Sarcoma Book Section


Authors: Shin, J. Y.; Mueller, B.; Alektiar, K.
Editors: Cuaron, J. J.; Braunstein, L. Z.
Article/Chapter Title: Sarcoma
Abstract: Soft tissue sarcomas are a rare type of malignant tumor that develop in connective tissue. In 2024, there will be an estimated 13,590 new cases and 5200 deaths. Soft tissue sarcomas of the extremity are more common, and the general treatment paradigm for larger and high-grade tumors consists of limb-sparing surgery with radiotherapy (RT) given pre- or postoperatively to optimize local control with rates >80%. In the early 1980s, the NCI (National Cancer Institute, United States) randomized trial by Rosenberg et al. showed that limb-sparing surgery followed by postoperative RT resulted in equivalent local control and survival outcomes as amputation. Subsequently, two prospective randomized trials demonstrated a significant improvement in local control with the addition of adjuvant RT to limb-sparing surgery. In the NCI randomized trial by Yang et al., which evaluated the role of external beam RT after limb-sparing surgery in 91 patients, the addition of adjuvant external beam RT improved local control from 78% to 100% in patients with high-grade disease. A randomized trial by Pisters et al. assessed the role of adjuvant brachytherapy after limb-sparing surgery and found that adjuvant brachytherapy improved local control over limb-sparing surgery alone from 69% to 82% for all patients and from 66% to 89% for patients with high-grade disease. The advent and essential role of intensity-modulated radiotherapy (IMRT) have been well-characterized in more recent studies. IMRT is associated with excellent 5-year local control rates >90%, suggesting that its precision has a beneficiary effect in improving local control and sparing normal tissue. In a study on 134 patients with high-grade primary soft tissue of the extremity, local control was better in the IMRT cohort when compared to those receiving adjuvant brachytherapy, with a 5-year local control rate of 92% vs. 81% (p = 0.04). And in a comparison between IMRT versus conventional external beam RT, IMRT again was associated with a significantly reduced local recurrence rate: 7.6% at 5 years in the IMRT cohort vs. 15.1% in the conventional cohort (p = 0.02). And the RTOG 0630 trial demonstrated that even with reduced treatment volumes, IMRT together with image-guided radiation therapy (IGRT) in the preoperative setting is safe and effective. Both post- and pre-operative IMRT has thus become the standard of care for the treatment of soft sarcoma of the extremity. The role of radiotherapy in retroperitoneal sarcoma remains controversial. While a number of studies have shown surgery with RT to be associated with improved local control when compared to surgery alone, other studies have demonstrated no benefit. The randomized phase III EORTC-62092 STRASS trial demonstrated no benefit in median abdominal recurrence-free survival (4.5 years in the RT plus surgery group vs. 5.0 years in the surgery only group, p = 0.95), with a higher number of serious adverse events reported in the RT plus surgery group. A multi-institutional retrospective study on 204 patients, however, demonstrated improved 5-year local recurrence-free survival for those receiving surgery and peri-operative RT vs. surgery alone (91% vs. 65%, p = 0.02).9 Postoperative RT is associated with a higher complication rate, and thus pre-operative RT is the preferred approach when combined RT plus surgery is recommended. A multi-disciplinary approach is recommended in the management of soft tissue sarcoma, and radiotherapy remains essential to optimize local control rates in those at higher risk for recurrence of resected disease. © The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature Switzerland AG 2025.
Keywords: radiotherapy; sarcoma; intensity-modulated radiotherapy; soft tissue sarcoma; brachytherapy; local control; randomized trial; arthroplasty; extremity; retroperitoneal; transplantation (surgical); transplants; external-beam radiotherapies; high grades
Book Title: Radiation Oncology Clinical Fundamentals
ISBN: 978-3-031-77558-1
Publisher: Springer Nature  
Publication Place: Cham, Switzerland
Date Published: 2025-01-01
Start Page: 113
End Page: 117
Language: English
DOI: 10.1007/978-3-031-77559-8_10
PROVIDER: scopus
DOI/URL:
Notes: Book chapter: 10 -- Source: Scopus
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MSK Authors
  1. Kaled M Alektiar
    333 Alektiar
  2. Boris A Mueller
    104 Mueller
  3. John Jacob Cuaron
    142 Cuaron
  4. Jacob Y Shin
    25 Shin