New trends in the treatment of sarcoma

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Weitere Verfasser: Porpiglia, Andrea S. (HerausgeberIn), Farma, Jeffrey M. (HerausgeberIn)
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Sprache:English
Veröffentlicht: Philadelphia, Pennsylvania Elsevier [2016]
Schriftenreihe:Surgical clinics of North America volume 96, number 5 (October 2016)
Online-Zugang:New trends in the treatment of sarcoma
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adam_text Titel: New trends in the treatment of sarcoma Autor: Porpiglia, Andrea S Jahr: 2016 New Trends in the Treatment of Sarcoma Contents Foreword xiii Ronald F. Martin Preface: Current Treatment of Sarcomas Andrea S. Porpiglia and Jeffrey M. Farma xv Epidemiology and Etiology of Sarcomas 901 Jane Y.C. Hui Sarcomas are rare malignancies of mesenchymal origin and are broadly divided into soft tissue sarcomas and bone sarcomas. The etiology of these tumors is largely unknown, and most sarcomas are sporadic. A small subset of sarcomas is associated with certain genetic syndromes and environmental factors. Ionizing radiation is the strongest environmental factor linked to sarcoma development. Bone and Soft Tissue Pathology: Diagnostic and Prognostic Implications 915 Julie Gibbs, Evita Henderson-Jackson, and Marilyn M. Bui Soft tissue and bone tumors are a heterogeneous group of tumors most often classified according to the type of tissue they most closely histologically resemble. Although sarcomas are rare, greater than 100 histologic subtypes of benign and malignant soft tissue and bone tumors are currently recognized. In this article, the authors review the current pathologic definitions, the classification and grading systems, supportive ancillary techniques, and the prognostic implications for some of the more common soft tissue and bone tumors. Radiologic Approach to Bone and Soft Tissue Sarcomas 963 Jamie T. Caracciolo and G. Douglas Letson Diagnostic imaging plays an important role in evaluation and treatment planning of patients with musculoskeletal tumors. This article discusses various imaging modalities available in the work-up, staging, and surveil- lance of patients with primary bone and soft tissue neoplasms. A system- atic approach to initial evaluation of newly suspected bone lesions and soft tissue masses is presented. Reviewed are relevant imaging features of musculoskeletal neoplasms that help predict tumor biology and risk of malignancy and findings that define internal tumor composition and allow for accurate preoperative histopathologic diagnosis before intervention. Finally, the role of diagnostic imaging in tumor staging, evaluation of response to neoadjuvant therapy, and postoperative surveillance is discussed. viii Contents Multimodality Management of Soft Tissue Tumors in the Extremity 977 Aimee M. Crago and Ann Y. Lee Most extremity soft tissue sarcomas present as a painless mass. Work-up should generally involve cross-sectional imaging with MRI and a core biopsy for pathologic diagnosis. Limb-sparing surgery is the standard of care, and may be supplemented with radiation for histologic subtypes at higher risk for local recurrence and chemotherapy for those at higher risk for distant metastases. This article reviews the work-up and surgical approach to extremity soft tissue sarcomas, and the role for radiation and chemotherapy, with particular attention given to the distinguishing characteristics of some of the most common subtypes. Retroperitoneal Sarcomas 993 Andrea S. Porpiglia, Sanjay S. Reddy, and Jeffrey M. Farma Retroperitoneal sarcomas are rare tumors, representing only 15% of all sarcomas. The mainstay of therapy is surgical resection with negative mar- gins. However, this is challenging because of the late presentation of many of these tumors and involvement with adjacent structures. Decisions on ra- diation therapy and chemotherapy should be made in a multidisciplinary setting at a tertiary referral center. Management of Truncal Sarcoma 1003 John E. Mullinax and Ricardo J. Gonzalez The management of truncal sarcoma presents unique challenges to the treating physician for reasons specific to this tumor location. First, the reconstruction options after resection of the abdominal or chest wall require a balance between cosmesis and structural integrity due to the multiplanar forces exerted