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Abstract
Osteosarcoma (OS), chondrosarcoma, and chordoma are characterized by multiple challenges
to the investigator, clinician, and patient. One consequence of their rarity among
sarcomas, as well as their biologic and clinical heterogeneity, is that management
guidelines are inadequate to inform the range of individual patient-treatment decisions
from diagnosis, approaches to surgery, chemotherapy, radiotherapy, treatment of recurrence,
palliative care, and quality of survivorship. Of high-grade sarcomas, OSs are among
the most curable, with more than two-thirds of patients with localized disease likely
to achieve long-term survival. Neoadjuvant chemotherapy comprising cisplatin, doxorubicin,
and methotrexate with intercalated surgery is the standard of care for resectable
OS in those younger than 40 years. Outcomes for OS presenting with unresectable metastases
or recurrent disease, or in those older than 40 years are generally poor. Overall
results have improved little for all patients with OS, and new treatments are needed.
Surgical resection remains the cornerstone of management for chondrosarcoma and chordoma.
However, the application of new biologic insights to therapeutic development indicates
that improved treatments may soon be routine for patients with chondrosarcoma and
chordoma for whom surgery alone is inadequate. For all these uncommon diseases, patients
should be offered specialist expert care delivered by experienced multidisciplinary
teams in high-volume centers.
Publisher:
American Society of Clinical Oncology (ASCO)
ISSN
(Print):
0732-183X
ISSN
(Electronic):
1527-7755
Publication date Created:
January
10 2018
Publication date
(Print):
January
10 2018
Volume: 36
Issue: 2
Pages: 188-193
Affiliations
[1
]Jeremy S. Whelan, University College London Hospitals NHS Foundation Trust, London,
United Kingdom; and Lara E. Davis, Knight Cancer Institute, Oregon Health & Science
University, Portland, OR.