Mesenchymal chondrosarcoma in children and young adults

A single institution retrospective review

Michael W. Bishop, Jessica M. Somerville, Armita Bahrami, Sue C. Kaste, Rodrigo B. Interiano, Jianrong Wu, Shenghua Mao, Frederick Boop, Regan Williams, Alberto S. Pappo, Sandeep Samant

Research output: Contribution to journalArticle

13 Citations (Scopus)

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.

Original languageEnglish (US)
Article number608279
JournalSarcoma
Volume2015
DOIs
StatePublished - Jan 1 2015

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Mesenchymal Chondrosarcoma
Young Adult
Radiation
Recurrence
Drug Therapy
Ifosfamide
Preclinical Drug Evaluations
Neoadjuvant Therapy
Chemoradiotherapy
Adjuvant Chemotherapy
Heterografts
Doxorubicin
Disease-Free Survival
Neck
Head
Bone and Bones
Lung
Survival
Proteins

All Science Journal Classification (ASJC) codes

  • Oncology
  • Radiology Nuclear Medicine and imaging

Cite this

Bishop, M. W., Somerville, J. M., Bahrami, A., Kaste, S. C., Interiano, R. B., Wu, J., ... Samant, S. (2015). Mesenchymal chondrosarcoma in children and young adults: A single institution retrospective review. Sarcoma, 2015, [608279]. https://doi.org/10.1155/2015/608279

Mesenchymal chondrosarcoma in children and young adults : A single institution retrospective review. / Bishop, Michael W.; Somerville, Jessica M.; Bahrami, Armita; Kaste, Sue C.; Interiano, Rodrigo B.; Wu, Jianrong; Mao, Shenghua; Boop, Frederick; Williams, Regan; Pappo, Alberto S.; Samant, Sandeep.

In: Sarcoma, Vol. 2015, 608279, 01.01.2015.

Research output: Contribution to journalArticle

Bishop, MW, Somerville, JM, Bahrami, A, Kaste, SC, Interiano, RB, Wu, J, Mao, S, Boop, F, Williams, R, Pappo, AS & Samant, S 2015, 'Mesenchymal chondrosarcoma in children and young adults: A single institution retrospective review', Sarcoma, vol. 2015, 608279. https://doi.org/10.1155/2015/608279
Bishop MW, Somerville JM, Bahrami A, Kaste SC, Interiano RB, Wu J et al. Mesenchymal chondrosarcoma in children and young adults: A single institution retrospective review. Sarcoma. 2015 Jan 1;2015. 608279. https://doi.org/10.1155/2015/608279
Bishop, Michael W. ; Somerville, Jessica M. ; Bahrami, Armita ; Kaste, Sue C. ; Interiano, Rodrigo B. ; Wu, Jianrong ; Mao, Shenghua ; Boop, Frederick ; Williams, Regan ; Pappo, Alberto S. ; Samant, Sandeep. / Mesenchymal chondrosarcoma in children and young adults : A single institution retrospective review. In: Sarcoma. 2015 ; Vol. 2015.
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N2 - 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.

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