New trends in the treatment of sarcoma
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Philadelphia, Pennsylvania
Elsevier
[2016]
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Schriftenreihe: | Surgical clinics of North America
volume 96, number 5 (October 2016) |
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245 | 1 | 0 | |a New trends in the treatment of sarcoma |c editors Andrea S. Porpiglia, Jeffrey M. Farma |
264 | 1 | |a Philadelphia, Pennsylvania |b Elsevier |c [2016] | |
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700 | 1 | |a Farma, Jeffrey M. |4 edt | |
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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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