Treatment of locally recurrent and unresectable, locally advanced soft tissue sarcoma of the extremities
- Thomas F DeLaney, MD
Thomas F DeLaney, MD
- Section Editor — Bone and Soft Tissue Tumors
- Professor of Radiation Oncology
- Harvard Medical School
- David C Harmon, MD
David C Harmon, MD
- Assistant Professor of Medicine
- Harvard Medical School
- Mark C Gebhardt, MD
Mark C Gebhardt, MD
- Frederic W. and Jane M. Ilfeld Professor of Orthopedic Surgery
- Harvard Medical School
- Section Editors
- 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
- Russell S Berman, MD
Russell S Berman, MD
- Section Editor — Skin and Soft Tissue Surgery
- Chief of Surgical Oncology
- New York University Langone Medical Center
Soft tissue sarcomas (STS) are rare malignant tumors that arise from extraskeletal connective tissues, including the peripheral nervous system. They can arise at any body site.
Treatment for locally recurrent and locally advanced, potentially unresectable STS of the extremities will be reviewed here. Issues relating to classification, diagnosis, and staging of STS, local treatment options for localized primary STS of the extremities and chest wall, the use of adjuvant and neoadjuvant chemotherapy for extremity STS, and the treatment of STS in locations other than the extremities and chest wall are discussed separately. (See "Local treatment for primary soft tissue sarcoma of the extremities and chest wall" and "Adjuvant and neoadjuvant chemotherapy for soft tissue sarcoma of the extremities" and "Head and neck sarcomas" and "Breast sarcoma: Epidemiology, risk factors, clinical presentation, diagnosis, and staging" and "Clinical features, evaluation, and treatment of retroperitoneal soft tissue sarcoma" and "Clinical presentation, histopathology, diagnostic evaluation, and staging of soft tissue sarcoma", section on 'Introduction'.)
IMPORTANCE OF MULTIDISCIPLINARY EVALUATION AND MANAGEMENT
Because of their rarity and the frequent need for multimodality treatment, evaluation and management of STS should ideally be carried out in a center with expertise in the treatment of sarcomas, including surgical, orthopedic, medical, pediatric, and radiation oncology. The multidisciplinary team approach to the care of STS optimizes treatment planning, minimizes duplication of diagnostic studies, and reduces the time to implementation of the definitive therapeutic protocol, and the expertise gained by dedicated subspecialists improves clinical outcomes.
LOCALLY RECURRENT DISEASE
For patients with a locally recurrent STS, we recommend reresection rather than nonoperative therapy, if possible. Surgery is an important component of successful therapy for these patients. As with primary treatment, the goal is to salvage the limb with a complete resection and wide margins. (See 'Reresection' below.)
For patients who have not been previously irradiated, we recommend external beam radiation therapy (RT) in addition to surgical resection to improve rates of local control. In this setting, we suggest preoperative, rather than postoperative, treatment for most patients. (See 'Adjunctive RT' below.)
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- IMPORTANCE OF MULTIDISCIPLINARY EVALUATION AND MANAGEMENT
- LOCALLY RECURRENT DISEASE
- Adjunctive treatment
- - Patients without prior radiation therapy
- Adjunctive RT
- Preoperative chemoradiotherapy
- - Doxorubicin
- - Ifosfamide
- - Gemcitabine and temozolomide
- - Multiagent chemotherapy and RT
- - Response assessment
- - Previously irradiated patients
- Regional chemotherapy approaches
- Chemotherapy with regional hyperthermia
- - Postoperative chemotherapy
- UNRESECTABLE LOCALLY ADVANCED DISEASE
- Radiation therapy and chemoradiotherapy
- Regional chemotherapy
- - Isolated limb perfusion
- - Isolated limb infusion
- Systemic chemotherapy with and without regional hyperthermia
- Palliative chemotherapy
- NCCN guidelines
- POSTTREATMENT SURVEILLANCE
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS
- Local recurrence
- Unresectable locally advanced
- Posttreatment follow-up