on this region. Second, the histologies that commonly arise in this region are often associated with high local recur- rence rates, which often require complex decision making due to prior therapy. Finally, sarcomas of the trunk in the inguinal region involve those organs in the genitourinary system, of which resection can have significant psychosocial implications. Management of Desmoids 1015 Valerie P. Grignol, Raphael Pollock, and John Harrison Howard Desmoid tumors are rare, comprising 3% of soft tissue tumors. Surgical resection has been the standard of care; however, this has begun to evolve into a movement of watchful waiting as observational studies have shown long-term stability of many tumors without treatment and even sponta- neous regression in 5% to 10% of cases. When surgical therapy is used, wide local excision with microscopically negative margins is the goal of resection but should not be at the expense of organ or limb function. Recurrence rates after surgical resection are approximately 20%; a variety of multimodal therapies are useful in controlling disease. Contents ix Dermatofibrosarcoma Protuberans 1031 Jeffrey Reha and Steven C. Katz Dermatofibrosarcoma protuberans (DFSP) is a rare superficial soft tissue sarcoma. Its rarity precludes large prospective studies. Clinical diagnosis requires an high index of suspicion. Effective management requires an appreciation of tumor biology and the nature of the characteristic infiltrative growth pattern. DFSP tends to recur locally, with a low risk of dissemination. Aggressive surgical resection with widely negative margins is essential to management. Radiotherapy may be indicated in special circumstances. Un- derstanding the molecular pathogenesis has resulted in use of tyrosine ki- nase inhibitor therapy for patients with locally advanced disease or in metastatic disease. DFSP patients require long-term follow-up. Management of Breast Sarcoma 1047 Cary Hsu, Susan A. McCloskey, and Parvin F. Peddi Breast sarcomas are exceptionally rare mesenchymal neoplasms composed of many histologic subtypes. Therapy is guided by principles established in the management of extremity sarcomas. The anatomic site does influence treatment decisions, particularly the surgical manage- ment. Surgery should be undertaken with the aim of achieving a widely negative margin. Selected patients can be managed with breast- conserving surgery. Breast reconstruction is increasingly being under- taken for selected patients. Radiation therapy and chemotherapy are used selectively for large, high-grade sarcomas for which there is signifi- cant concern for local and distant recurrence. Management of Gastrointestinal Stromal Tumors 1059 Margaret von Mehren Gastrointestinal stromal tumors had the reputation for poor outcomes because of their lack of response to nonsurgical interventions. The discov- ery of gain-of-function mutations involving receptor tyrosine kinase growth factor receptors altered the biological understanding and management. Beginning in 2000, management of these tumors has changed dramati- cally because of the availability of tyrosine kinase inhibitors. The role of surgery continues to be refined. This article reviews how surgery and sys- temic therapy are being used, incorporating definitions of risk. Decisions on how to treat a patient is based on the risk of progression, pathologic characteristics, and tumor location. Management of Bone Sarcoma 1077 Christina J. Gutowski, Atrayee Basu-Mallick, and John A. Abraham Treatment of bone sarcoma requires careful planning and involvement of an experienced multidisciplinary team. Significant advancements in sys- temic therapy, radiation, and surgery in recent years have contributed to improved functional and survival outcomes for patients with these difficult tumors, and emerging technologies hold promise for further advancement. X Contents Pediatric Sarcomas 1107 Regan F. Williams, Israel Fernandez-Pineda, and Ankush Gosain Pediatric sarcomas are a heterogeneous group of tumors accounting for approximately 10% of childhood solid tumors. Treatment is focused on multimodality therapy, which has improved the prognosis over the past two decades. Current regimens focus on decreasing treatment for low-risk patients to decrease the long-term side effects while maximizing therapy for patients with metastatic disease to improve