regarding surgical margins, systemic therapy, radiation dose and schedule, treatment intent, and performance status likely influenced this selection bias. As an example, multiple series have shown that negative margins are strongly associated with improved survival outcomes in pediatric NRSTS, and many of these patients do not receive radiation therapy (10, 24, 25). Conversely, positive margins are often an indication for adjuvant radiation therapy, yet these patients fare much worse.
Anticipated intraoperative electron beam boost, external beam radiation therapy, and limb-sparing surgical resection for patients with pediatric soft-tissue sarcomas of the extremity: A multicentric pooled analysis of long-term outcomes
2014, International Journal of Radiation Oncology Biology PhysicsCitation Excerpt :To the date it is not yet completely clear which NRSTS patients with resectable disease will fail locally and thus might particularly benefit from the addition of radiation therapy (2). It is known from retrospective single-institution studies that for completely resected tumors the width of a clear surgical margin influences the local recurrence rate (23, 24). Spunt et al (23) retrospectively reviewed the St. Jude Children's Research Hospital experience of 125 pediatric NRSTS and reported after a median follow-up of 9.2 years 5-year survival and DFS rates of 89% and 77%.
Pulmonary metastasectomy in pediatric/adolescent patients with synovial sarcoma: An institutional review
2013, Journal of Pediatric SurgeryCitation Excerpt :Many reports that have evaluated the role of metastasectomy in pediatric/adolescent patients with NRMS have analyzed an aggregate patient population with different primary histologies. For instance, four recently published adult and pediatric series examining pulmonary metastasectomy for metastatic STS evaluated a total of 301 patients, of which only 12% had SS [2,10–12]. In this retrospective review, our aim was to identify prognostic factors, best surgical approach, and survival outcomes for pediatric and adolescent patients with SS who underwent surgical resection for confirmed pulmonary metastasis at Memorial Sloan-Kettering Cancer Center between June 1971 and May 2011.
The non-rhabdomyosarcoma soft tissue sarcomas, desmoid tumor and osteosarcoma
2018, Pediatric Oncology
Conflict of interest: none.