The Journal of Bone and Joint Surgery (American). 2004;86:2412-2418
© 2004 The Journal of Bone and Joint Surgery, Inc.
Dedifferentiated Chondrosarcoma: The Role of Chemotherapy with Updated Outcomes
Ian D. Dickey, MD1,
Peter S. Rose, MD1,
Bruno Fuchs, MD, PhD1,
Lester E. Wold, MD1,
Scott H. Okuno, MD1,
Franklin H. Sim, MD1 and
Sean P. Scully, MD, PhD2
1 Department of Orthopedic Surgery, Mayo Clinic College of Medicine, 200 First
Street S.W., Med Sci 3-69, Rochester, MN 55905
2 Department of Orthopaedics and Rehabilitation, University of Miami School of
Medicine (D-27), P.O. Box 016960, Miami, FL 33101. E-mail address:
sscully{at}med.miami.edu
Investigation performed at the Department of Orthopedic Surgery, Mayo
Clinic College of Medicine, Rochester, Minnesota
A commentary is available with the electronic versions of this article,
on our web site
(www.jbjs.org)
and on our quarterly CD-ROM (call our subscription department, at
781-449-9780, to order the CD-ROM).
The authors did not receive grants or outside funding in support of their
research or preparation of this manuscript. They did not receive payments or
other benefits or a commitment or agreement to provide such benefits from a
commercial entity. No commercial entity paid or directed, or agreed to pay or
direct, any benefits to any research fund, foundation, educational
institution, or other charitable or nonprofit organization with which the
authors are affiliated or associated.
Background: There are very few published data on the survival of
patients with dedifferentiated chondrosarcoma, or, more specifically, on the
efficacy and role of chemotherapy, especially in the era of modern diagnostic
and treatment modalities. The current study examines the influence of
advancements in imaging and chemotherapy on outcome and serves as an extension
to a previous study published in 1986.
Methods: Forty-two patients with dedifferentiated chondrosarcoma who
had presented to our institution between 1986 and 2000 were identified, and a
retrospective chart review was performed. The study group included twenty-four
men and eighteen women with an average age of sixty-six years. The diagnosis
of dedifferentiated chondrosarcoma was verified histologically, and data on
treatment, adjuvant therapy, and survival were obtained from the medical
records of all patients. All patients had been followed for a minimum of
twenty-four months.
Results: The tumors were classified, according to the system of the
Musculoskeletal Tumor Society, as grade IIA (five), grade IIB (twenty-six),
and grade III (eleven). Three patients underwent biopsy only, eighteen had a
limb-sacrificing procedure, and twenty-one had a limb-sparing procedure. In
the group of patients who underwent resection, the surgical margins were
classified as intralesional in three, marginal in two, wide in nineteen, and
radical in fifteen. Twenty-seven patients received neoadjuvant therapy; of
these, twenty-three received chemotherapy only, two received radiotherapy
only, and two received combined therapy. The median survival time was 7.5
months, and the five-year rate of disease-free survival was 7.1%. With the
numbers available, there was no significant difference in the rate of
disease-free survival with respect to the use of chemotherapy (p = 0.54), the
location of surgical margins (p = 0.14), the histological subtype (p = 0.87),
the tumor stage at the time of diagnosis (p = 0.43), the tumor size (p =
0.79), or the performance of limb-sparing as opposed to limb-sacrificing
procedures (p = 0.42).
Conclusions: Despite advances in diagnostic modalities and adjuvant
therapies, dedifferentiated chondrosarcoma continues to carry a poor
prognosis. While local control is achieved in the majority of cases, distant
disease remains the greatest clinical challenge, developing in 90% of
patients. Efforts are needed to continue to encourage earlier diagnosis and to
develop effective adjuvant therapies for the control of distant disease. The
routine use of current adjuvant chemotherapy and its inherent risks in this
population should be reconsidered.
Level of Evidence: Therapeutic study, Level IV (case
series [no, or historical, control group]). See Instructions to Authors for a
complete description of levels of evidence.

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