survival. Pediatric sarcomas can be divided into soft tissue sarcomas and osseous tumors. Soft tissue sarcomas are further delineated into rhabdomyosarcomas, which affect young children and nonrhabdomyosarcomas, which are most common in adolescents. The most common bone sarcomas are os- teosarcomas and Ewing s sarcoma. The Role for Radiation Therapy in the Management of Sarcoma 1127 Brooke K. Leachman and Thomas J. Galloway Although there is no consensus regarding the optimal sequencing of external beam radiotherapy and surgery for extremity soft tissue sarcoma, radiation therapy delivered before or after limb-sparing surgery signifi- cantly improves local control, particularly for high-grade tumors. Large database analyses suggest that improved local control may translate into an overall survival benefit. Best practices require ample communica- tion between the radiation and surgical teams to ensure appropriate tis- sues are targeted, unnecessary radiation is avoided, and patients are afforded the best opportunity for cure while maintaining function. Modern experiences with intensity-modulated radiotherapy/image-guided radia- tion therapy suggest toxicity is reduced through field size reduction and precise targeting, improving the therapeutic ratio. Systemic Therapy for Advanced Soft Tissue Sarcoma 1141 Jennifer Y. Sheng and Sujana Mowa Soft tissue sarcomas are rare tumors that present with distant metastasis in up to 10% of patients. Survival has improved significantly because of advancements in histologic classification and improved management approaches. Older agents such as doxorubicin, ifosfamide, gemcitabine, and paclitaxel continue to demonstrate objective response rates from 18% to 25%. Newer agents such as trabectedin, eribulin, aldoxorubicin, and olaratumab have demonstrated improvements in progression-free survival, overall survival, or toxicity profiles. Future studies on treatment of advanced soft tissue sarcoma will continue to concentrate on reducing toxicity, personalization of therapy, and targeting novel pathways. Local Recurrence of Extremity Soft Tissue Sarcoma 1157 Whitney M. Guerrero and Jeremiah L. Deneve The management of recurrent soft tissue sarcoma is a challenging problem for clinicians and has a significant physical, mental, emotional, and oncologic impact for the patient. Despite excellent limb-preservation therapies, Contents xi approximately one-quarter of patients may eventually develop recurrence of disease. How to most appropriately manage these patients is a matter of debate. Several treatment options exist, including surgical resection, irradi- ation, systemic chemotherapy, amputation, and regional therapies. This article highlights the management of recurrent extremity soft tissue sarcoma. Surgical Management of Metastatic Disease 1175 Emily Z. Keung, Mark Fairweather, and Chandrajit P. Raut Sarcomas are rare cancers of mesenchymal cell origin that include many histologic subtypes and molecularly distinct entities. For primary resectable sarcoma, surgery is the mainstay of treatment. Despite treat- ment, approximately 50% of patients with soft tissue sarcoma are diag- nosed with or develop distant metastases, significantly affecting their survival. Although systemic therapy with conventional chemotherapy re- mains the primary treatment modality for those with metastatic sarcoma, increased survival has been achieved in select patients who receive multi- modality therapy, including surgery, for their metastatic disease. This article provides an overview of the literature on surgical management of pulmonary and hepatic sarcoma metastases. Index 1193
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spellingShingle New trends in the treatment of sarcoma
Surgical clinics of North America
title New trends in the treatment of sarcoma
title_auth New trends in the treatment of sarcoma
title_exact_search New trends in the treatment of sarcoma
title_full New trends in the treatment of sarcoma editors Andrea S. Porpiglia, Jeffrey M. Farma
title_fullStr New trends in the treatment of sarcoma editors Andrea S. Porpiglia, Jeffrey M. Farma
title_full_unstemmed New trends in the treatment of sarcoma editors Andrea S. Porpiglia, Jeffrey M. Farma
title_short New trends in the treatment of sarcoma
title_sort new trends in the treatment of sarcoma
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