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Sarcoma
Volume 2015, Article ID 608279, 6 pages
http://dx.doi.org/10.1155/2015/608279
Clinical Study

Mesenchymal Chondrosarcoma in Children and Young Adults: A Single Institution Retrospective Review

1Department of Oncology, St. Jude Children’s Research Hospital, 262 Danny Thomas Place, Memphis, TN 38105, USA
2Department of Pediatrics, University of Tennessee Health Science Center, Memphis, TN 38105, USA
3Department of Otolaryngology, University of Tennessee Health Science Center, Memphis, TN 38105, USA
4Department of Pathology, St. Jude Children’s Research Hospital, 262 Danny Thomas Place, Memphis, TN 38105, USA
5Department of Radiological Sciences, St. Jude Children’s Research Hospital, 262 Danny Thomas Place, Memphis, TN 38105, USA
6Department of Radiology, University of Tennessee Health Science Center, Memphis, TN 38105, USA
7Department of Surgery, University of Tennessee Health Science Center, Memphis, TN 38105, USA
8Department of Biostatistics, St. Jude Children’s Research Hospital, 262 Danny Thomas Place, Memphis, TN 38105, USA
9Department of Neurosurgery, University of Tennessee Health Science Center, Memphis, TN 38105, USA

Received 29 January 2015; Revised 4 May 2015; Accepted 21 May 2015

Academic Editor: Silvia Stacchiotti

Copyright © 2015 Michael W. Bishop et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Background. Mesenchymal chondrosarcoma is an aggressive, uncommon histologic entity arising in bone and soft tissues. We reviewed our institutional experience with this rare diagnosis. Methods. We conducted a retrospective chart review on patients with mesenchymal chondrosarcoma over a 24-year period. Clinicopathologic and radiographic features were reviewed. Results. Twelve patients were identified. Nine were females; median age was 14.5 years (1.2–19.7 years). The most common site was the head/neck (7/12). Disease was localized in 11/12 patients (one with lung nodules). Six with available tissue demonstrated NCOA2 rearrangement by FISH. Six underwent upfront surgical resection, and six received neoadjuvant therapy (2 chemotherapy alone and 4 chemotherapy and radiation). All patients received adjuvant chemotherapy (most commonly ifosfamide/doxorubicin) and/or radiation (median dose 59.4 Gy). At a median follow-up of 4.8 years, 5-year disease-free survival and overall survival were 68.2% (95% CI 39.8%, 96.6%) and 88.9% (95% CI 66.9%, 100%). Two patients had distant recurrences at 15 and 42 months, respectively. Conclusion. Aggressive surgical resection of mesenchymal chondrosarcoma with chemoradiotherapy yields excellent local control and may reduce likelihood of late recurrence. Characterization of downstream targets of the HEY1-NCOA2 fusion protein, xenograft models, and drug screening are needed to identify novel therapeutic strategies.