LWBK1006-26 LWW-Govindan-Review December 12, 2011 19:29
Chapter 26•Sarcomas 373definitive radiation would offer lower chances of success. Chemotherapy
alone would provide palliation, but the chance of cure is unclear.Answer 26.17. The answer is C.
Parosteal osteosarcoma is usually a low-grade tumor that involves the
bone cortex and is associated with a better prognosis than classic osteosar-
coma. Resection is the preferred treatment, and there is no clear role
for chemotherapy. In contrast, periosteal osteosarcoma is an aggressive,
high-grade cortical sarcoma that is best treated like classic osteosarcoma
with resection and chemotherapy. For classic high-grade osteosarcoma,
resection alone is curative in 20%, but the addition of chemotherapy to
resection improves cure rates to 60% to 80%.Answer 26.18. The answer is D.
The preferred therapy for chondrosarcoma is resection. Although chon-
drosarcomas as a group are known to be resistant to chemotherapy, mes-
enchymal and dedifferentiated chondrosarcomas have a high metastatic
potential and appears to benefit from adjuvant chemotherapy. Most chon-
drosarcomas are low grade and usually occur in patients aged more than
40 years. Children have a worse prognosis than adults.Answer 26.19. The answer is A.
GCT of bone is a tumor with a high risk for local recurrence but a low
risk for distal metastases. Appropriate treatment is resection, preferably
by curettage and debridement. Amputation is reserved for massive local
recurrences. Radiation is reserved for lesions of the spine that can lead
to cord compression. There is no benefit of preoperative or adjuvant
chemotherapy for GCT of bone.Answer 26.20. The answer is B.
A randomized trial of patients with Ewing’s sarcoma treated with either
vincristine, doxorubicin, and cyclophosphamide (VAC) alone or alter-
nating with IE given as preoperative and adjuvant chemotherapy clearly
showed an overall survival and progression-free survival advantage with
the alternating regimen of VAC and IE versus VAC alone in patients with
nonmetastatic (but not in patients with metastatic) disease. Resection
alone or with radiation would be significantly less effective than when
used in combination with chemotherapy.Answer 26.21. The answer is C.
Alveolar soft part sarcoma is associated with der (17)t(X;17) (p11,q25).
Ewing’s sarcoma is associated with five known cytogenetic abnormali-
ties, includingt(11; 22) (q24; q12). Synovial sarcoma is associated with
t(X;18) (p11; q11), whereas myxoid liposarcoma is associated witht(12;
16) (q13; p11).