title: Mesenchymal Chondrosarcoma in Children and Young Adults: A Single Institution Retrospective Review.
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.
authors.affiliation: Department of Oncology, St. Jude Children's Research Hospital, 262 Danny Thomas Place, Memphis, TN 38105, USA ; Department of Pediatrics, University of Tennessee Health Science Center, Memphis, TN 38105, USA.
authors.forename: Michael W
authors.initials: MW
authors.lastname: Bishop
authors.affiliation: Department of Otolaryngology, University of Tennessee Health Science Center, Memphis, TN 38105, USA.
authors.forename: Jessica M
authors.initials: JM
authors.lastname: Somerville
authors.affiliation: Department of Pathology, St. Jude Children's Research Hospital, 262 Danny Thomas Place, Memphis, TN 38105, USA.
authors.forename: Armita
authors.initials: A
authors.lastname: Bahrami
authors.affiliation: Department of Oncology, St. Jude Children's Research Hospital, 262 Danny Thomas Place, Memphis, TN 38105, USA ; Department of Radiological Sciences, St. Jude Children's Research Hospital, 262 Danny Thomas Place, Memphis, TN 38105, USA ; Department of Radiology, University of Tennessee Health Science Center, Memphis, TN 38105, USA.
authors.forename: Sue C
authors.initials: SC
authors.lastname: Kaste
authors.affiliation: Department of Surgery, University of Tennessee Health Science Center, Memphis, TN 38105, USA.
authors.forename: Rodrigo B
authors.initials: RB
authors.lastname: Interiano
authors.affiliation: Department of Biostatistics, St. Jude Children's Research Hospital, 262 Danny Thomas Place, Memphis, TN 38105, USA.
authors.forename: Jianrong
authors.initials: J
authors.lastname: Wu
authors.affiliation: Department of Biostatistics, St. Jude Children's Research Hospital, 262 Danny Thomas Place, Memphis, TN 38105, USA.
authors.forename: Shenghua
authors.initials: S
authors.lastname: Mao
authors.affiliation: Department of Neurosurgery, University of Tennessee Health Science Center, Memphis, TN 38105, USA.
authors.forename: Frederick A
authors.initials: FA
authors.lastname: Boop
authors.affiliation: Department of Surgery, University of Tennessee Health Science Center, Memphis, TN 38105, USA.
authors.forename: Regan F
authors.initials: RF
authors.lastname: Williams
authors.affiliation: Department of Oncology, St. Jude Children's Research Hospital, 262 Danny Thomas Place, Memphis, TN 38105, USA.
authors.forename: Alberto S
authors.initials: AS
authors.lastname: Pappo
authors.affiliation: Department of Otolaryngology, University of Tennessee Health Science Center, Memphis, TN 38105, USA.
authors.forename: Sandeep
authors.initials: S
authors.lastname: Samant