Mesenchymal Chondrosarcoma in Children and Young Adults: A Single Institution Retrospective Review
Joint Authors
Pappo, Alberto
Bishop, Michael W.
Somerville, Jessica M.
Bahrami, Armita
Kaste, Sue C.
Interiano, Rodrigo B.
Wu, Jianrong
Mao, Shenghua
Boop, Frederick A.
Williams, Regan F.
Samant, Sandeep
Source
Issue
Vol. 2015, Issue 2015 (31 Dec. 2015), pp.1-6, 6 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2015-06-03
Country of Publication
Egypt
No. of Pages
6
Main Subjects
Abstract EN
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.
American Psychological Association (APA)
Bishop, Michael W.& Somerville, Jessica M.& Bahrami, Armita& Kaste, Sue C.& Interiano, Rodrigo B.& Wu, Jianrong…[et al.]. 2015. Mesenchymal Chondrosarcoma in Children and Young Adults: A Single Institution Retrospective Review. Complexity،Vol. 2015, no. 2015, pp.1-6.
https://search.emarefa.net/detail/BIM-1076084
Modern Language Association (MLA)
Bishop, Michael W.…[et al.]. Mesenchymal Chondrosarcoma in Children and Young Adults: A Single Institution Retrospective Review. Complexity No. 2015 (2015), pp.1-6.
https://search.emarefa.net/detail/BIM-1076084
American Medical Association (AMA)
Bishop, Michael W.& Somerville, Jessica M.& Bahrami, Armita& Kaste, Sue C.& Interiano, Rodrigo B.& Wu, Jianrong…[et al.]. Mesenchymal Chondrosarcoma in Children and Young Adults: A Single Institution Retrospective Review. Complexity. 2015. Vol. 2015, no. 2015, pp.1-6.
https://search.emarefa.net/detail/BIM-1076084
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1076084