Adjuvant and neoadjuvant chemotherapy for soft tissue sarcoma of the extremities
- Robert Maki, MD, PhD
Robert Maki, MD, PhD
- Section Editor — Bone and Soft Tissue Tumors
- Professor of Medicine
- Monter Cancer Center
- Hofstra-Northwell School of Medicine
Soft tissue sarcomas (STS) are uncommon malignant tumors that arise from extraskeletal connective tissues, including the peripheral nervous system. They can arise at any body site, but are most common in the extremities, particularly the lower limbs. (See "Clinical presentation, histopathology, diagnostic evaluation, and staging of soft tissue sarcoma", section on 'Clinical presentation'.)
In treating STS of the extremities, the major therapeutic goals are long-term survival, avoidance of a local recurrence, maximizing function, and minimizing morbidity. Surgical resection is the cornerstone of potentially curative treatment. For nearly all patients with extremity sarcomas >5 cm, the addition of radiation therapy (RT) improves local control, and it has also had a significant impact on limb salvage. There are advantages to preoperative (neoadjuvant) as compared with postoperative (adjuvant) administration of RT, and for neoadjuvant therapy utilizing combinations of RT and chemotherapy, particularly for recurrent and large, high-grade primary tumors. These topics are discussed in detail elsewhere. (See "Overview of multimodality treatment for primary soft tissue sarcoma of the extremities and chest wall" and "Treatment of locally recurrent and unresectable, locally advanced soft tissue sarcoma of the extremities".)
Systemic chemotherapy is a routine component of treatment for several sarcomas that occur predominantly in children (eg, rhabdomyosarcoma, Ewing sarcoma, and osteogenic sarcoma). However, the value of adjuvant chemotherapy in patients undergoing resection of the adult-type localized extremity STS (eg, leiomyosarcoma, liposarcoma, synovial sarcoma) remains controversial due to the complexity of the group of diagnoses involved.
This topic review will discuss the use of adjuvant and neoadjuvant chemotherapy in the treatment of adult-type extremity STS. The role of chemotherapy in the treatment of retroperitoneal STS, rhabdomyosarcoma and the Ewing sarcoma family of tumors, and neoadjuvant combined modality approaches for patients with large, high-grade or recurrent extremity STS are discussed in detail elsewhere. (See "Clinical features, evaluation, and treatment of retroperitoneal soft tissue sarcoma", section on 'Adjuvant chemotherapy' and "Rhabdomyosarcoma in childhood, adolescence, and adulthood: Treatment" and "Treatment of the Ewing sarcoma family of tumors" and "Surgical treatment and other localized therapy for metastatic soft tissue sarcoma".)
Pediatric-type sarcomas — The addition of systemic chemotherapy to local therapy significantly improves outcomes for the common pediatric types of sarcoma (rhabdomyosarcoma, osteogenic sarcoma, and the Ewing sarcoma family of tumors of both soft tissue and bone). Most modern treatment plans utilize initial (induction or neoadjuvant) chemotherapy followed by local treatment and additional (adjuvant) chemotherapy.
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- ADJUVANT CHEMOTHERAPY
- Pediatric-type sarcomas
- - Rhabdomyosarcoma
- - Ewing sarcoma
- - Extraosseous osteogenic sarcomas
- Sarcomas more commonly seen in adults
- - Early randomized trials
- - SMAC meta-analysis
- - Later randomized trials
- - Updated meta-analyses
- - Pooled analysis of the EORTC trials
- - Impact of histology
- - Summary
- NEOADJUVANT CHEMOTHERAPY
- Chemotherapy with regional hyperthermia
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS